**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}**

**1. 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.

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

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

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

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

**4. Clinician submitting case:**

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

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

**5. Email**
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**6. Postal**
address\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

7\. **Case identifier/number** (Center number + individual case number)
: \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

8\. **Gender**: □ Female

□ Male

**9A**. **Age (years) at onset** of first symptom assumed to be related
to the disease: \_\_\_\_years \_\_\_ months (include years and months
for children)

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

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

□ Of European descent

> □ 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:**

A. Idiopathic Inflammatory Myopathy (IIM), adults or children:

*Myositis Onset: adult □ (diagnosed at age ≥18) or childhood □
(diagnosed at age \< 18 years) □*

(check only one diagnosis):

> □ Polymyositis

□ Dermatomyositis

□ Amyopathic dermatomyositis

□ Hypomyopathic dermatomyositis

□ Inclusion body myositis

□ Immune-mediated necrotizing myopathy

> ***B. NOT*** Idiopathic Inflammatory Myopathy (Not IIM), adults or
> children, but in which the diagnosis of idiopathic myositis was
> considered in the differential diagnosis:

*Disease Onset: adult □ (diagnosed at age ≥18) or childhood □ (diagnosed
at age \< 18 years)*

□ Non-inflammatory inclusion body myopathy

□ Dystrophy, specify diagnosis

□ Metabolic myopathy, specify diagnosis

□ Mitochondrial myopathy, specify diagnosis

□ Drug or toxin associated myopathy, specify diagnosis

□ Infectious myopathy, specify diagnosis

□ Endocrine myopathy, specify diagnosis

□ Motor neuron diseases, please specify diagnosis

□ Other neuromuscular disease, specify diagnosis

□ Other rheumatic disease, specify diagnosis

□ Other dermatologic disease, specify diagnosis

□ Other diagnosis, specify

\_\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\--

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

□ Muscle weakness

□ Muscle biopsy abnormalities

□ Elevated muscle enzymes

□ EMG abnormalities

□ Rashes

□ Skin biopsy

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

G9. Clinician's additional comments on the
case:\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

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

**G10. Other diagnoses in this case:** (check all that apply):

□ Hypothyroidism

□ Hyperthyroidism

□ Type I diabetes

□ Rheumatoid arthritis

□ Juvenile idiopathic arthritis

□ Systemic lupus erythematosus

□ Systemic sclerosis

□ Sjögren´s syndrome

□Mixed connective tissue disease

□ Malignancy

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

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

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

> □ Non applicable

***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  | **[Not    | **[Comme  |
|                              | esent | bsent | availa    | nts]{.und |
|                              | ]{.un | ]{.un | ble]{.und | erline}** |
|                              | derli | derli | erline}** |           |
|                              | ne}** | ne}** |           |           |
+------------------------------+-------+-------+-----------+-----------+
| **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  | **[Not    | **[Comme  |
|                              | esent | bsent | availa    | nts]{.und |
|                              | ]{.un | ]{.un | ble]{.und | erline}** |
|                              | derli | derli | erline}** |           |
|                              | ne}** | ne}** |           |           |
+------------------------------+-------+-------+-----------+-----------+
| **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 neck    |       |       |           |           |
| (V-sign)*                    |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *5S. Erythema of the back of |       |       |           |           |
| neck and shoulders (Shawl    |       |       |           |           |
| 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  | **[Not    | **[Comme  |
|                              | esent | bsent | availa    | nts]{.und |
|                              | ]{.un | ]{.un | ble]{.und | erline}** |
|                              | derli | derli | erline}** |           |
|                              | ne}** | ne}** |           |           |
+------------------------------+-------+-------+-----------+-----------+
| **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)     |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 3O. Acute onset (days to 2   |       |       |           |           |
| weeks) of symptoms           |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 4O. Subacute onset (\> 2     |       |       |           |           |
| weeks to ≤2 months) of       |       |       |           |           |
| symptoms                     |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 5O. Insidious onset of       |       |       |           |           |
| symptoms \> 2 months to      |       |       |           |           |
| years                        |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 6O. History of episodic      |       |       |           |           |
| weakness associated with     |       |       |           |           |
| exercise or fasting          |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *7O. Arthritis*              |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *8O. Polyarthralgia*         |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 9O. Joint contractures       |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| *10O. Unexplained Fevers*    |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 11O. Interstitial lung       |       |       |           |           |
| disease                      |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 12O. Dysphagia or esophageal |       |       |           |           |
| dysmotility                  |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+
| 13O. Objective improvement   |       |       |           |           |
| in strength or other disease |       |       |           |           |
| manifestation after an       |       |       |           |           |
| adequate trial of            |       |       |           |           |
| corticosteroid and/or other  |       |       |           |           |
| immunosuppressive or immune  |       |       |           |           |
| modulating therapy           |       |       |           |           |
+------------------------------+-------+-------+-----------+-----------+

+------------------------------+------+----------+-----------+--------+
|                              | *    | **[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        | *     | *     |           | **[Not  |
| available y/n*                 | *[Pos | *[Neg |           | tested] |
|                                | itive | ative |           | {.under |
|                                | ]{.un | ]{.un |           | line}** |
|                                | derli | derli |           |         |
|                                | ne}** | ne}** |           |         |
+--------------------------------+-------+-------+-----------+---------+
| 9L. *Autoantibodies*           |       |       |           |         |
|                                |       |       |           |         |
| 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/Scl70,    |       |       |           |         |
|                                |       |       |           |         |
| Anti-Ribosomal P antigen       |       |       |           |         |
|                                |       |       |           |         |
| Anti-Sm                        |       |       |           |         |
|                                |       |       |           |         |
| Anti-SmB                       |       |       |           |         |
|                                |       |       |           |         |
| Anti-SmD                       |       |       |           |         |
|                                |       |       |           |         |
| RF                             |       |       |           |         |
|                                |       |       |           |         |
| Anti-CCP                       |       |       |           |         |
|                                |       |       |           |         |
| Other, please specify          |       |       |           |         |
+--------------------------------+-------+-------+-----------+---------+
| .                              |       |       |           |         |
+--------------------------------+-------+-------+-----------+---------+
|                                | **[Pr | **[A  | **[Not    | **[Co   |
|                                | esent | bsent | availa    | mments] |
|                                | ]{.un | ]{.un | ble]{.und | {.under |
|                                | derli | derli | erline}** | line}** |
|                                | ne}** | ne}** |           |         |
+--------------------------------+-------+-------+-----------+---------+
| *EMG performed y/n*            |       |       |           |         |
+--------------------------------+-------+-------+-----------+---------+
| *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   |       |       |           |         |
+--------------------------------+-------+-------+-----------+---------+
| 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    |       |       |           |         |
| with dermatomyositis (or       |       |       |           |         |
| lupus)                         |       |       |           |         |
+--------------------------------+-------+-------+-----------+---------+

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

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

**[\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_]{.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 weaker 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 weaker 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 weaker than neck extensors
  extensors*                      as 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 weaker 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 weaker 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 neck       Confluent erythema around the anterior
  (V-sign)*                       base of the neck and the upper chest,
                                  often in the shape of a "V"

  *5S. 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

  *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

  3O. 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

  4O. 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

  5O. Insidious onset of symptoms Onset and progression of the syndrome
  \> 2 months to years            to the full disease presentation over a
                                  time period of more than 2 months

  6O. History of episodic         Patient report of weakness after
  weakness associated with        exercise or fasting, which is
  exercise or fasting             intermittent, rather than continuous

  *7O. Arthritis*                 Inflammation, including swelling,
                                  warmth, tenderness, and/or redness of
                                  one or more joints detected by physical
                                  exam

  *8O. Polyarthralgia*            Pain in two or more joints reported by
                                  the patient

  9O. 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.

  *10O. Unexplained fevers*       Two or more episodes of documented body
                                  temperature of ≥ 38 degrees Celsius
                                  without obvious cause

  11O. Interstitial lung disease  Radiologic (chest x-ray or chest CT
                                  scan) documentation of inflammation or
                                  scarring (fibrosis) of the parenchyma
                                  of the lung

  12O. Dysphagia or esophageal    Difficulty in swallowing or objective
  dysmotility                     evidence of abnormal motility of the
                                  esophagus

  13O. Objective improvement in   Documented increased strength after an
  strength or other disease       adequate corticosteroid treatment trial
  manifestation after an adequate (definition: corticosteroids: --
  trial of corticosteroid therapy prednisone ≥0.75-2 mg/kg/day (or
  and/or other immunosuppressive  equivalent) for at least 1 month , a)
  or immune modulating therapy    or after an adequate treatment trial
                                  with another form of 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 degenerating fibers     |
| type II muscle fibers,         | appear pale and loose the           |
| phagocytosis, degeneration of  | cross-striations associated with    |
| myofibers*                     | 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 focal basophilia with   |
| myofibers*                     | large nuclei                        |
+--------------------------------+-------------------------------------+
| *3B. Endomysial, infiltration  | *Muscle biopsy reveals endomysial   |
| of mononuclear cells (MNCs)    | mononuclear cells abutting the      |
| surrounding but not invading,  | sarcolemma of otherwise healthy,    |
| myofibers*                     | non-necrotic muscle fibers, but     |
|                                | there is no clear invasion of the   |
|                                | muscle fibers*                      |
+--------------------------------+-------------------------------------+
| *4B. Non-necrotic fibers       | Muscle biopsy reveals mononuclear   |
| surrounded and invaded by      | cells surrounding and invading      |
| MNCs*                          | otherwise healthy, non-necrotic     |
|                                | muscle fibers.                      |
+--------------------------------+-------------------------------------+
| *5B. Perimysial and/or         | Mononuclear cells are located in    |
| perivascular infiltration of   | the perimysium and/or located       |
| (MNCs)*                        | 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 fibers: On modified      |
| cytochrome C oxidase negative  | Gomori-Trichrome, staining fibers   |
| 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 feature of the biopsy is  |
| fibers as the                  | necrotic muscle fibers. There may   |
|                                | be phagocytosis of necrotic fibers  |
| predominant feature.           | but otherwise there is minimal      |
| Inflammatory cells are sparse; | inflammatory cell infiltrate        |
| perimysial infiltrate is not   | evident except in the vicinity of   |
| evident.                       | necrotic muscle fibres and no       |
|                                | perimysial infiltrate by routine    |
|                                | histochemistry (Hematoxylin and     |
|                                | Eosin or Trichrome stains)          |
+--------------------------------+-------------------------------------+
| 11B Immunohistochemistry       |                                     |
| stainings available yes/no     |                                     |
+--------------------------------+-------------------------------------+
| 12B. MHC Class I antigen       | Immunostaining reveals expression   |
| present on scattered or more   | of MHC class I on the sarcolemma of |
| muscle fibers                  | scattered or more generally on      |
|                                | muscle fibers.                      |
+--------------------------------+-------------------------------------+
| 13B. Endomysial CD8+ cells     | Immunohistochemistry of the muscle  |
| surrounding myofibers with MHC | biopsy reveals CD8 + T cells        |
| Class I expression on          | surrounding otherwise healthy,      |
| myofibers                      | non-necrotic muscle fibers that     |
|                                | express MHC class I antigen on      |
|                                | their sarcolemma.                   |
+--------------------------------+-------------------------------------+
| 14B. Membrane attach complex   | Immunocytochemistry demonstrates    |
| (MAC) depositions on small     | deposition of membrane attack       |
| blood vessels, , or            | 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 expression is          |
| perifascicular fibers          | predominant on perifascicular       |
|                                | muscle fibers*                      |
+--------------------------------+-------------------------------------+
| 17B. Electron microscopy       |                                     |
| information available y/n      |                                     |
+--------------------------------+-------------------------------------+
| 18B. Tubuloreticular           | *Tubuloreticular inclusions are     |
| inclusions in endothelial      | evident in endothelial cells on     |
| cells on electron microscopy   | electron microscopy*                |
+--------------------------------+-------------------------------------+
| 19B. Intracellular amyloid     |                                     |
| deposits or 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 highest absolute    |
| (CK) activity*                 | value available and the upper       |
|                                | limits of normal with units         |
+--------------------------------+-------------------------------------+
| *2L. Serum Lactate             | Please list absolute values and     |
| dehydrogenase (LDH) activity*  | upper limits of normal with units   |
+--------------------------------+-------------------------------------+
| *3L. Serum aspartate           | Please list absolute values and     |
| aminotransferase               | upper limits of normal with units   |
| (ASAT/AST/SGOT) activity*      |                                     |
+--------------------------------+-------------------------------------+
| *4L. Serum alanine             | Please list absolute values and     |
| aminotransferase               | upper limits of normal with units   |
| (ALAT/ALT/SGPT) activity*      |                                     |
+--------------------------------+-------------------------------------+
| 5L. Serum Aldolase activity    | Please list absolute values and     |
|                                | upper limits of normal with units   |
+--------------------------------+-------------------------------------+
| *6L. Erythrocyte sedimentation | Please list absolute values and     |
| rate (ESR)*                    | upper limits of normal with units   |
+--------------------------------+-------------------------------------+
| *7L. C-reactive protein (CRP)* | Please list absolute values and     |
|                                | upper limits of normal with units   |
+--------------------------------+-------------------------------------+
| 8L. Autoantibodies             | Autoantibodies tested, check all    |
|                                | that apply. If tested:              |
| ANA                            |                                     |
|                                | specify negative or positive and    |
| Anti-Jo-1 (anti-His)           | when possible which method was      |
|                                | used,                               |
| 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/Scl70,    |                                     |
|                                |                                     |
| Anti-Ribosomal P antigen       |                                     |
|                                |                                     |
| Anti-Sm                        |                                     |
|                                |                                     |
| Anti-SmB                       |                                     |
|                                |                                     |
| Anti-SmD                       |                                     |
|                                |                                     |
| RF                             |                                     |
|                                |                                     |
| Anti-CCP                       |                                     |
|                                |                                     |
| Other, please specify          |                                     |
+--------------------------------+-------------------------------------+
| EMG performed y/n              |                                     |
+--------------------------------+-------------------------------------+
| *1. . Electromyogram (EMG) -   | Increased insertional activity:     |
| Increased insertional and      | upon insertion of the EMG needle    |
| spontaneous activity in the    | there are fibrillation potentials,  |
| form of fibrillation           | positive sharp waves, or complex    |
| potentials, positive sharp     | repetitive discharges or myotonic   |
| waves, or complex repetitive   | discharges*.*                       |
| 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 least 20 individual  |
| analysis reveals the presence  | motor unit action potentials        |
| of short duration, small       | reveals that the average duration   |
| amplitude, polyphasic motor    | is short, amplitude is small, and   |
| unit action potentials         | phases are greater than 4.          |
| (MUAPs)*                       |                                     |
+--------------------------------+-------------------------------------+
| *MRI performed y/n*            |                                     |
+--------------------------------+-------------------------------------+
| 1\. Muscle edema on STIR or    | Increased signal in muscle, often   |
| T2-weighted magnetic resonance | symmetric, by short tau inversion   |
| imaging (MRI)                  | recovery (STIR)- or T-2 weighted    |
|                                | MRI imaging, without other known    |
|                                | cause                               |
+--------------------------------+-------------------------------------+
| 2\. Muscle atrophy or          | Decreased muscle volume (i.e.,      |
| replacement of muscle by fat   | muscle atrophy) or increased fat    |
| on T1-weighted MRI scanning.   | content of muscle by T1-weighted    |
|                                | MRI imaging, without other known    |
|                                | cause                               |
+--------------------------------+-------------------------------------+
| 13L. Skin biopsy compatible    | Biopsy findings consistent with     |
| with dermatomyositis (or       | dermatomyositis or lupus (these     |
| lupus)                         | 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 documented clinical       |
| making the diagnosis not       | signs, symptoms or laboratory       |
| listed above]{.underline}**    | 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**

-   [Autoimmune thyroid
    diseases](http://www.wrongdiagnosis.com/a/autoimmune_thyroid_diseases/intro.htm) -
    [Graves
    Disease](http://www.wrongdiagnosis.com/g/graves_disease/intro.htm)
    or [Hashimoto\'s
    Thyroiditis](http://www.wrongdiagnosis.com/h/hashimotos_thyroiditis/intro.htm)

-   [Type 1 diabetes](http://www.wrongdiagnosis.com/d/diab1/intro.htm)

-   [Rheumatoid
    arthritis](http://www.wrongdiagnosis.com/r/rheumatoid_arthritis/intro.htm)
    or [juvenile rheumatoid arthritis]{.underline}

-   [Systemic Lupus
    Erythematosus](http://www.wrongdiagnosis.com/l/lupus/intro.htm)
    (Lupus)

-   [Multiple
    Sclerosis](http://www.wrongdiagnosis.com/m/multiple_sclerosis/intro.htm)

-   Inflammatory bowel disease - [Crohn\'s
    disease](http://www.wrongdiagnosis.com/c/crohns_disease/intro.htm)
    or [Ulcerative colitis]{.underline}

-   [Psoriasis](http://www.wrongdiagnosis.com/p/psoriasis/intro.htm)

-   [Sjogren\'s
    Syndrome](http://www.wrongdiagnosis.com/s/sjogrens_syndrome/intro.htm)

-   [Scleroderma](http://www.wrongdiagnosis.com/s/scleroderma/intro.htm)
    (Systemic sclerosis)

-   [Addison\'s
    Disease](http://www.wrongdiagnosis.com/a/addisons_disease/intro.htm)

-   [Pernicious
    anemia](http://www.wrongdiagnosis.com/p/pernicious_anemia/intro.htm)

-   [Myasthenia
    Gravis](http://www.wrongdiagnosis.com/m/myasthenia_gravis/intro.htm)

-   [Primary biliary
    cirrhosis](http://www.wrongdiagnosis.com/p/primary_biliary_cirrhosis/intro.htm)

-   [Mixed connective tissue
    disease](http://www.wrongdiagnosis.com/m/mixed_connective_tissue_disease/intro.htm)

-   [Vitiligo](http://www.wrongdiagnosis.com/v/vitiligo/intro.htm)

-   [Autoimmune Hemolytic
    Anemia](http://www.wrongdiagnosis.com/a/autoimmune_hemolytic_anemia/intro.htm)

-   [Autoimmune
    Thrombocytopenia](http://www.wrongdiagnosis.com/a/autoimmune_thrombocytopenia/intro.htm)

-   [Celiac
    Disease](http://www.wrongdiagnosis.com/c/celiac_disease/intro.htm)

-   [Pemphigus](http://www.wrongdiagnosis.com/p/pemphigus/intro.htm)

-   [Alopecia
    Areata](http://www.wrongdiagnosis.com/a/alopecia_areata/intro.htm)

-   [Behcet\'s
    Disease](http://www.wrongdiagnosis.com/b/behcets_disease/intro.htm)

-   Myositis - [Polymyositis]{.underline},
    [Dermatomyositis](http://www.wrongdiagnosis.com/d/dermatomyositis/intro.htm)
    or [Inclusion body myositis]{.underline}

-   [Goodpasture's
    syndrome](http://www.wrongdiagnosis.com/g/goodpasture_syndrome/intro.htm)

-   [Wegener\'s Granulomatosis]{.underline}

-   [Polyarteritis nodosa]{.underline}

-   [Henoch Schonlein Purpura.]{.underline}

-   [None]{.underline}

-   [Unknown]{.underline}

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

-   Non-inflammatory inclusion body myopathy

-   Muscular Dystrophy

-   Metabolic myopathy

-   Mitochondrial myopathy

-   Drug or toxin associated myopathy

-   Infectious myopathy

-   Endocrine myopathy

-   Motor neuron disease

-   [None]{.underline}

-   [Unknown]{.underline}

-   

> Appendix C. Autoantibodies

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/Scl70,

Anti-Ribosomal P antigen

Anti-Sm

Anti-SmB

Anti-SmD

RF

Anti-CCP

Other, please specify