Version July 2025
| Disorder | Extrapulmonary features | Serology | Essential diagnostic criteria |
| Anti-GBM disease | GN >90% | +Anti-GBM antibodies | +Serum anti-GBM antibodies |
| Extrarenal disease rare | Linear IgG in glomeruli | ||
| SLE | GN >90% | +ANA | Clinical features, +ANA, immune complexes in glomeruli by IF, EM |
| Extrarenal disease common (arthritis, serositis, purpura, photosensitivity) | |||
| GPA | GN >90% | +ANCA | Necrotizing granulomatous vasculitis in upper airway or lung biopsy or Pauci-immune necrotizing GN with extensive upper airway disease or cavitary lung nodules |
| Extrarenal disease common | |||
| |||
| |||
| Nonspecific systemic necrotizing vasculitis (microscopic polyarteritis, polyangiitis, overlap syndrome) | GN >90% | ±ANCA (usually + if include p-ANCA) | Necrotizing, crescentic GN (or pathologic evidence of vasculitis elsewhere) and exclude GPA to extent possible |
| Extrarenal features may include arthritis, purpura, mononeuropathy | |||
| Idiopathic pulmonary hemosiderosis | None | ± IgA tissue transglutaminase antibodies ± IgA endomysial antibodies | Exclusion of all other causes |
