Necrotizing myopathy with pipestem capillaries, microvascular deposition of the complement membrane attack complex (MAC), and minimal cellular infiltration

Neurology. 1991 Jun;41(6):936-9. doi: 10.1212/wnl.41.6.936.

Abstract

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Aged
  • Carcinoma, Transitional Cell / pathology
  • Carcinoma, Transitional Cell / therapy
  • Complement Membrane Attack Complex / immunology*
  • Connective Tissue Diseases / immunology
  • Connective Tissue Diseases / pathology*
  • Endothelium, Vascular / ultrastructure
  • Female
  • Humans
  • Immunohistochemistry
  • Immunotherapy
  • Male
  • Microscopy, Electron
  • Middle Aged
  • Muscles / blood supply
  • Muscles / pathology
  • Muscles / ultrastructure
  • Muscular Diseases / pathology*
  • Necrosis
  • Urinary Bladder Neoplasms / pathology
  • Urinary Bladder Neoplasms / therapy

Substances

  • Complement Membrane Attack Complex