**Development of Classification Criteria for the**

**Idiopathic Inflammatory Myopathies and their Major Subgroups**

**[Data Collection Form, Variable List and Glossary]{.underline}**

**[In reviewing patients please use the following criteria for selecting
cases for submission:]{.underline}**

> i\. The subject has been diagnosed for at least 6 months
>
> ii\. The physician is certain of the diagnosis -- only cases with
> known idiopathic inflammatory myopathy or, as comparators, known
> non-IIM cases (but in which myositis was considered in the initial
> differential diagnosis) are chosen

iii\. The patients in whom most complete data are available

> iv\. The most recent cases are chosen first -- these would likely
> result in more consistent evaluations and therapy

**[GENERAL INFORMATION]{.underline}**

**Have you received approval from your local IRB or ethics committee for
participation in this project?**

Yes □ Exempt □ No □

Please scan and email or fax the letter of approval to Dr Ingrid
Lundberg, Rheumatology Unit Karolinska University Hospital, Stockholm,
Sweden. Ingrid.Lundberg@ki.se, Fax no: +46 8 5177 3080.

Hereafter you will receive a study number of your center.

**Date of data entry:** (dd/month/year)
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_-

**1.** Has your patient been diagnosed with the diagnosis relevant for
this study for more than 6 months?

A yes is required, if No select a new case. Yes □ No □

**2. Center** (name of university or hospital from where data is
entered)

\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**3. Clinician submitting case:**

**Family
name**\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**First
name**\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**Email**
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**Postal**
address\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**4**. **Case identifier/number**

Please fill in the study ID numbers that you have been given by the
coordinating center.

Center number \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

Physician number \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

Case number \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**5. Gender**: □ Female

□ Male

**6**. Adult onset (diagnosed at age ≥18) or childhood onset (diagnosed
at age \< 18 years)

Adult □ Childhood □

**7**. **Age (years) at onset** of first symptom assumed to be related
to the disease: \_\_\_\_years and for children months: \_\_\_ months
(include years and months for children)

**8. Age (years) at diagnosis**: \_\_\_\_years \_\_\_ months (include
years and months for children)

**9. Age (years) at last evaluation**: \_\_\_\_years \_\_\_ months
(include years and months for children)

**10. Ethnicity**: (check all that apply)

□ Caucasian

> □ Of African descent
>
> □ Of Asian descent
>
> □ Of Native American descent
>
> □ Of Pacific Island descent
>
> □ Of Hispanic descent
>
> □ Of Mixed descent
>
> □ Unknown

**11.** Study diagnosis according to the clinician submitting the case

□ Idiopathic inflammatory myopathy (IIM) adults or children

□ Not Idiopathic inflammatory myopathy (Not IIM) adults or children

**12.** Study diagnosis: Idiopathic Inflammatory Myopathy (IIM) in
adults or children

Please select only one diagnosis, where requested please specify
diagnosis below in the text box.

> □ Polymyositis

□ Dermatomyositis

□ Amyopathic dermatomyositis

□ Hypomyopathic dermatomyositis

□ Inclusion body myositis

□ Immune-mediated necrotizing myopathy

□ Juvenile dermatomyositis

□ Juvenile polymyositis

□ Other diagnosis, specify diagnosis below

> □ Not Idiopathic Inflammatory Myopathy (IIM)

\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

> **13. Not** Idiopathic Inflammatory Myopathy (Not IIM), adults or
> children, but in which the diagnosis of idiopathic myositis was
> considered in the differential diagnosis:
>
> Please select only one diagnosis, where requested please specify
> diagnosis below in the text box.

  -----------------------------------------------------------------------
  □ Becker´s dystrophy

  □ Duchenne´s dystrophy

  □ Fascioscapulohumeral dystrophy

  □ Limb-girdle dystrophy

  □ Myotonic dystrophy

  □ Non-inflammatory inclusion body myopathy

  □ Other dystrophy, specify diagnosis

  □ Dysferlinopathy

  □ Acid maltase deficiency

  □ Allergies

  □ Bacterial myopathy

  □ Carnitine deficiency

  □ Celiac disease

  □ Crohn's disease

  □ Cushing syndrome

  □ Cysticerosis

  □ Diabetes mellitus

  □ Drug or toxin associated myopathy, specify diagnosis

  □ Exogenous steroid myopathy

  □ Familial periodic paralysis

  □ Fibromyalgia

  □ Filiarisis

  □ Glucocorticoid induced myopathy

  □ Gullain-Barre syndrome

  □ Hypercalcemia

  □ Hypereosinophilic syndrome

  □ Hypersensitivity conditions

  □ Hyperthyroidism

  □ Hypocalcemia

  □ Hypokalemia

  □ Hypothyroidism

  □ Immune mediated skin conditions, specify diagnosis below

  □ Juvenile idiopathic arthritis

  □ Kearns-Sayre syndrome

  □ Mc Ardle´s disease

  □ Metabolic myopathy, specify diagnosis

  □ Mitochondrial encephalomyopathy, lactic acidosis, stroke (MELAS)

  □ Mitochondrial myopathy, specify diagnosis

  □ Mixed connective tissue disease

  □ Motor neuron diseases, specify diagnosis

  □ Multiple sclerosis

  □ Myasthenia gravis

  □ Myoadenylate deaminase deficiency

  □ Myoclonic epilepsy, ragged red fibers (MERRF)

  □ Palmityltransferase deficiency

  □ Parasitic myopathy

  □ Phosphofructokinase deficiency

  □ Psoriasis

  □ Seborhheic dermatitis

  □ Statin induced myopathy

  □ Systemic lupus erythematosus (SLE)

  □ Systemic sclerosis

  □ Systemic vasculitis, specify diagnosis below

  □ Toxoplasmosis

  □ Trichinosis

  □ Trypanasoma

  □ Ulcerative colitis

  □ Verrucae vulgaris

  □ Viral myopathy

  □ Other dermatologic disease, specify diagnosis below

  □ Other endocrine myopathy, specify diagnosis

  □ Other infectious myopathy, specify diagnosis

  □ Other neuromuscular disease, specify diagnosis below

  □ Other systemic autoimmune disease, specify diagnosis below

  □ Other diagnosis, specify

  □ None applicable (Inflammatory Myopathy)
  -----------------------------------------------------------------------

If other diagnosis please
specify:\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\--

**14. Basis for study diagnosis** (check all supporting reasons):

□ Muscle weakness

□ Muscle biopsy abnormalities

□ Elevated muscle enzymes

□ EMG abnormalities

□ Rashes

□ Skin biopsy

□ Autoantibodies

□ MRI

□ Other, please specify
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**14. Other diagnoses in this case:** (check all that apply):

> □ Non applicable

□ Systemic sclerosis

□ Sjögren´s syndrome

□ Mixed connective tissue disease

□ Rheumatoid arthritis

> □ Systemic lupus erythematosus
>
> □ Hypothyroidism

□ Hyperthyroidism

□ Type I diabetes

□ Juvenile idiopathic arthritis

□ Malignancy

If yes, please specify type of
malignancy\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

If yes, add age at diagnosis of malignancy
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

□ Other, please specify
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

***Before completing the following tables, please review the Glossary of
Definitions below.***

The variables in italics have been included in previous sets of criteria
for inflammatory myopathies

+------------------------------+-------+-------+-----------+-----------+
|                              | **[Pr | **[A  | **[In     | **[Comme  |
|                              | esent | bsent | formation | nts]{.und |
|                              | ]{.un | ]{.un | not       | erline}** |
|                              | derli | derli | availa    |           |
|                              | ne}** | ne}** | ble]{.und |           |
|                              |       |       | erline}** |           |
+------------------------------+-------+-------+-----------+-----------+
| **Clinical Muscle Variables  |       |       |           |           |
| -- present at any**          |       |       |           |           |
|                              |       |       |           |           |
| **time during the disease    |       |       |           |           |
| course**                     |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *1M. Objective symmetric     |       |       |           |           |
| weakness, usually            |       |       |           |           |
| progressive, of the proximal |       |       |           |           |
| upper extremities*           |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *2M, Objective shoulder      |       |       |           |           |
| abductor weakness*           |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *3M. Objective elbow flexor  |       |       |           |           |
| weakness*                    |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *4M. Objective elbow         |       |       |           |           |
| extensor weakness*           |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *5M. Wrist and finger        |       |       |           |           |
| flexors are relatively       |       |       |           |           |
| weaker than shoulder         |       |       |           |           |
| abductors on the same side*  |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *6M. Wrist flexors are       |       |       |           |           |
| relatively weaker than wrist |       |       |           |           |
| extensors on the same side*  |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *7M. Objective finger flexor |       |       |           |           |
| weakness*                    |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *8M. Objective symmetric     |       |       |           |           |
| weakness, usually            |       |       |           |           |
| progressive, of the proximal |       |       |           |           |
| lower extremities*           |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *9M. Objective hip flexor    |       |       |           |           |
| weakness*                    |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *10M. Objective hip abductor |       |       |           |           |
| weakness*                    |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *11M. Objective knee         |       |       |           |           |
| extensor weakness*           |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 12M. Knee extensors are as   |       |       |           |           |
| weak or relatively weaker    |       |       |           |           |
| than hip girdle muscle on    |       |       |           |           |
| the same side                |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 13M. Objective muscle        |       |       |           |           |
| weakness of distal lower     |       |       |           |           |
| extremities                  |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *14M. Objective axial        |       |       |           |           |
| weakness*                    |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 15M. Objective neck flexor   |       |       |           |           |
| weakness                     |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *16M. Neck flexors are*      |       |       |           |           |
| relatively *weaker than neck |       |       |           |           |
| extensors*                   |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *17M. In the legs proximal   |       |       |           |           |
| muscles are* relatively      |       |       |           |           |
| *weaker than distal muscles* |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *18M. In the arms proximal   |       |       |           |           |
| muscles are relatively       |       |       |           |           |
| weaker than distal muscles*  |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 19M In the legs distal       |       |       |           |           |
| muscles relatively weaker    |       |       |           |           |
| than proximal muscles        |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 20M In the arms distal       |       |       |           |           |
| muscles are relatively       |       |       |           |           |
| weaker than proximal muscles |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 21M. Muscle tenderness       |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 22M. Muscle atrophy of       |       |       |           |           |
| distal forearms              |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 23M. Muscle atrophy of       |       |       |           |           |
| thighs                       |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
|                              | **[Pr | **[A  | **[In     | **[Comme  |
|                              | esent | bsent | formation | nts]{.und |
|                              | ]{.un | ]{.un | not       | erline}** |
|                              | derli | derli | availa    |           |
|                              | ne}** | ne}** | ble]{.und |           |
|                              |       |       | erline}** |           |
+------------------------------+-------+-------+-----------+-----------+
| **Skin Variables -- present  |       |       |           |           |
| at any time**                |       |       |           |           |
|                              |       |       |           |           |
| **during the disease         |       |       |           |           |
| course**                     |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *1S. Heliotrope rash*        |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *2S. Gottron´s papules*      |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *3S. Gottron's sign*         |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *4S. Erythema of the back of |       |       |           |           |
| neck and shoulders (Shawl    |       |       |           |           |
| sign)*                       |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *5S. Erythema of the neck    |       |       |           |           |
| (V-sign)*                    |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *6S. Periorbital edema*      |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| **7**S. Linear extensor      |       |       |           |           |
| erythema                     |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 8S. Calcification            |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 9S. Periungual erythema or   |       |       |           |           |
| nailfold capillary           |       |       |           |           |
| abnormality                  |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 10S. Mechanic's hands        |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 11S. Photodistributed        |       |       |           |           |
| violaceous erythema          |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 12 S. Raynaud's phenomenon   |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 13S. Cuticular overgrowth    |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 14S Poikiloderma             |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
|                              | **[Pr | **[A  | **[In     | **[Comme  |
|                              | esent | bsent | formation | nts]{.und |
|                              | ]{.un | ]{.un | not       | erline}** |
|                              | derli | derli | availa    |           |
|                              | ne}** | ne}** | ble]{.und |           |
|                              |       |       | erline}** |           |
+------------------------------+-------+-------+-----------+-----------+
| **Other Clinical Variables   |       |       |           |           |
| -- present at any**          |       |       |           |           |
|                              |       |       |           |           |
| **time during the disease    |       |       |           |           |
| course**                     |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 1O. Family history of        |       |       |           |           |
| autoimmune disease (see      |       |       |           |           |
| Appendix A)                  |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 2O. Family history of muscle |       |       |           |           |
| disease (See Appendix B)     |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 3Oa. Acute onset (days to 2  |       |       |           |           |
| weeks) of symptoms           |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 3Ob. Subacute onset (\> 2    |       |       |           |           |
| weeks to ≤2 months) of       |       |       |           |           |
| symptoms                     |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 3Oc. Insidious onset of      |       |       |           |           |
| symptoms \> 2 months to      |       |       |           |           |
| years                        |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 4O. History of episodic      |       |       |           |           |
| weakness associated with     |       |       |           |           |
| exercise or fasting          |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *5O. Arthritis*              |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *6O. Polyarthralgia*         |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 7O. Joint contractures       |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *8O. Unexplained Fevers*     |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 9O. Interstitial lung        |       |       |           |           |
| disease                      |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 1O. Dysphagia or esophageal  |       |       |           |           |
| dysmotility                  |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+

  ----------------------------- ---------- ---------- --------- ------------ -------
  13O. Objective improvement in Improved   Not        Unknown   Inadequate   Not
  strength or other disease                improved             trial        used
  manifestation after an                                                     
  adequate trial of                                                          
  glucocorticoids and/or other                                               
  immunosuppressive or immune                                                
  modulating therapy for at                                                  
  least 8 w. Check all that                                                  
  apply.                                                                     

  **prednisone ≥0.75-2                                                       
  mg/kg/day (or equivalent)**                                                

  **methotrexate ≥10 mg/week                                                 
  (children: ≥0.3 mg/kg/week)**                                              

  **azathioprine 75 mg/d (or 2                                               
  mg/kg/day)**                                                               

  **Other**                                                                  

  Specify other                                                              
  immunosuppressive medication:                                              
  ----------------------------- ---------- ---------- --------- ------------ -------

+------------------------------+------+----------+-----------+--------+
|                              | *    | **[Absen | **[Not    | **     |
|                              | *[Pr | t]{.unde | availa    | [Comme |
|                              | esen | rline}** | ble]{.und | nts]{. |
|                              | t]{. |          | erline}** | underl |
|                              | unde |          |           | ine}** |
|                              | rlin |          |           |        |
|                              | e}** |          |           |        |
+------------------------------+------+----------+-----------+--------+
| **Muscle Biopsy Variables -- |      |          |           |        |
| from any biopsy**            |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| **Muscle biopsy data         |      |          |           |        |
| available**                  |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| *1B. Necrosis of type I and  |      |          |           |        |
| type II muscle fibers,       |      |          |           |        |
| phagocytosis, degeneration   |      |          |           |        |
| of myofibers*                |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| *2B. Regeneration of         |      |          |           |        |
| myofibers*                   |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| *3B. Endomysial infiltration |      |          |           |        |
| of mononuclear cells         |      |          |           |        |
| surrounding, but not         |      |          |           |        |
| invading, myofibers*         |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| *4B. Non-necrotic fibers     |      |          |           |        |
| surrounded and invaded by    |      |          |           |        |
| mononuclear cells*           |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| *5B.Perimysial and/or        |      |          |           |        |
| perivascular infiltration of |      |          |           |        |
| mononuclear cells*           |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| *6B. Perifascicular atrophy* |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 7B. Vacuolated muscle fibers |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 8B. Rimmed vacuoles          |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 9B. Ragged red fibers, or    |      |          |           |        |
| cytochrome C                 |      |          |           |        |
| oxidase-negative fibers      |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 10B. Many necrotic muscle    |      |          |           |        |
| fibers as the                |      |          |           |        |
|                              |      |          |           |        |
| predominant feature.         |      |          |           |        |
| Inflammatory cells are       |      |          |           |        |
| sparse; perimysial           |      |          |           |        |
| infiltrate is not evident.   |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 11\. Immunohistochemistry    |      |          |           |        |
| data available if not go to  |      |          |           |        |
| xxx                          |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 12B. MHC Class I antigen     |      |          |           |        |
| present on scattered or more |      |          |           |        |
| muscle fibers                |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 13B. Endomysial CD8+ cells   |      |          |           |        |
| surrounding myofibers with   |      |          |           |        |
| MHC Class I expression on    |      |          |           |        |
| myofibers                    |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 14B. Membrane attack complex |      |          |           |        |
| (MAC) depositions on small   |      |          |           |        |
| blood vessels                |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 15B. Reduced capillary       |      |          |           |        |
| density                      |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 16B. MHC-1 expression of     |      |          |           |        |
| perifascicular fibers        |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 17B. Electron microscopy     |      |          |           |        |
| available y/n                |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 18B. Tubuloreticular         |      |          |           |        |
| inclusions in endothelial    |      |          |           |        |
| cells on electron microscopy |      |          |           |        |
+------------------------------+------+----------+-----------+--------+
| 19B. Intracellular amyloid   |      |          |           |        |
| deposits or 15-18 nm         |      |          |           |        |
| tubulofilaments by electron  |      |          |           |        |
| microscopy (EM)              |      |          |           |        |
+------------------------------+------+----------+-----------+--------+

+--------------------------------+-------+-------+---+-------+---------+
| **Laboratory Variables --      |       |       |   |       |         |
| record the most abnormal test  |       |       |   |       |         |
| values during the disease      |       |       |   |       |         |
| course**                       |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
|                                | **[   | **[   |   |       | **      |
|                                | Value | Upper |   |       | [Units] |
|                                | ]{.un | n     |   |       | {.under |
|                                | derli | ormal |   |       | line}** |
|                                | ne}** | limit |   |       |         |
|                                |       | ]{.un |   |       |         |
|                                |       | derli |   |       |         |
|                                |       | ne}** |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| *1L. Serum creatine kinase     |       |       |   |       |         |
| (CK) activity*                 |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| *2L. Serum lactate             |       |       |   |       |         |
| dehydrogenase (LDH) activity*  |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| *3L. Serum aspartate           |       |       |   |       |         |
| aminotransferase               |       |       |   |       |         |
| (ASAT/AST/SGOT) activity*      |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| *4L. Serum alanine             |       |       |   |       |         |
| aminotransferase               |       |       |   |       |         |
| (ALAT/ALT/SGPT) activity*      |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| 5L. Serum Aldolase activity    |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| *6L. Erythrocyte sedimentation |       |       |   |       |         |
| rate (ESR)*                    |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| *7L. C-reactive protein (CRP)* |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
|                                |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| *8L. **Autoantibody tests      | **Y   | **    |   |       |         |
| available y/n***               | es:** | No:** |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
|                                |       |       |   |       |         |
| -----------------------------  |       |       |   |       |         |
| ---------- ---------- -------- |       |       |   |       |         |
|   9L. Autoantibodies           |       |       |   |       |         |
|      Positive   Negative   Not |       |       |   |       |         |
|                                |       |       |   |       |         |
|                         tested |       |       |   |       |         |
|                                |       |       |   |       |         |
|   ANA                          |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-Jo-1 (anti-His)         |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-Mi-2                    |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-SRP                     |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-Ku                      |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti- PL7                    |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti- PL-12                  |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti PM-Scl                  |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-SSA                     |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-Ro52/SSA                |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-Ro60/SSA                |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-La/SSB                  |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-ribonucleoprotein       |       |       |   |       |         |
|                                |       |       |   |       |         |
|   (RNP)-70K (U1snRNP)          |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-RNP-A                   |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-RNP-C                   |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-Centromere B (ACA       |       |       |   |       |         |
| )                              |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-Topoisomerase-1/S       |       |       |   |       |         |
| cl70,                          |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-Ribosomal P antig       |       |       |   |       |         |
| en                             |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-Sm                      |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-SmB                     |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-SmD                     |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   RF                           |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Anti-CCP                     |       |       |   |       |         |
|                                |       |       |   |       |         |
|                                |       |       |   |       |         |
|   Other, please specify        |       |       |   |       |         |
| below                          |       |       |   |       |         |
|                                |       |       |   |       |         |
| -----------------------------  |       |       |   |       |         |
| ---------- ---------- -------- |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
|                                | **[Pr | **[A  | * |       | **[Co   |
|                                | esent | bsent | * |       | mments] |
|                                | ]{.un | ]{.un | [ |       | {.under |
|                                | derli | derli | I |       | line}** |
|                                | ne}** | ne}** | n |       |         |
|                                |       |       | f |       |         |
|                                |       |       | o |       |         |
|                                |       |       | r |       |         |
|                                |       |       | m |       |         |
|                                |       |       | a |       |         |
|                                |       |       | t |       |         |
|                                |       |       | i |       |         |
|                                |       |       | o |       |         |
|                                |       |       | n |       |         |
|                                |       |       | n |       |         |
|                                |       |       | o |       |         |
|                                |       |       | t |       |         |
|                                |       |       | a |       |         |
|                                |       |       | v |       |         |
|                                |       |       | a |       |         |
|                                |       |       | i |       |         |
|                                |       |       | l |       |         |
|                                |       |       | a |       |         |
|                                |       |       | b |       |         |
|                                |       |       | l |       |         |
|                                |       |       | e |       |         |
|                                |       |       | ] |       |         |
|                                |       |       | { |       |         |
|                                |       |       | . |       |         |
|                                |       |       | u |       |         |
|                                |       |       | n |       |         |
|                                |       |       | d |       |         |
|                                |       |       | e |       |         |
|                                |       |       | r |       |         |
|                                |       |       | l |       |         |
|                                |       |       | i |       |         |
|                                |       |       | n |       |         |
|                                |       |       | e |       |         |
|                                |       |       | } |       |         |
|                                |       |       | * |       |         |
|                                |       |       | * |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| ***EMG performed y/n***        | **Y   |       | * |       |         |
|                                | es:** |       | * |       |         |
|                                |       |       | N |       |         |
|                                |       |       | o |       |         |
|                                |       |       | : |       |         |
|                                |       |       | * |       |         |
|                                |       |       | * |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| *1 . Electromyogram (EMG) -    |       |       |   |       |         |
| Increased insertional and      |       |       |   |       |         |
| spontaneous activity in the    |       |       |   |       |         |
| form of fibrillation           |       |       |   |       |         |
| potentials, positive sharp     |       |       |   |       |         |
| waves, or complex repetitive   |       |       |   |       |         |
| discharges*                    |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| *1L. EMG - Morphometric        |       |       |   |       |         |
| analysis reveals the presence  |       |       |   |       |         |
| of short duration, small       |       |       |   |       |         |
| amplitude, polyphasic motor    |       |       |   |       |         |
| unit action potentials         |       |       |   |       |         |
| (MUAPs)*                       |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| MRI of muscles performed y/n   | **Y   |       | * |       |         |
|                                | es:** |       | * |       |         |
|                                |       |       | N |       |         |
|                                |       |       | o |       |         |
|                                |       |       | : |       |         |
|                                |       |       | * |       |         |
|                                |       |       | * |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| 1\. Muscle edema on STIR or    |       |       |   |       |         |
| T2-weighted magnetic resonance |       |       |   |       |         |
| imaging (MRI)                  |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| 2\. Muscle atrophy and/or      |       |       |   |       |         |
| increased muscle fat content   |       |       |   |       |         |
| on T1-weighted MRI scanning    |       |       |   |       |         |
| consistent with myositis       |       |       |   |       |         |
+--------------------------------+-------+-------+---+-------+---------+
| 13L. Skin biopsy compatible    |       |       | N | Not   |         |
| with dermatomyositis (or       |       |       | / | perf  |         |
| lupus)                         |       |       | a | ormed |         |
+--------------------------------+-------+-------+---+-------+---------+

**Other features important in making the diagnosis not listed above --
please specify:**

**[\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_]{.underline}**

**[\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_]{.underline}**

Other laboratory features important in making the diagnosis not listed
above -- please specify:

**[\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_]{.underline}**

**Glossary for the**

**INTERNATIONAL myositis CLASSIFICATION CRITERIA PROJECT**

This document is a GLOSSARY to be used for completing the INTERNATIONAL
MYOSITIS CLASSIFICATION CRITERIA PROJECT DATASHEET. Please read this
carefully prior to completing the DATASHEET and refer to it whenever
questions arise as to how to best enter your data.

  ------------------------------- ---------------------------------------
  **Clinical Muscle Variables --  **Definition**
  present at any time during the  
  disease course**                

  *1M. Objective symmetric        Weakness of proximal upper extremities
  weakness, usually progressive,  as defined by manual muscle testing or
  of the proximal upper           other objective strength testing, which
  extremities*                    is present on both sides and is usually
                                  progressive over time

  *2M, Objective shoulder         Weakness of the shoulder abductors as
  abductor weakness*              defined by manual muscle testing or
                                  other objective strength testing

  *3M. Objective elbow flexor     Weakness of the elbow flexors as
  weakness*                       defined by manual muscle testing or
                                  other objective strength testing

  *4M. Objective elbow extensor   Weakness of the elbow extensors as
  weakness*                       defined by manual muscle testing or
                                  other objective strength testing

  *5M. Wrist and finger flexors   Muscle grades for wrist and finger
  are relatively weaker than      flexors are relatively lower than for
  shoulder abductors on the same  shoulder abductors, as defined by
  side*                           manual muscle testing or other
                                  objective strength testing

  *6M. Wrist flexors are          Muscle grades for wrist flexors are
  relatively weaker than wrist    relatively lower than for wrist
  extensors on the same side*     extensors as defined by manual muscle
                                  testing or other objective strength
                                  testing

  *7M. Objective finger flexor    Finger flexor weakness as defined by
  weakness*                       manual muscle testing or other
                                  objective strength testing

  *8M. Objective symmetric        Weakness of proximal lower extremities
  weakness, usually progressive,  as defined by manual muscle testing or
  of the proximal lower           other objective strength testing, which
  extremities*                    is present on both sides and is usually
                                  progressive over time

  *9M. Objective hip flexor       Weakness of the hip flexors as defined
  weakness*                       by manual muscle testing or other
                                  objective strength testing

  *10M. Objective hip abductor    Weakness of the hip abductors as
  weakness*                       defined by manual muscle testing or
                                  other objective strength testing

  *11M. Objective knee extensor   Weakness of the knee extensors as
  weakness*                       defined by manual muscle testing or
                                  other objective strength testing

  12M. Knee extensors are as weak Muscle grades for knee extensors are
  or *relatively* weaker than hip comparable to or weaker than for hip
  girdle muscles on the same side girdle muscles on the same side, as
                                  defined by manual muscle testing or
                                  other objective strength testing

  13M. Objective muscle weakness  Weakness of distal lower extremities as
  of distal lower extremities     defined by manual muscle testing or
                                  other objective strength testing or
                                  functional testing (e.g., ability to
                                  walk on heals or tip toes)

  *14M. Objective axial weakness* Weakness of axial muscles, including
                                  neck flexors and extensors, abdominal
                                  and trunk muscles, as defined by manual
                                  muscle testing or other objective
                                  strength testing

  15M. Objective neck flexor      Weakness of the neck flexors as defined
  weakness                        by manual muscle testing or other
                                  objective strength testing

  *16M. Neck flexors are          Muscle grades for neck flexors are
  relatively weaker than neck     relatively lower than neck extensors as
  extensors*                      defined by manual muscle testing or
                                  other objective strength testing

  *17M. In the legs proximal      Muscle grades for proximal muscles in
  muscles are relatively weaker   the legs are relatively lower than
  than distal muscles*            distal muscles in the legs as defined
                                  by manual muscle testing or other
                                  objective strength testing

  *18M. In the arms proximal      Muscle grades for proximal muscles in
  muscles are relatively weaker   the arms are relatively lower than
  than distal muscles*            distal muscles in the arms as defined
                                  by manual muscle testing or other
                                  objective strength testing

  19M In the legs distal muscles  Distal muscles in the legs are
  are relatively weaker than      relatively weaker than proximal muscles
  proximal muscles                in the legs as defined by manual muscle
                                  testing or other objective strength
                                  testing

  20M In the arms distal muscles  Distal muscles in the arms are
  are relatively weaker than      relatively weaker than proximal muscles
  proximal muscles                in the arms as defined by manual muscle
                                  testing or other objective strength
                                  testing

  21M. Muscle tenderness          Pain in any muscle induced by squeezing
                                  or palpating the muscle

  22M. Muscle atrophy of distal   Objective clinical evidence by physical
  forearms                        exam of decreased distal forearm muscle
                                  mass

  23M. Muscle atrophy of thighs   Objective clinical evidence by physical
                                  exam of decreased thigh muscle mass

  **Skin Variables -- present at  **Definition**
  any time during the disease     
  course**                        

  *1S. Heliotrope rash*           Purple, lilac-colored or erythematous
                                  patches over the eyelids or in a
                                  periorbital distribution, often
                                  associated with periorbital edema

  *2S. Gottron's papules*         Erythematous to violaceous papules over
                                  the extensor surfaces of joints, which
                                  are sometimes scaly. May occur over the
                                  finger joints, elbows, knees, malleoli
                                  and toes.

  *3S. Gottron's sign*            Erythematous to violaceous macules over
                                  the extensor surfaces of joints, which
                                  are not palpable

  *4S. Erythema of the back of    Confluent erythema around the posterior
  the neck and shoulders (Shawl   base of the neck, back and upper
  sign)*                          shoulders, often in the distribution of
                                  a shawl

  *5S. Erythema of the neck       Confluent erythema around the anterior
  (V-sign)*                       base of the neck and the upper chest,
                                  often in the shape of a "V"

  *6S. Periorbital edema*         Swelling around the one or both orbits

  7S. Linear extensor erythema    Erythema specifically located over the
                                  extensor tendon sheaths of the hands,
                                  forearms, feet and/or forelegs

  8S. Calcification               Dystrophic calcium deposits, observed
                                  clinically or by imaging, which
                                  involves the skin, subcutaneous tissue,
                                  fascia or muscle

  9S. Periungual erythema or      Erythema proximal to the nail bed or
  nailfold capillary abnormality  dilatation of periungual capillaries,
                                  which may be accompanied by vessel
                                  dropout or tortuosity, and which is
                                  visible by naked eye examination or
                                  with magnification such as with
                                  otoscopy or by use of the
                                  ophthalmoscope

  10S. Mechanic's hands           Scaling or cracking of the skin over
                                  the lateral or palmar aspects of the
                                  fingers or thumbs

  11S. Photodistributed           Erythema over the face which may be
  violaceous erythema             isolated malar erythema, but may
                                  include more extensive erythema
                                  including periorbital, chin, temporal,
                                  ear and frontal regions

  12S. Raynaud´s phenomenon       Discoloration of fingertips or other
                                  acral areas (two or three colors) to
                                  emotion or cold

  13S. Cuticular overgrowth       Enlargement or overgrowth of the
                                  cuticle onto the nailbed

  14S. Poikiloderma               A fine speckled pattern of
                                  hyperpigmented and hypopigmented
                                  macules interspersed with fine
                                  teleangiectasia and cutaneous atrophy

  **Other Clinical Variables --   **Definition**
  present at any time during the  
  disease course**                

  1O. Family history of           Patient history or documentation that
  autoimmune disease              one or more of the diseases listed in
                                  Appendix A were diagnosed in a blood
                                  relative.

  2O. Family history of muscle    Patient history or documentation that
  disease                         one or more of the diseases listed in
                                  Appendix B were diagnosed in a blood
                                  relative

  3OA. Acute onset (days to 2     Onset and progression, from days to 2
  weeks) of symptoms              weeks, of the first symptoms of the
                                  syndrome to the full disease
                                  presentation

  3OB. Subacute onset (\> 2 weeks Onset and progression, from 2 weeks to
  to ≤ 2 months) of symptoms      2 months, of the first symptoms of the
                                  syndrome to the full disease
                                  presentation

  3OC. Insidious onset of         Onset and progression of the syndrome
  symptoms \> 2 months to years   to the full disease presentation over a
                                  time period of more than 2 months

  4O. History of episodic         Patient report of weakness after
  weakness associated with        exercise or fasting, which is
  exercise or fasting             intermittent, rather than continuous

  *5O. Arthritis*                 Inflammation, including swelling,
                                  warmth, tenderness, and/or redness of
                                  one or more joints detected by physical
                                  exam

  *6O. Polyarthralgia*            Pain in two or more joints reported by
                                  the patient

  7O. Joint contractures          Fixed limitation in the normal range of
                                  motion of joints in the absence of
                                  synovitis excluding reducible
                                  deformities, avascular necrosis and
                                  deforming arthropathy.

  *8O. Unexplained fevers*        Two or more episodes of documented body
                                  temperature of ≥ 38 degrees Celsius
                                  without obvious cause

  9O. Interstitial lung disease   Radiologic (chest x-ray or chest CT
                                  scan) documentation of inflammation or
                                  scarring (fibrosis) of the parenchyma
                                  of the lung

  10O. Dysphagia or esophageal    Difficulty in swallowing or objective
  dysmotility                     evidence of abnormal motility of the
                                  esophagus

  11O. Objective improvement in   Documented increased strength after an
  strength or other disease       adequate glucocorticoid treatment trial
  manifestation after an adequate (definition: corticosteroids: --
  trial of glucocorticoid therapy prednisone ≥0.75-2 mg/kg/day (or
  and/or other immunosuppressive  equivalent)) or after an adequate
  or immune modulating therapy    treatment trial with another form of
  for at least 8 weeks.           immunosuppressive therapy for 8 weeks
                                  (for methotrexate, ≥10 mg/week
                                  (children: ≥0.3 mg/kg/week); for
                                  azathioprine 75 mg/d (or 2 mg/kg/day)
                                  or other (Check all that apply.)
  ------------------------------- ---------------------------------------

+--------------------------------+------------------+------------------+
| **Muscle Biopsy Variables --   | **Definition**   |                  |
| from any biopsy**              |                  |                  |
+--------------------------------+------------------+------------------+
| *1B. Necrosis of type I and    | Necrotic or      |                  |
| type II muscle fibers,         | degenerating     |                  |
| phagocytosis, degeneration of  | fibers appear    |                  |
| myofibers*                     | pale and loose   |                  |
|                                | the              |                  |
|                                | cross-striations |                  |
|                                | associated with  |                  |
|                                | the contractile  |                  |
|                                | apparatus.       |                  |
|                                | Vacuolation, or  |                  |
|                                | myofibrillar     |                  |
|                                | rarefaction may  |                  |
|                                | be seen. They    |                  |
|                                | may be invaded   |                  |
|                                | by macrophages   |                  |
|                                | (Phagocytosis)   |                  |
|                                | and vary in      |                  |
|                                | diameter with    |                  |
|                                | accompanying     |                  |
|                                | mononuclear      |                  |
|                                | infiltrates      |                  |
+--------------------------------+------------------+------------------+
| *2B. Regeneration of           | Fibers with      |                  |
| myofibers*                     | focal basophilia |                  |
|                                | with large       |                  |
|                                | nuclei           |                  |
+--------------------------------+------------------+------------------+
| *3B. Endomysial, infiltration  | *Muscle biopsy   |                  |
| of mononuclear cells (MNCs)    | reveals          |                  |
| surrounding but not invading,  | endomysial       |                  |
| myofibers*                     | mononuclear      |                  |
|                                | cells abutting   |                  |
|                                | the sarcolemma   |                  |
|                                | of otherwise     |                  |
|                                | healthy,         |                  |
|                                | non-necrotic     |                  |
|                                | muscle fibers,   |                  |
|                                | but there is no  |                  |
|                                | clear invasion   |                  |
|                                | of the muscle    |                  |
|                                | fibers*          |                  |
+--------------------------------+------------------+------------------+
| *4B. Non-necrotic fibers       | Muscle biopsy    |                  |
| surrounded and invaded by      | reveals          |                  |
| MNCs*                          | mononuclear      |                  |
|                                | cells            |                  |
|                                | surrounding and  |                  |
|                                | invading         |                  |
|                                | otherwise        |                  |
|                                | healthy,         |                  |
|                                | non-necrotic     |                  |
|                                | muscle fibers.   |                  |
+--------------------------------+------------------+------------------+
| *5B. Perimysial and/or         | Mononuclear      |                  |
| perivascular infiltration of   | cells are        |                  |
| (MNCs)*                        | located in the   |                  |
|                                | perimysium       |                  |
|                                | and/or located   |                  |
|                                | around blood     |                  |
|                                | vessels (in      |                  |
|                                | either           |                  |
|                                | perimysial or    |                  |
|                                | endomysial       |                  |
|                                | vessels).        |                  |
+--------------------------------+------------------+------------------+
| *6B. Perifascicular atrophy*   | Muscle biopsy    |                  |
|                                | reveals several  |                  |
|                                | rows of muscle   |                  |
|                                | fibers which are |                  |
|                                | smaller in the   |                  |
|                                | perifascicular   |                  |
|                                | region than      |                  |
|                                | fibers more      |                  |
|                                | centrally        |                  |
|                                | located.         |                  |
+--------------------------------+------------------+------------------+
| 7B. Vacuolated muscle fibers   | Muscle biopsy    |                  |
|                                | reveals multiple |                  |
|                                | muscle fibers    |                  |
|                                | containing       |                  |
|                                | vacuoles         |                  |
+--------------------------------+------------------+------------------+
| 8B. Rimmed vacuoles            | Rimmed vacuoles  |                  |
|                                | are bluish by    |                  |
|                                | Hematoxylin and  |                  |
|                                | Eosin staining   |                  |
|                                | and reddish by   |                  |
|                                | modified Gomori- |                  |
|                                | Trichrome        |                  |
|                                | stains.          |                  |
+--------------------------------+------------------+------------------+
| 9B. Ragged red fibers, or      | Ragged red       |                  |
| cytochrome C oxidase negative  | fibers: On       |                  |
| fibers                         | modified         |                  |
|                                | G                |                  |
|                                | omori-Trichrome, |                  |
|                                | staining fibers  |                  |
|                                | may appear to    |                  |
|                                | contain cracks   |                  |
|                                | and increased    |                  |
|                                | red stain in the |                  |
|                                | subsarcolemmal   |                  |
|                                | regions. These   |                  |
|                                | fibers may stain |                  |
|                                | intensely blue   |                  |
|                                | with nicotinic   |                  |
|                                | acid adenine     |                  |
|                                | dinucleotide     |                  |
|                                | dehydrogenase    |                  |
|                                | (NADH) or        |                  |
|                                | succinate        |                  |
|                                | dehydrogenase    |                  |
|                                | (SDH) stain or   |                  |
|                                | have absent or   |                  |
|                                | diminished       |                  |
|                                | staining with    |                  |
|                                | cytochrome C     |                  |
|                                | oxidase stain.   |                  |
+--------------------------------+------------------+------------------+
| 10B. Many necrotic muscle      | The major        |                  |
| fibers as the                  | feature of the   |                  |
|                                | biopsy is        |                  |
| predominant feature.           | necrotic muscle  |                  |
| Inflammatory cells are sparse; | fibers. There    |                  |
| perimysial infiltrate is not   | may be           |                  |
| evident.                       | phagocytosis of  |                  |
|                                | necrotic fibers  |                  |
|                                | but otherwise    |                  |
|                                | there is minimal |                  |
|                                | inflammatory     |                  |
|                                | cell infiltrate  |                  |
|                                | evident except   |                  |
|                                | in the vicinity  |                  |
|                                | of necrotic      |                  |
|                                | muscle fibres    |                  |
|                                | and no           |                  |
|                                | perimysial       |                  |
|                                | infiltrate by    |                  |
|                                | routine          |                  |
|                                | histochemistry   |                  |
|                                | (Hematoxylin and |                  |
|                                | Eosin or         |                  |
|                                | Trichrome        |                  |
|                                | stains)          |                  |
+--------------------------------+------------------+------------------+
| 11B Immunohistochemistry       | Yes: No:         |                  |
| stainings available yes/no     |                  |                  |
+--------------------------------+------------------+------------------+
| 12B. MHC Class I antigen       | Immunostaining   |                  |
| present on scattered or more   | reveals          |                  |
| muscle fibers                  | expression of    |                  |
|                                | MHC class I on   |                  |
|                                | the sarcolemma   |                  |
|                                | of scattered or  |                  |
|                                | more generally   |                  |
|                                | on muscle        |                  |
|                                | fibers.          |                  |
+--------------------------------+------------------+------------------+
| 13B. Endomysial CD8+ cells     | Immu             |                  |
| surrounding myofibers with MHC | nohistochemistry |                  |
| Class I expression on          | of the muscle    |                  |
| myofibers                      | biopsy reveals   |                  |
|                                | CD8 + T cells    |                  |
|                                | surrounding      |                  |
|                                | otherwise        |                  |
|                                | healthy,         |                  |
|                                | non-necrotic     |                  |
|                                | muscle fibers    |                  |
|                                | that express MHC |                  |
|                                | class I antigen  |                  |
|                                | on their         |                  |
|                                | sarcolemma.      |                  |
+--------------------------------+------------------+------------------+
| 14B. Membrane attach complex   | Imm              |                  |
| (MAC) depositions on small     | unocytochemistry |                  |
| blood vessels, , or            | demonstrates     |                  |
|                                | deposition of    |                  |
|                                | membrane attack  |                  |
|                                | complex (MAC,    |                  |
|                                | C5b-9) on or     |                  |
|                                | around small     |                  |
|                                | blood vessels.   |                  |
+--------------------------------+------------------+------------------+
| 15B. Reduced capillary density | *Reduced         |                  |
|                                | capillary        |                  |
|                                | density as       |                  |
|                                | appreciated on   |                  |
|                                | quantitative     |                  |
|                                | analysis*        |                  |
+--------------------------------+------------------+------------------+
| 16B MHC-1 expression of        | *MHC-class 1     |                  |
| perifascicular fibers          | expression is    |                  |
|                                | predominant on   |                  |
|                                | perifascicular   |                  |
|                                | muscle fibers*   |                  |
+--------------------------------+------------------+------------------+
| 17B. Electron microscopy       | *Yes: No:*       |                  |
| information available y/n      |                  |                  |
+--------------------------------+------------------+------------------+
| 18B. Tubuloreticular           | *Tubuloreticular |                  |
| inclusions in endothelial      | inclusions are   |                  |
| cells on electron microscopy   | evident in       |                  |
|                                | endothelial      |                  |
|                                | cells on         |                  |
|                                | electron         |                  |
|                                | microscopy*      |                  |
+--------------------------------+------------------+------------------+
| 19B. Intracellular amyloid     | Intracellular    |                  |
| deposits                       | amyloid deposits |                  |
|                                | are evident in   |                  |
|                                | electron         |                  |
|                                | microscopy       |                  |
+--------------------------------+------------------+------------------+
| 20B.15-18 nm tubulofilaments   |                  |                  |
| by electron microscopy (EM)    |                  |                  |
+--------------------------------+------------------+------------------+
| **Laboratory Variables --      | **Definition**   |                  |
| record the highest values      |                  |                  |
| during the disease course**    |                  |                  |
+--------------------------------+------------------+------------------+
| *1L. Serum Creatine kinase     | Please list the  |                  |
| (CK) activity*                 | highest absolute |                  |
|                                | value available  |                  |
|                                | and the upper    |                  |
|                                | limits of normal |                  |
|                                | with units       |                  |
+--------------------------------+------------------+------------------+
| *2L. Serum Lactate             | Please list      |                  |
| dehydrogenase (LDH) activity*  | absolute values  |                  |
|                                | and upper limits |                  |
|                                | of normal with   |                  |
|                                | units            |                  |
+--------------------------------+------------------+------------------+
| *3L. Serum aspartate           | Please list      |                  |
| aminotransferase               | absolute values  |                  |
| (ASAT/AST/SGOT) activity*      | and upper limits |                  |
|                                | of normal with   |                  |
|                                | units            |                  |
+--------------------------------+------------------+------------------+
| *4L. Serum alanine             | Please list      |                  |
| aminotransferase               | absolute values  |                  |
| (ALAT/ALT/SGPT) activity*      | and upper limits |                  |
|                                | of normal with   |                  |
|                                | units            |                  |
+--------------------------------+------------------+------------------+
| 5L. Serum Aldolase activity    | Please list      |                  |
|                                | absolute values  |                  |
|                                | and upper limits |                  |
|                                | of normal with   |                  |
|                                | units            |                  |
+--------------------------------+------------------+------------------+
| *6L. Erythrocyte sedimentation | Please list      |                  |
| rate (ESR)*                    | absolute values  |                  |
|                                | and upper limits |                  |
|                                | of normal with   |                  |
|                                | units            |                  |
+--------------------------------+------------------+------------------+
| *7L. C-reactive protein (CRP)* | Please list      |                  |
|                                | absolute values  |                  |
|                                | and upper limits |                  |
|                                | of normal with   |                  |
|                                | units            |                  |
+--------------------------------+------------------+------------------+
| *8L. **Autoantibody tests      | **Yes:**         | **No:**          |
| available y/n***               |                  |                  |
+--------------------------------+------------------+------------------+
|                                |                  |                  |
| -----------------------------  |                  |                  |
| ---------- ---------- -------- |                  |                  |
|   9L. Autoantibodies           |                  |                  |
|      Positive   Negative   Not |                  |                  |
|                                |                  |                  |
|                         tested |                  |                  |
|                                |                  |                  |
|   ANA                          |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-Jo-1 (anti-His)         |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-Mi-2                    |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-SRP                     |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-Ku                      |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti- PL7                    |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti- PL-12                  |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti PM-Scl                  |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-SSA                     |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-Ro52/SSA                |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-Ro60/SSA                |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-La/SSB                  |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-ribonucleoprotein       |                  |                  |
|                                |                  |                  |
|   (RNP)-70K (U1snRNP)          |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-RNP-A                   |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-RNP-C                   |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-Centromere B (ACA       |                  |                  |
| )                              |                  |                  |
|                                |                  |                  |
|   Anti-Topoisomerase-1/S       |                  |                  |
| cl70,                          |                  |                  |
|                                |                  |                  |
|   Anti-Ribosomal P antig       |                  |                  |
| en                             |                  |                  |
|                                |                  |                  |
|   Anti-Sm                      |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-SmB                     |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-SmD                     |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   RF                           |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Anti-CCP                     |                  |                  |
|                                |                  |                  |
|                                |                  |                  |
|   Other, please specify        |                  |                  |
| below                          |                  |                  |
|                                |                  |                  |
| -----------------------------  |                  |                  |
| ---------- ---------- -------- |                  |                  |
+--------------------------------+------------------+------------------+
| **EMG performed y/n**          | **Yes:**         | **No:**          |
+--------------------------------+------------------+------------------+
| *1. . Electromyogram (EMG) -   | Increased        |                  |
| Increased insertional and      | insertional      |                  |
| spontaneous activity in the    | activity: upon   |                  |
| form of fibrillation           | insertion of the |                  |
| potentials, positive sharp     | EMG needle there |                  |
| waves, or complex repetitive   | are fibrillation |                  |
| discharges*                    | potentials,      |                  |
|                                | positive sharp   |                  |
|                                | waves, or        |                  |
|                                | complex          |                  |
|                                | repetitive       |                  |
|                                | discharges or    |                  |
|                                | myotonic         |                  |
|                                | discharges*.*    |                  |
|                                |                  |                  |
|                                | Increased        |                  |
|                                | spontaneous      |                  |
|                                | activity:        |                  |
|                                | fibrillation     |                  |
|                                | potentials,      |                  |
|                                | positive sharp   |                  |
|                                | waves, complex   |                  |
|                                | repetitive       |                  |
|                                | discharges or    |                  |
|                                | pseudomyotonic   |                  |
|                                | discharges are   |                  |
|                                | seen on needle   |                  |
|                                | EMG even when    |                  |
|                                | the needle is    |                  |
|                                | resting in the   |                  |
|                                | muscle without   |                  |
|                                | further movement |                  |
+--------------------------------+------------------+------------------+
| *2. . EMG - Morphometric       | Analysis of at   |                  |
| analysis reveals the presence  | least 20         |                  |
| of short duration, small       | individual motor |                  |
| amplitude, polyphasic motor    | unit action      |                  |
| unit action potentials         | potentials       |                  |
| (MUAPs)*                       | reveals that the |                  |
|                                | average duration |                  |
|                                | is short,        |                  |
|                                | amplitude is     |                  |
|                                | small, and       |                  |
|                                | phases are       |                  |
|                                | greater than 4.  |                  |
+--------------------------------+------------------+------------------+
| ***MRI performed y/n***        | **Yes:**         | **No:**          |
+--------------------------------+------------------+------------------+
| 1\. Muscle oedema on STIR or   | Increased signal |                  |
| T2-weighted magnetic resonance | in muscle, often |                  |
| imaging (MRI)                  | symmetric, by    |                  |
|                                | short tau        |                  |
|                                | inversion        |                  |
|                                | recovery (STIR)- |                  |
|                                | or T-2 weighted  |                  |
|                                | MRI imaging,     |                  |
|                                | without other    |                  |
|                                | known cause      |                  |
+--------------------------------+------------------+------------------+
| 2\. Muscle atrophy or          | Decreased muscle |                  |
| replacement of muscle by fat   | volume (i.e.,    |                  |
| on T1-weighted MRI scanning    | muscle atrophy)  |                  |
| consistent with myositis       | or increased fat |                  |
|                                | content of       |                  |
|                                | muscle by        |                  |
|                                | T1-weighted MRI  |                  |
|                                | imaging, without |                  |
|                                | other known      |                  |
|                                | cause            |                  |
+--------------------------------+------------------+------------------+
| 13L. Skin biopsy compatible    | Biopsy findings  |                  |
| with dermatomyositis (or       | consistent with  |                  |
| lupus)                         | dermatomyositis  |                  |
|                                | or lupus (these  |                  |
|                                | could include:   |                  |
|                                | intradermal or   |                  |
|                                | perivascular     |                  |
|                                | inflammatory     |                  |
|                                | cell infiltrate, |                  |
|                                | liquefaction,    |                  |
|                                | basal cell       |                  |
|                                | degeneration,    |                  |
|                                | epidermal        |                  |
|                                | atrophy,         |                  |
|                                | hyperkeratosis,  |                  |
|                                | melanin          |                  |
|                                | incontinence,    |                  |
|                                | mucin            |                  |
|                                | deposition)      |                  |
+--------------------------------+------------------+------------------+
| **[Other features important in | Any other        |                  |
| making the diagnosis not       | documented       |                  |
| listed above]{.underline}**    | clinical signs,  |                  |
|                                | symptoms or      |                  |
|                                | laboratory       |                  |
|                                | findings, not    |                  |
|                                | listed above,    |                  |
|                                | that were        |                  |
|                                | important in     |                  |
|                                | diagnosing the   |                  |
|                                | patient          |                  |
+--------------------------------+------------------+------------------+

**Appendix A. List of autoimmune diseases, adult or juvenile onset, to
be considered for family history question 1O.** **Specify in the
comments box.**

-   Addison\'s Disease

-   Alopecia Areata

-   Autoimmune Hemolytic Anemia

-   Autoimmune Thrombocytopenia

-   Autoimmune thyroid diseases - Graves Disease or Hashimoto\'s

-   Behcet\'s Disease

-   Celiac Disease

-   Goodpasture's syndrome

-   Henoch Schonlein Purpura

-   Inflammatory bowel disease - Crohn\'s disease or Ulcerative colitis

-   Mixed connective tissue disease

-   Multiple Sclerosis

-   Myasthenia Gravis

-   Myositis - Polymyositis, Dermatomyositis or Inclusion body myositis

-   Pemphigus

-   Pernicious anemia

-   Polyarteritis nodosa

-   Primary biliary cirrhosis

-   Psoriasis

-   Rheumatoid arthritis or juvenile rheumatoid arthritis

-   Scleroderma (Systemic sclerosis)

-   Sjogren\'s Syndrome

-   Systemic Lupus Erythematosus (Lupus)

-   Thyroiditis

-   Type 1 diabetes

-   Vitiligo

-   Wegener\'s Granulomatosis

-   Other, please specify below

-   None

-   No information available

Other (please specify)
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**Appendix B. List of muscle diseases, adult or juvenile onset, to be
considered for family history question 2O. Specify in the comments
box.**

-   Drug or toxin associated myopathy

-   Endocrine myopathy

-   Infectious myopathy

-   Metabolic myopathy

-   Mitochondrial myopathy

-   Motor neuron disease

-   Muscular Dystrophy

-   No information available

-   None

-   Non-inflammatory inclusion body myopathy

[Other, please specify
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_]{.underline}