Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of adult polymyositis with peculiar muscular pathology of innumerable muscle fiber necrosis and regeneration accentuated in the perifascicular area. A 51-year-old woman developed generalized weakness of the extremities, trunk and bulbar muscles subacutely for two months. Anterior tibial muscle biopsy showed numerous tube-like necrotic/regenerative muscle fibers predominantly in the perifascicular area. The diameters of the muscle fibers were smaller in the periphery of the fascicles. Small arteries at the center of the fascicles occasionally showed marked perivascular cuffing, although complement component C9 was negative in the vessel wall. The gradient of the diameters of necrosis/regeneration fibers was thought to have been caused by ischemia of the muscles, which persisted at the perifascicular area leading to recurrent necrosis and regeneration and gradually invaded towards the center of the fascicles. We designated this muscular pathology as perifascicular necrosis and regeneration, and regarded it was an acute severe form of the perifascicular ischemic lesions of myositis.
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PMID:[Perifascicular necrosis and regeneration in a case of adult polymyositis]. 191 31

Monoclonal antibodies to human complement component C9 were used to localise the membrane attack complex (MAC) of human complement on muscle fibres from patients with autoimmune myositis. A pigeon erythrocyte 'model' system was used to screen the available monoclonal antibodies for their ability to bind C9 after its insertion into the MAC. Three of the five antibodies tested were demonstrated, using radioiodine- or rhodamine-labelled second antibody, to bind. The antibody giving maximum binding in this system was used to demonstrate that MACs were present on histologically normal as well as on necrotic fibres in myositis. No binding was seen in muscle from control subjects. These results suggest a primary role of complement in the pathogenesis of muscle fibre necrosis in myositis.
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PMID:Immunolocalization of complement component C9 on necrotic and non-necrotic muscle fibres in myositis using monoclonal antibodies: a primary role of complement in autoimmune cell damage. 637 Aug 40