Steroids - Prednisolone 40-80 mg daily in divided doses with gradual reduction to maintenance (alternate day) dose once improved. If stopped too early, relapse may occur. Cimetidine protects against the risk of gastrointestinal haemorrhage or perforation. In refractory cases immunosuppressive drugs - methotrexate, azathioprine, cyclophosphamide, cyclosporin or high dose intravenous immunoglobulin - may be used. Outcome
Mortality is now low though only 10% recover completely. In the rest, the disease becomes inactive after 2 years and patients are left with varying degrees of disability. When associated with collagen disease, eventual outcome will depend on the nature of that disease. When associated with neoplasm, steroids may cause temporary improvement. Removal of an associated tumour can result in remission.
Prior to the availability of treatment the outcome in inflammatory myopathy was variable and obviously influenced by the presence of associated neoplasia or collagenosis. Periods of relative improvement could occur but generally progression prevailed. Death occurred as a result of respiratory failure, gastrointestinal haemorrhage, perforation and cardiac arrest.
POLYMYOSITIS AND DERMATOMYOSITIS ASSOCIATED WITH MALIGNANT DISEASES
Approximately 10% of adults with inflammatory myopathy have underlying neoplasia usually carcinoma. In dermatomyositis, of those over 40 years of age as many as 60% harbour neoplasia. Neoplasia may present before or after the development of inflammatory myopathy.
POLYMYOSITIS AND DERMATOMYOSITIS ASSOCIATED WITH COLLAGEN VASCULAR
Approximately 15% of adults with inflammatory myopathy have symptoms and signs of an associated collagen vascular disorder.
In 5-10% of persons with these disorders (systemic lupus erythematosus etc), inflammatory myopathy develops at some stage in their illness.
In the 'overlap' syndromes (mixed collagen vascular diseases) muscle involvement is more common. INCLUSION BODY MYOSITIS
Present after age 50 yrs. Patchy and asymetric in distribution.
Muscle biopsy shows basophilic inclusion granules. Often clinically confused with polymyositis but response to immunotherapy is poor.
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