Abstract
Autoantibodies in systemic vasculitis.
Highlights
Systemic vasculitis is a heterogeneous group of disorders characterized by inflammation and necrosis in the vessel wall
Antineutrophil cytoplasmic antibodies are the diagnostic biomarkers for some small-vessel necrotizing vasculitis such as granulomatosis with polyangiitis (GPA), mycroscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and renal limited vasculitis (RLV)
A study that compared three generations of proteinase 3 (PR3)-antineutrophil cytoplasmic antibodies (ANCA) assays found the highest sensitivity with the third generation anchor enzyme-linked immunosorbent assay (ELISA): 96.0% [95% confidence interval (CI): 79.6–99.3%], followed by indirect immunofluorescence (IIF): 92.0%, by second generation capture ELISA: 72.0%, and by first generation direct ELISA: 60.0% [9]
Summary
Systemic vasculitis is a heterogeneous group of disorders characterized by inflammation and necrosis in the vessel wall. Disease manifestations usually cluster into clinical phenotypes and definite diagnosis rely on confirmation by tissue biopsy, angiography, or by serologic tests. The main serologic tests for the diagnosis of primary systemic vasculitides are antineutrophil cytoplasmic antibodies (ANCA), cryoglobulins, anti-glomerular basement membrane (anti-GBM) antibodies, and anti-C1q antibodies [3]. Several other autoantibodies have been investigated in systemic vasculitis, the clinical usefulness of these antibodies still needs further investigation [1, 2] (Table 1). The main autoantibodies and their clinical associations in systemic vasculitis are reviewed.
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