Inflammatory myopathy

Inflammatory myopathies are disorders of muscle in which there is clinical and laboratory evidence of an inflammatory process:

These are acquired muscle disorders as opposed to the inherited dystrophies and may be classified as follows:

Polymyositis Adult form

Dermatomyositis Inclusion body myositis

Inflammatory myopathy associated with malignant disease

Inflammatory myopathy associated with collagen vascular disorders, e.g. lupus erythematosus, systemic sclerosis, rheumatoid arthritis

Infective - viral, e.g. Coxsackievirus, echovirus, parasitic: e.g. toxoplasmosis, schistosomiasis.

Drug-induced - penicillamine, cholesterol lowering agents e.g. clofibrate, bezafibrate. POLYMYOSITIS/DERMATOMYOSITIS

There are two principal forms of inflammatory myopathy - polymyositis and dermatomyositis which are separated clinically by the dermatological findings in the latter. All age groups are affected. Annual incidence is 8 per 100000. These disorders are sporadic though familial cases are described.

An autoimmune basis for these disorders is supported by:

- response to immunosuppressive therapy.

- association with other known immunological disorders, e.g. collagen vascular disorders.

- elevated IgG in blood and presence of circulating autoantibodies, e.g. antinuclear antibody in some cases.

an increased incidence of ccrtain histocompatibility antigens (IILA antigens)-B8, DR3.

- the reproduction of a similar disorder in laboratory animals by injection of muscle extract with Freund's adjuvant.

Humoral and cell mediated immune mechanisms seem responsible for these disorders but the trigger factor(s) remain unknown.

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