The antineutrophil cytoplasmic antibody-associated vasculitides

Am J Med. 2004 Jul 1;117(1):39-50. doi: 10.1016/j.amjmed.2004.02.030.


Wegener's granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome are small- to medium-vessel vasculitides linked by overlapping pathology and the presence of antineutrophil cytoplasmic antibodies (ANCA). Commonly referred to as the ANCA-associated vasculitides, these diseases are challenging to diagnose and to treat. Distinguishing the ANCA-associated vasculitides from other forms of vasculitis or nonvasculitic processes (such as infection) can be particularly difficult. This review describes the clinical and pathologic hallmarks of the ANCA-associated vasculitides, discusses the role of ANCA assays in diagnosis and treatment, and outlines an approach to the evaluation and management of these diseases.

Publication types

  • Review

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Cyclophosphamide / therapeutic use
  • Diagnosis, Differential
  • Ear
  • Eosinophilia / epidemiology
  • Eye
  • Heart
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Kidney
  • Lung
  • Nose
  • Peripheral Nerves
  • Pharynx
  • Prognosis
  • Vasculitis / drug therapy
  • Vasculitis / immunology*
  • Vasculitis / physiopathology*


  • Antibodies, Antineutrophil Cytoplasmic
  • Immunosuppressive Agents
  • Cyclophosphamide