Anti-neutrophil cytoplasmic antibodies in generalized autoimmune diseases

Armin Schnabel*, Stefanie Hauschildt, Wolfgang L. Gross

*Corresponding author for this work
39 Citations (Scopus)


Anti-neutrophil cytoplasmic antibodies (ANCA) are a system of autoantibodies which are strongly associated with the primary systemic vasculitides (PSV). cANCA, as detected by indirect immunofluorescence, are mostly reactive to pro-teinase 3 (PR3) and bear high sensitivity and specificity for Wegener’s granulomatosis. pANCA have varied subspecificities and clinical associations. The most important subspecificity of pANCA is anti-myeloperoxidase (MPO), which is strongly associated with microscopic polyaniitis and pauci-immune crescentic glomerulonephritis. pANCA also occur at low to moderate frequency in other PSV, collagen vascular disease, inflammatory bowel disease and autoimmune liver disease. In systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA), pANCA of varied subspecificity have been found, including anti-MPO at low rate and low titer, while cANCA and anti-PR3 were generally absent. Consequently, anti-PR3 (PR3-ANCA) and anti-MPO (MPO-ANCA) at moderate and high titer are distinguishing features of the PSV and, in an appropriate clinical setting, argue strongly against the presence of SLE or RA. Since no significant clinical association has been found for other pANCA subspecificities, cANCA, PR3-ANCA and MPO-ANCA remain the critical elements of ANCA testing in the clinical diagnosis of generalized autoimmune diseases.

Original languageEnglish
JournalInternational Archives of Allergy and Immunology
Issue number3
Pages (from-to)201-206
Number of pages6
Publication statusPublished - 01.01.1996

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)


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