Antineutrophil cytoplasmic autoantibody-associated diseases: a pathologist's perspective

Am J Kidney Dis. 1991 Aug;18(2):164-70. doi: 10.1016/s0272-6386(12)80874-2.


Antineutrophil cytoplasmic autoantibodies (ANCA) are a useful diagnostic serologic marker for the most common forms of necrotizing vasculitis, provide a means of categorizing vasculitides so that diagnostically useful shared pathologic and clinical characteristics can be recognized, and offer insight into the pathogenesis of previously idiopathic diseases. ANCA-associated vasculitides can be categorized into a number of distinctive clinicopathologic categories, eg, Wegener's granulomatosis, Churg-Strauss syndrome, pulmonary renal syndrome, microscopic polyarteritis nodosa, leukocytoclastic angiitis, and necrotizing and crescentic glomerulonephritis. At least the latter four syndromes can also be caused by other ANCA-negative immunopathogenic mechanisms, eg, immune complex deposition. Therefore, thorough diagnostic classification requires both an assessment of clinicopathologic category, as well as an assessment of immunopathologic category. Although different ANCA-associated vasculitic syndromes have distinctive clinical and pathologic features, all ANCA-associated vasculitides share a number of common pathologic features, ie, focal distribution, necrosis, and neutrophil infiltration. ANCA assays have very good sensitivity and specificity for ANCA-associated diseases, but the prevalence of these diseases in the patient population being analyzed must be taken into consideration when determining the predictive value of a test result. As with all serologic tests, ANCA results must be integrated with other clinical and pathologic data in order to reach the most accurate diagnostic conclusion.

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies / isolation & purification*
  • Biomarkers / chemistry
  • Churg-Strauss Syndrome / pathology
  • Granulomatosis with Polyangiitis / pathology
  • Humans
  • Microscopy, Fluorescence
  • Necrosis
  • Neutrophils / immunology*
  • Neutrophils / pathology
  • Vasculitis / immunology
  • Vasculitis / pathology*


  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Biomarkers