## CASE EXAMPLES FOR THE MYOSITIS DISEASE DAMAGE ASSESSMENT EXERCISE IN LONDON

####### USE OF THE MYOSITIS DAMAGE INDEX (MDI)

The purpose of this exercise is to provide some guidelines in scoring
these instruments. The scores provided in 3 cases are suggestions for
your consideration, but we realize there may be some differences of
opinion. These cases particularly focus on scoring of the muscle systems
and global activity/damage VAS scores, although the discussion presents
the scoring of other involved organ systems as well.

Prepared by Fred Miller, Lisa Rider, Clarissa Pilkington, and David
Isenberg - May 2002

# 

#### **Disease Damage Assessments**

## Case 4 - PM at follow-up

A 52 y.o. Asian woman had an insidious onset of weakness, which was
likely undiagnosed for many years after onset. She underwent a full
evaluation, which resulted in a diagnosis of definite polymyositis. She
refused treatment with corticosteroids or other therapies but took
herbal remedies for an additional 3 years.

Two years ago, when her muscles were very painful, she was confined to a
wheelchair and unable to swallow solids. Her family convinced her to
seek medical treatment and she was found to have very active
polymyositis. She received intravenous pulse solumedrol and was begun on
high dose oral prednisone in divided doses and methotrexate therapy.

While on high dose oral prednisone, she developed brittle
insulin-dependent diabetes that never resolved, aseptic necrosis of the
left hip requiring joint replacement, and myocarditis with subsequent
congestive heart failure, which also limits her function. She never
regained much strength and at first was confined to a wheelchair, but
has been bed-bound for a year, requiring assistance for most activities
of daily living (HAQ = 2.8). Her myalgias resolved, but her swallowing
continued to be a worsening problem, eventually requiring a nasogastric
feeding tube to prevent aspiration. She develops severe shortness of
breath with any activity, and sometimes at rest, requiring intermittent
nasal oxygen. On exam she had severe muscle atrophy of all limbs and
4-6/10 strength (MMT8 = 38 out of 80). Her CPK is 65 (normal range
15-250 U/l), serum creatinine is 0.4 (normal range 0.7 -- 1.2 mg/dl),
and the rest of her laboratory testing, including CXR, is normal. She is
essentially unchanged from 6 months ago.

**MYOSITIS DISEASE ACTIVITY and DAMAGE ASSESSMENT TOOL VALUES**

##### Proposed Muscle Disease Activity Assessments: VAS = 0 cm

+--------+---------------------------------+---------------------------+
| ###### | **(Absent) (Maximum)**          | ###### Ma                 |
| # [MUS |                                 | ximum Value Guidelines (E |
| CLE DI |                                 | xamples of maximal score) |
| SEASE] |                                 |                           |
| {.unde |                                 | Severe muscle weakness    |
| rline} |                                 | resulting in being bed    |
|        |                                 | bound and an inability to |
| ###### |                                 | perform self care         |
| # [ACT |                                 |                           |
| IVITY] |                                 |                           |
| {.unde |                                 |                           |
| rline} |                                 |                           |
+--------+---------------------------------+---------------------------+

29\. Myositis:

a)  Resulting overall in major loss of function **[OR]{.underline}**
    ***[0]{.underline}*** 1 2 3 4 NA

b)  Resulting overall in moderate loss of function **[OR]{.underline}**
    ***[0]{.underline}*** 1 2 3 4 NA

c)  Resulting overall in mild loss of function ***[0]{.underline}*** 1 2
    3 4 NA

```{=html}
<!-- -->
```
30. Myalgia ***[0]{.underline}*** 1 2 3 4 NA

**Proposed Global Disease Activity Assessment: VAS = 0 cm.**

+------+------------------------------------+-------------------------+
| GL   | **(Absent) (Maximum)**             | Overall evaluation for  |
| OBAL |                                    | the totality of disease |
| DIS  |                                    | activity in ALL         |
| EASE |                                    | systems, (**INCLUDING   |
|      |                                    | MUSCLE DISEASE          |
| #### |                                    | ACTIVITY)**             |
| ###  |                                    |                         |
| [GLO |                                    |                         |
| BAL  |                                    |                         |
| DISE |                                    |                         |
| ASE] |                                    |                         |
| {.un |                                    |                         |
| derl |                                    |                         |
| ine} |                                    |                         |
|      |                                    |                         |
| ##   |                                    |                         |
| #### |                                    |                         |
| # [A |                                    |                         |
| CTIV |                                    |                         |
| ITY] |                                    |                         |
| {.un |                                    |                         |
| derl |                                    |                         |
| ine} |                                    |                         |
|      |                                    |                         |
| #### |                                    |                         |
| ###  |                                    |                         |
+------+------------------------------------+-------------------------+

Proposed Myositis Damage Index (MDI) Muscle Damage Assessment VAS = 10
cm

+-------+---------------------------------+---------------------------+
| ###   | **(Absent) (Maximum)**          | ###### Ma                 |
| ####  |                                 | ximum Value Guidelines (E |
| [MUSC |                                 | xamples of maximal score) |
| LE]{. |                                 |                           |
| under |                                 | Severe muscle atrophy or  |
| line} |                                 | weakness resulting in     |
|       |                                 | being bed bound and an    |
| **[D  |                                 | inability to perform self |
| AMAGE |                                 | care                      |
| ]{.un |                                 |                           |
| derli |                                 |                           |
| ne}** |                                 |                           |
+-------+---------------------------------+---------------------------+

1\. Muscle atrophy (clinical) 0 ***[1]{.underline}*** NA

2\. Muscle weakness not attributable to active muscle disease 0
***[1]{.underline}*** NA

######### 3. Muscle dysfunction: decrease in aerobic exercise capacity 0 1 **[NA]{.underline}** 

######### 

######### 4. Muscle atrophy assessed by radiographic methods 0 1 **[NA]{.underline}** 

######### 5. Low serum creatinine 0 **[1]{.underline}** NA

Creatinine value = \_\_0.4\_ mg/dL or umol/L; lower limit normal value =
\_\_0.7\_\_mg/dL or umol/L

**Proposed Myositis Damage Index (MDI) Global Damage Assessment: VAS = 9
cm.**

+------+---------------------------------+----------------------------+
| ###  | **(Absent) (Maximum)**          | ######                     |
| #### |                                 | Maximum Value Guidelines ( |
|  [GL |                                 | Examples of maximal score) |
| OBAL |                                 |                            |
|  DAM |                                 | None                       |
| AGE] |                                 |                            |
| {.un |                                 |                            |
| derl |                                 |                            |
| ine} |                                 |                            |
+------+---------------------------------+----------------------------+

**Discussion of Case 4:** The patient has no evidence of disease
activity, but has severe damage of many organ systems. She has severe
muscle damage resulting in fixed muscle weakness with extensive atrophy,
severe functional impairment and a bed-bound state (MDI Muscle VAS = 10
cm). Other damaged organ systems include: skeletal with avascular
necrosis requiring hip replacement (MDI Skeletal VAS = 9 cm); GI system
requiring a nasogastric tube (MDI GI VAS = 8 cm); pulmonary system with
impaired respiratory musculature (MDI Pulmonary VAS = 8 cm); cardiac
involvement with congestive heart failure and functional impairment (MDI
Cardiovascular VAS = 7 cm) and endocrine system involvement with
steroid-induced, brittle insulin-dependent diabetes (MDI Endocrine VAS =
6 cm). Her extremely limited function (HAQ = 2.8 out of 3.0 maximum) and
multiple systems with high degree of damage place her MDI Global Damage
score at 9 cm, but her ability to breath and swallow without external
support systems keep her from attaining a MDI Global Damage VAS score of
10 cm.

# 

## 

### [Case 5 -- PM at follow-up]{.underline}

A 69-year-old Latino male presents with a vague history of 2-3 years of
increasing fatigue with muscle weakness in the upper and lower
extremities, unexpected falling and slowly increasing dysphagia with
solid but not liquid foods. He denies alcohol or drug use, muscle pain
and weight loss. His past medical history is unremarkable and family
history is negative for any neuromuscular or autoimmune diseases except
for SLE in a younger sister. He was seen by you a year ago and a
detailed workup then only revealed muscle weakness, proximal \> distal,
in the 6-7/10 range of the upper and lower extremities (MMT8 = 52 out of
80) and moderate atrophy of the shoulder, arm and thigh muscles. His HAQ
was 2.0 and he reportedly was unable to climb stairs without assistance
and could not keep up with family members when walking. Laboratories
were remarkable for a CK of 325 (normal range 20 to 250) with 100% MM, a
creatinine of 0.8 (normal 0.9-1.4 mg/dl) and a positive ANA at 1:640,
speckled pattern. All of his other extensive laboratory and autoantibody
tests were negative or unremarkable. A barium swallow examination showed
moderate esophageal dysfunction but no evidence of aspiration. His CXR
and EKG were normal, but his EMG showed evidence of active muscle
inflammation in the upper and lower extremities. A thigh muscle biopsy
showed variation in myofiber size, degeneration and regeneration, rare
scattered endomysial mononuclear cell infiltrations, fatty replacement
of myofibers and increased interstitial spaces, but no evidence of
inclusion bodies or other abnormalities. An extensive cancer work up was
negative. You suggested he begin using a cane for ambulation to minimize
the risk of falling.

A year ago, he initially refused all therapy, especially corticosteroids
given his sister's negative experience with them, but after further
explanations, finally agreed to take 40 mg prednisone /d and was told to
return in 1 month for follow up; he returned 6 months later with a
normal CK but no change in muscle strength or swallowing. You begin a
slow steroid taper over the next 6 months to no prednisone today and he
remains completely unchanged in all respects from your initial
evaluation 12 months ago except for normalization of all muscle enzymes
and a creatinine of 0.7 (normal 0.9-1.4 mg/dl).

**MYOSITIS DISEASE ACTIVITY and DAMAGE ASSESSMENT TOOL VALUES**

##### Muscle Disease Activity Assessments: VAS = 0 cm

+--------+---------------------------------+---------------------------+
| ###### | **(Absent) (Maximum)**          | ###### Ma                 |
| # [MUS |                                 | ximum Value Guidelines (E |
| CLE DI |                                 | xamples of maximal score) |
| SEASE] |                                 |                           |
| {.unde |                                 | Severe muscle weakness    |
| rline} |                                 | resulting in being bed    |
|        |                                 | bound and an inability to |
| ###### |                                 | perform self care         |
| # [ACT |                                 |                           |
| IVITY] |                                 |                           |
| {.unde |                                 |                           |
| rline} |                                 |                           |
+--------+---------------------------------+---------------------------+

29\. Myositis:

d)  Resulting overall in major loss of function **[OR]{.underline}**
    ***[0]{.underline}*** 1 2 3 4 NA

e)  Resulting overall in moderate loss of function **[OR]{.underline}**
    ***[0]{.underline}*** 1 2 3 4 NA

f)  Resulting overall in mild loss of function ***[0]{.underline}*** 1 2
    3 4 NA

```{=html}
<!-- -->
```
31. Myalgia ***[0]{.underline}*** 1 2 3 4 NA

### 

**Global Disease Activity Assessment: VAS = 0 cm**

+------+------------------------------------+-------------------------+
| GL   | **(Absent) (Maximum)**             | Overall evaluation for  |
| OBAL |                                    | the totality of disease |
| DIS  |                                    | activity in ALL         |
| EASE |                                    | systems, (**INCLUDING   |
|      |                                    | MUSCLE DISEASE          |
| #### |                                    | ACTIVITY)**             |
| ###  |                                    |                         |
| [GLO |                                    |                         |
| BAL  |                                    |                         |
| DISE |                                    |                         |
| ASE] |                                    |                         |
| {.un |                                    |                         |
| derl |                                    |                         |
| ine} |                                    |                         |
|      |                                    |                         |
| ##   |                                    |                         |
| #### |                                    |                         |
| # [A |                                    |                         |
| CTIV |                                    |                         |
| ITY] |                                    |                         |
| {.un |                                    |                         |
| derl |                                    |                         |
| ine} |                                    |                         |
|      |                                    |                         |
| #### |                                    |                         |
| ###  |                                    |                         |
+------+------------------------------------+-------------------------+

Proposed Myositis Damage Index (MDI) Muscle Damage Assessment VAS = 6 cm

+-------+---------------------------------+---------------------------+
| ###   | **(Absent) (Maximum)**          | ###### Ma                 |
| ####  |                                 | ximum Value Guidelines (E |
| [MUSC |                                 | xamples of maximal score) |
| LE]{. |                                 |                           |
| under |                                 | Severe muscle atrophy or  |
| line} |                                 | weakness resulting in     |
|       |                                 | being bed bound and an    |
| **[D  |                                 | inability to perform self |
| AMAGE |                                 | care                      |
| ]{.un |                                 |                           |
| derli |                                 |                           |
| ne}** |                                 |                           |
+-------+---------------------------------+---------------------------+

1\. Muscle atrophy (clinical) 0 ***[1]{.underline}*** NA

2\. Muscle weakness not attributable to active muscle disease 0
***[1]{.underline}*** NA

######### 3. Muscle dysfunction: decrease in aerobic exercise capacity 0 **[1]{.underline}** NA 

######### 

######### 4. Muscle atrophy assessed by radiographic methods 0 1 **[NA]{.underline}** 

######### 5. Low serum creatinine 0 **[1]{.underline}** NA

Creatinine value = \_\_0.7\_ mg/dL or umol/L; lower limit normal value =
\_\_0.9\_\_mg/dL or umol/L

### MDI Global Damage Assessment: VAS = 5 cm 

+------+---------------------------------+----------------------------+
| ###  | **(Absent) (Maximum)**          | ######                     |
| #### |                                 | Maximum Value Guidelines ( |
|  [GL |                                 | Examples of maximal score) |
| OBAL |                                 |                            |
|  DAM |                                 | None                       |
| AGE] |                                 |                            |
| {.un |                                 |                            |
| derl |                                 |                            |
| ine} |                                 |                            |
+------+---------------------------------+----------------------------+

**Discussion of Case 5:** The patient has a clinical picture suggestive
of, but not diagnostic of, inclusion body myositis with initial moderate
muscle disease activity, which resolves with steroid therapy (not clear
that it resolved) and some GI involvement. He has moderate muscle
disease damage with fixed muscle atrophy, residual moderate muscle
weakness, aerobic exercise limitations, functional impairment and
decreased creatinine with no evidence of active myositis (MDI muscle
damage VAS = 6 cm). His dysphagia is also fixed and represents a MDI GI
VAS score of 5 cm. No other organ systems are affected with disease
damage. His global MDI damage score is 5 cm.

# 

## Case 6 -- JDM at follow-up

During the one-and-a-half years after Case 3 presented, the child
received oral corticosteroids, photoprotection and physical therapy and
was treated as an outpatient; no additional therapy was needed.
Prednisone was eventually tapered and discontinued because she developed
moderate transient hypertension requiring treatment and bilateral
cataract formation, which impaired her vision. Her strength showed
gradual improvement but she complains of not being back to normal,
having difficulty keeping up with classmates at recess, and of getting
tired more easily than before her illness started.

Today she appears well with normal vital signs; there are mild atrophic
skin changes over the 2nd and 3rd knuckles, a mild heliotrope rash and
slightly enlarged periungual capillaries and 2 small superficial plaques
of calcinosis over her elbows. Abdominal strength is grade 8/10, neck
and arm flexors have mild atrophy and are grade 8/10, but strength is
otherwise normal (MMT8=76 out of 80). CK is 110 (normal 20 to 215 U/l),
and aldolase is 4 (normal 1 to 9 U/l), and creatinine is 0.7 (normal 0.7
-- 1.2 mg/dl). Essentially, she is unchanged from 6 months ago.

**MYOSITIS DISEASE ACTIVITY ASSESSMENT TOOL VALUES**

Proposed Muscle Disease Activity Assessments: VAS = 0 cm

+--------+---------------------------------+---------------------------+
| ###### | **(Absent) (Maximum)**          | ###### Ma                 |
| # [MUS |                                 | ximum Value Guidelines (E |
| CLE DI |                                 | xamples of maximal score) |
| SEASE] |                                 |                           |
| {.unde |                                 | Severe muscle weakness    |
| rline} |                                 | resulting in being bed    |
|        |                                 | bound and an inability to |
| ###### |                                 | perform self care         |
| # [ACT |                                 |                           |
| IVITY] |                                 |                           |
| {.unde |                                 |                           |
| rline} |                                 |                           |
+--------+---------------------------------+---------------------------+

29\. Myositis:

g)  Resulting overall in major loss of function **[OR]{.underline}**
    ***[0]{.underline}*** 1 2 3 4 NA

h)  Resulting overall in moderate loss of function **[OR]{.underline}**
    ***[0]{.underline}*** 1 2 3 4 NA

i)  Resulting overall in mild loss of function ***[0]{.underline}*** 1 2
    3 4 NA

```{=html}
<!-- -->
```
32. Myalgia ***[0]{.underline}*** 1 2 3 4 NA

Proposed Global Disease Activity Assessment: VAS = 1 cm.

+------+------------------------------------+-------------------------+
| GL   | **(Absent) (Maximum)**             | Overall evaluation for  |
| OBAL |                                    | the totality of disease |
| DIS  |                                    | activity in ALL         |
| EASE |                                    | systems, (**INCLUDING   |
|      |                                    | MUSCLE DISEASE          |
| #### |                                    | ACTIVITY)**             |
| ###  |                                    |                         |
| [GLO |                                    |                         |
| BAL  |                                    |                         |
| DISE |                                    |                         |
| ASE] |                                    |                         |
| {.un |                                    |                         |
| derl |                                    |                         |
| ine} |                                    |                         |
|      |                                    |                         |
| ##   |                                    |                         |
| #### |                                    |                         |
| # [A |                                    |                         |
| CTIV |                                    |                         |
| ITY] |                                    |                         |
| {.un |                                    |                         |
| derl |                                    |                         |
| ine} |                                    |                         |
|      |                                    |                         |
| #### |                                    |                         |
| ###  |                                    |                         |
+------+------------------------------------+-------------------------+

Proposed Myositis Damage Index (MDI) Muscle Damage Assessment VAS = 2 cm

+-------+---------------------------------+---------------------------+
| ###   | **(Absent) (Maximum)**          | ###### Ma                 |
| ####  |                                 | ximum Value Guidelines (E |
| [MUSC |                                 | xamples of maximal score) |
| LE]{. |                                 |                           |
| under |                                 | Severe muscle atrophy or  |
| line} |                                 | weakness resulting in     |
|       |                                 | being bed bound and an    |
| **[D  |                                 | inability to perform self |
| AMAGE |                                 | care                      |
| ]{.un |                                 |                           |
| derli |                                 |                           |
| ne}** |                                 |                           |
+-------+---------------------------------+---------------------------+

1\. Muscle atrophy (clinical) 0 ***[1]{.underline}*** NA

2\. Muscle weakness not attributable to active muscle disease 0
***[1]{.underline}*** NA

######### 3. Muscle dysfunction: decrease in aerobic exercise capacity 0 **[1]{.underline}** NA 

######### 

######### 4. Muscle atrophy assessed by radiographic methods 0 1 **[NA]{.underline}** 

######### 5. Low serum creatinine **[0]{.underline}** 1 NA

Creatinine value = \_\_0.7\_ mg/dL or umol/L; lower limit normal value =
\_\_0.7\_\_mg/dL or umol/L

**Proposed MDI Global Damage Assessment: VAS = 2 cm.**

+------+---------------------------------+----------------------------+
| ###  | **(Absent) (Maximum)**          | ######                     |
| #### |                                 | Maximum Value Guidelines ( |
|  [GL |                                 | Examples of maximal score) |
| OBAL |                                 |                            |
|  DAM |                                 | None                       |
| AGE] |                                 |                            |
| {.un |                                 |                            |
| derl |                                 |                            |
| ine} |                                 |                            |
+------+---------------------------------+----------------------------+

**Discussion of Case 6:** One-and-a-half years later her disease is
improved, with normalization of muscle enzymes and without evidence of
muscle disease activity (Muscle disease VAS = 0 cm), but there is mildly
active skin disease based upon the finding of a persistent Heliotrope
rash and periungual capillary changes (Cutaneous numerical score = 2
\[feature is the same\], MITAX Category B \[requiring low dose oral or
topical steroids or hydroxychloroquine\]); Cutaenous VAS activity = 2
cm; and continued mild fatigue (Constitutional numerical score = 2,
MITAX Category C; constitutional VAS activity = 1 cm. Her Global disease
activity VAS = 1 cm).

Evidence of damage is present. She has stable, persistent muscle
weakness with atrophy, as well as aerobic exercise impairment, without
other known cause (such as steroid myopathy) and no evidence of muscle
disease activity (MDI Muscle VAS = 2 cm). Regarding her skin, she has
atrophic Gottron's lesions from prior active disease and several
superficial plaques of calcinosis (MDI Cutaneous VAS = 2 cm). She also
has bilateral cataract formation with impaired vision (MDI Ocular VAS =
5 cm). She has no other evidence of damage, giving her a MDI Global
Damage Assessment VAS = 2 cm.

#