Necrotizing myopathy with pipestem capillaries, microvascular deposition of the complement membrane attack complex (MAC), and minimal cellular infiltration.
Three adult patients, two with undifferentiated connective tissue disease and one with carcinoma, had a distinctive pathologic reaction pattern consisting of necrotizing myopathy, minimal cellular infiltration, and a microangiopathy with thick "pipestem" vessels and microvascular deposits of complement membrane attack complex. Quantitative analysis revealed focal capillary depletion. This pattern represents an immune-mediated microangiopathy and is distinct from that observed in other inflammatory myopathies.
Emslie-Smith
A M
AM
Muscle Research Laboratory, Mayo Clinic, Rochester, MN 55905.
Engel
A G
AG
D000368:Aged; D002295:Carcinoma, Transitional Cell / Q000473:pathology / Q000628:therapy; D015938:Complement Membrane Attack Complex / Q000276:immunology*; D003240:Connective Tissue Diseases / Q000276:immunology / Q000473:pathology*; D004730:Endothelium, Vascular / Q000648:ultrastructure; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007167:Immunotherapy; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009132:Muscles / Q000098:blood supply / Q000473:pathology / Q000648:ultrastructure; D009135:Muscular Diseases / Q000473:pathology*; D009336:Necrosis; D001749:Urinary Bladder Neoplasms / Q000473:pathology / Q000628:therapy
D015938:Complement Membrane Attack Complex