Research Article| Volume 18, ISSUE 1, P1-9, January 1973

Granulomatous polymyositis

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      An account is given of a pathological condition, more common in women, which presents clinically as a slowly progressive muscular disorder of adult life.
      The essential histological lesion is an infiltration of the connective tissue sheaths of voluntary muscle by non-specific granulomas composed of epithelioid cells, histiocytes, multinucleated giant cells and lymphocytes. The granulomas are non-caseating and show an outer zone of fibrosis and an inner fine reticulin network. Special stains have failed to detect bacterial, parasitic or fungal agents.
      Twenty-four cases of chronic granulomatous polymyositis have been previously reported in the literature. Post-mortem findings have, so far, been described in only 1 of these cases. The clinical, electromyographic and pathological findings in a further case are presented. Light-microscopic observations revealed granulomatous lesions in biopsy specimens of deltoid and peroneus brevis muscles. The cytoplasm of many of the multinucleated giant cells in the granulomas contained large amounts of acid mucopolysaccharide. Although there was atrophy of muscle fibres in the vicinity of the lesions, the structure of the fibres was preserved and there was no evidence of abnormal cellular infiltrates in the sarcoplasm. Muscle fibres remote from the lesions showed no abnormalities. The intramuscular nerves showed branching of the subterminal axons, beaded axons, occasional degenerate motor end-plates and large numbers of spherical axonal swellings in the intramuscular nerve bundles.
      Histochemical studies were incomplete, but there was an apparently normal distribution of glycogen, lipid, lactic dehydrogenase and phosphorylase activity in Type I and Type II fibres.
      Because of incomplete pathological data, the nosology of this condition, for the time being, must remain uncertain. Some workers consider that it is a form of sarcoidosis restricted to voluntary muscle.
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