ANCA-Associated Vasculitis
• ANCA (antineutrophil cytoplasmic antibody)-associated vasculitis (AAV) are subclassified as: ° Granulomatosis with polyangiitis (GPA)
î Microscopic polyangiitis (MPA)
î Eosinophilic granulomatosis with polyangiitis (EGPA)
î Renal limited vasculitis with pauci-immune necrotizing glomerulonephritis (RLV)
• ANCA antibody association (Table 25-9):
TABLE 25-9
SENSITIVITY OF ANCA ANTIBODIES
| Disease | PR3 (%) | MPO (%) | Negative ANCA (%) |
| GPA | 66 | 24 | 10 |
| MPA | 26 | 58 | 10-15 |
| RLV | 20 | 64 | 15-20 |
| EGPA | 10 | 50 | 35-50 |
Atypical ANCA pattern is associated with drug-induced vasculitis, nonvasculitic rheumatic diseases, primary sclerosing cholangitis, ulcerative colitis, cystic fibrosis, and endocarditis.
ANCA, antineutrophil cytoplasmic antibodies; EGPA, eosinophilic granulomatosis with polyangiitis; GPA, granulomatosis with polyangiitis; MPA, microscopic polyangiitis; MPO, myeloperoxidase; RLV, renal limited vasculitis.Modified from Mark M. Antineutrophil cytoplasmic antibody-associated vasculitis. In: West S, Kolfenbach J. Rheumatology Secrets. 4th ed. Elsevier; 2020:224-235. Copyright © 2020 Elsevier. With permission.
• Brief review of each of the ANCA-associated vasculitis (AAV) is presented below.
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