RHEUMATOLOGY

Polymyositis and Dermatomyositis

• Inflammatory striated myopathies
• Proximal muscle weakness (can cause dysphagia, dysphonia, resp weakness)
• Increased risk of malignancies, esp dermatomyositis

Criteria for Diagnosis

• Dermatomyositis: 1 / 3 skin lesions and 4 / 8 myopathic features
• Polymyositis: no skin lesions and 4 / 8 myopathic features

Skin lesions
• Heliotrope rash (iliac-purple rash on eyelids, often with edema)
• Gottron sign (roughened red papules over knuckles, elbows and knees)
• Shawl sign (macular rash over back and shoulder)

Myopathic features
• Proximal muscle weakness
• Non-destructive arthritis, arthralgias
• Elevated muscle enzymes (ALT, CK)
• Muscle pain on gripping/spontaneous
• EMG: fibrillation potentials
• Positive anti-Jo1 antibody/anti-Mi-2
• Systemic inflammatory signs (fever, increased CRP, ESR)
• Pathologic findings of inflammatory myositis (inflammatory infiltration of striated muscles)

Other features
• Mechanic’s hands: painful rough skin crackling affecting the tips and lateral aspects of the fingers
• Raynaud’s
• Interstitial lung fibrosis

Differentials
• Other myopathies (carcinomatous myopathy, inclusion-body myositis, muscular dystrophies)
• Myolysis (rhabdomyolysis)
• Infection (e.g. HIV)
• Drugs (e.g. penicillamine, colchicine, statin, chloroquine)

Management
• First line: screen for malignancy and prednisolone
o The cancers parallel the frequencies of malignancies in the population. Common
malignancies include lung, breast and colorectal. Lancet 2003 recommends annual CXR,
CT abdo-pelvis, breast mammogram and flex sigmoidoscopy.
• Second line: immunosuppressants e.g. azathioprine, methotrexate, cyclophosphamide, ciclosporin

Questions

Classification of polymyositis-dermatomyositis
I. Primary idiopathic polymyositis
II. Primary idiopathic dermatomyositis
III. Dermatomyositis with associated with neoplasm
IV. Childhood dermatomyositis associated with vasculitis
V. Polymyositis with associated collagen vascular disease

Overlap syndrome
• Dermatomyositis overlaps with systemic sclerosis and mixed connective tissue disease.
• Dermatomyositis and systemic sclerosis have specific antinuclear autoantibody (anti-PM/Sc1)
Causes of Proximal Muscle Weakness
1. Myopathic 2. NMJ disorder 3. Neurogenic

Causes of Myopathy
A. Hereditary muscular dystrophies
1. Duchenne’s (X-linked recessive, pseudohypertrophy of calves, Gower’s sign, cardiac and
respiratory failure)
2. Becker’s (X-linked recessive, similar presentation as DMD but slower and less severe onset
and progression)
3. Limb-girdle (AR, shoulder and pelvic girdle, third decade onset)
4. Fascioscapulohumeral dystrophy (FSHD, AD, facial and pectoral girdle weakness of
hypertrophy of the deltoid)
5. Dystrophia myotonica (AD)
B. Acquired myopathies (PACE PODS)
1. polymyositis or dermatomyositis
2. alcohol
3. carcinoma
4. endocrine e.g. hypo/hyperthyroid, Cushing’s, acromegaly, hypopituitarism
5. periodic paralysis (hypo/hyper/normokalemia)
6. osteomalacia
7. drugs (clofibrate, steroids, chloroquine)
8. sarcoidosis

Causes of NMJ disorder

Myasthenia gravis

Neurogenic disorder
Kugelberg-Welander disease (AR, proximal muscle wasting and fasciculation as a result of anterior horn cell
damage), motor neuron disease, polyradiculopathy