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pauci-immune glomerulonephritis
Etiology:
- ANCA-associated vasculitis
- Wegener's granulomatosis
- microscopic polyangiitis
- Churg-Strauss syndrome
Epidemiology:
- 15-25% of glomerulonephritis
Pathology:
- microscopic vessel vasculitis
- necrotizing lesions in glomeruli
- little of no immune deposits
- may occur with or without systemic vasculitis
- ANCA-associated vasculitis
Clinical manifestations:
- variable: minimal change with hematuria to rapidly progressive glomerulonephritis
- constitutional symptoms
- low grade fever
- myalgia, arthralgia
- fatigue
- leukocytoclastic vasculitis resulting in palpable purpura
- pulmonary infiltrates to pulmonary hemorrhage
Laboratory:
- anti-neutrophil cytoplasmic antibody (ANCA)
- urinalysis: hematura
- serum complement: normal
Special laboratory:
- renal biopsy
- absent or minimal staining for immunoglobulin
Complications:
- frequent cause of rapidly progressive glomerulonephritis
Management:
- induction with glucocorticoids plus cyclophosphamide +/- plasmapheresis
- rituximab may be as effective & safer than cyclophosphamide in patients with mild-moderate disease [1]
- maintenance with azathioprine, mycophenolate, or methotrexate [1]
prognosis -older age, severe pulmonary disease, & severe renal failure at presentation portend poor prognosis [1]
General
glomerulonephritis (GN, nephritic syndrome)
References
- Medical Knowledge Self Assessment Program (MKSAP) 17, 18.
American College of Physicians, Philadelphia 2015, 2018.