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24 Cards in this Set

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What are the three steps to approaching a patient with generalized muscle weakness?
1. Distinguish as TRUE muscle weakness

2. Localize site of lesion

3. Determine cause
How do you determine TRUE muscle weakness?
Formal muscle testing
How do you localize muscle lesion site?
EMG/NCV
List three Idiopathic Inflammatory Myopathies (IIM).
1. Polymyositis
2. Dermatomyositis
3. Juevenile DM/PM
What population group is most likely at risk for PM/DM?
Black women
In caucasian patients, what are the genetic components associated with IIM? x3
HLA-DR3

HLA-DR6

HLA-138
What genetic component is associated with an increased risk of development of PM and Juvenile DM
HLA-DR3
What is the diagnostic criteria for PM/DM? x6
Symmetric weakness

Proximal muscle weakness

EMG myopathic changes

Characteristic abnormalities in biopsy

Serum muscle enzymes elevated

Typical rash for DM
In PM/DM, what is the definite test for diagnosis?
Muscle Biopsy
What are the three rules to follow when doing a biopsy?
1. Must be obtained from weak/inflamed muscle

2. Avoid muscles with atrophy

3. Prefer to use Open Needle Biopsy
What are the common pathological features of PM/DM? x4
1. Muscle fiber Degeneration
2. Muscle fiber Regeneration
3. Muscle fiber Necrosis
4. Inflammatory cell infiltrate (lymphocytic)
Which IIM is a humorally mediated disorder?
DM
Which IIM is a cell mediated disorder?
PM
Pathological features specific for DM. x4
Perivascular Inflammation

Perifascicular atrophy/necrosis

B lymphocytes

Increased ratio of CD4/CD8 T-cells
Pathological features specific for PM. x3
Infiltrate within fascicle

CD8+ T cells

NO VASCULOPATHY
PM/DM:

- Symptoms x3
1. Symmetric and Proximal weakness

2. Reflux

3. Aspiration

4. Dysphagia

5. Dysphonia
PM/DM:

- What muscles are spared?
- Pain
Facial and ocular muscles

20-25%
PM/DM:

- Non-muscle related symptoms x4
Fever

Raynaud's

Arthritis

Weight loss
Skin manifestations of DM x2
Gottron's papules

Heliotrope rash
What is pathognomonic for DM?
Gottron's papules
What labs would you expect in PM/DM? x4
Increased CK
Increased Aldolase

Positive ANA
Positive anti-JO-1
Prognosis for PM/DM?
80% to 90% have 15 yr survival rate
In what population group is morbidity/mortality worse?
Black females
What type of patients should be screened for underlying malignancy in association with IIM?
Over 50 y.o. with new onset IIM.