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ANCA-associated glomerulonephritis
Etiology:
- anti-neutrophil cytoplasmic antibody (ANCA)
- cANCA
- pANCA
Epidemiology:
- accounts for > 1/2 of rapidly progressive glomerulonephritis in elderly
Pathology:
- pauci-immune crescentic glomerulonephritis
Clinical manifestations:
- prodrome of malaise, arthralgia, myalgia, flu-like symptoms
- variable, from hematuria to rapidly progressive glomerulonephritis [1]
- variable, from pulmonary infiltrate to hemoptysis [1]
- epistaxis
- acute renal failure
- lacy or reticular rash characteristic of leukocytoclastic vasculitis (palpable purpura) [1]
Laboratory:
- urinalysis
- dark brown urine, hematuria, proteinuria
- renal function tests
- neutrophil cytoplasmic antibody (ANCA) in serum
- cANCA in serum
- cANCA in serum
- serum complement levels are normal
Special laboratory:
- renal biopsy with immunofluorescence staining
- pauci-immune staining (ANCA-associated glomerulonephritis)
- linear staining (anti-glomerular basement membrane disease)
- granular staining (lupus nephritis)
Management:
- plasmapheresis QD for 2 weeks for ANCA-associated glomerulonephritis associated with pulmonary hemorrhage [1]
- combination of glucocorticoids + cyclophosphamide or rituximab [1]
- indicated for pulmonary alveolar disease [1]
- reduces progression to ESRD at 1 year, but not 1 year mortality
- mortality benefit for immunosuppression begins to emerge after 1 year [2]
General
ANCA-associated vasculitis
crescentic glomerulonephritis (rapidly progressive glomerulonephritis)
References
- Medical Knowledge Self Assessment Program (MKSAP) 18, 19
American College of Physicians, Philadelphia 2018, 2021
- Medical Knowledge Self Assessment Program (MKSAP) 19
Board Basics. An Enhancement to MKSAP19.
American College of Physicians, Philadelphia 2022
- Bomback AS, Appel GB, Radhakrishnan J et al
ANCA-associated glomerulonephritis in the very elderly.
Kidney Int. 2011 Apr;79(7):757-64. Epub 2010 Dec 15.
PMID: 21160463 Free Article