AUTOIMMUNITY TO LYSOSOMAL-ENZYMES - NEW CLUES TO VASCULITIS AND GLOMERULONEPHRITIS

CGM KALLENBERG*, JWC TERVAERT, FJ VANDERWOUDE, R GOLDSCHMEDING, AEGK VONDEMBORNE, JJ WEENING

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

68 Citations (Scopus)

Abstract

The demonstration of autoantibodies to neutrophil cytoplasmic antigens (ANCA) in (1) systemic vasculitides such as Wegner's granulomatosis, classic polyarteritis nodosa and the Churg Strauss syndrome and (2) idiopathic crescentic glomerulonephritis has placed these disorders within the spectrum of autoimmune diseases. The target antigens have been identified as myeloid lysosomal enzymes that are involved in tissue destruction by neutrophils. Recent data suggest a pathogenetic role for these autoimmune responses. The autoantibodies may activate neutrophils, resulting in extensive tissue necrosis. Alternatively, T-cell-mediated immunity to these positively-charged enzymes that localize to polyanionic basement membranes may, in concert with the presence of autoantibodies, result in glomerulonephritis and vasculitis.

Original languageEnglish
Pages (from-to)61-64
Number of pages4
JournalImmunology Today
Volume12
Issue number2
DOIs
Publication statusPublished - Feb-1991

Keywords

  • GLOMERULAR BASEMENT-MEMBRANE
  • PEROXIDE-HALIDE SYSTEM
  • WEGENERS GRANULOMATOSIS
  • POLYMORPHONUCLEAR LEUKOCYTE
  • AUTOANTIBODIES
  • NEUTROPHIL
  • INJURY
  • MYELOPEROXIDASE
  • IMMUNE
  • DEGRADATION

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