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ANCA-Negative Pauci-Immune GN — ESENeph MCQ

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HardRenal VasculitisANCA-Negative Pauci-Immune GNESENeph

A 40-year-old man with CKD G3a (eGFR 55) has isolated haematuria and 0.3 g/day proteinuria. Kidney biopsy shows segmental necrotising glomerulonephritis with fibrinoid necrosis in 2 of 15 glomeruli. ANCA is negative. ANA is negative. Anti-GBM is negative. Complement is normal. IF shows pauci-immune pattern. What should be considered?

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Correct answer: E — ANCA-negative pauci-immune glomerulonephritis – treat as ANCA vasculitis

Approximately 10-20% of pauci-immune crescentic/necrotising GN is ANCA-negative by conventional assays. These patients are managed identically to ANCA-positive vasculitis per KDIGO 2024, as the histology and clinical behaviour are similar. Some patients may have antibodies detectable by research assays only. Treatment with glucocorticoids plus Rituximab or Cyclophosphamide is recommended. Response rates and prognosis are comparable to ANCA-positive disease.

Reference: KDIGO 2024 – ANCA-Associated Vasculitis; Chen et al 2007 – ANCA-Negative Vasculitis