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Diagnosis: Pauci-immune necrotizing and crescentic Glomerulonephritis (ANCA-associated)

Crescentic GN, recognized as the most severe form of glomerular inflammation, describes the histopathological finding of extra-capillary cells, both proliferating parietal epithelial cells and infiltrating leucocytes, and plasma proteins within Bowman’s space that is often associated with necrosis and fragmentation of the underlying glomerular tuft and periglomerular inflammation. Classically it has been categorized based on the pattern of immunoglobulin deposition within the diseased glomerulus: type I with linear IgG deposition, as seen in anti-GBM disease; type II with immune-complex deposition, seen in LN, cryoglobulinaemia and HScP; and type III with minimal or no immunoglobulin deposition (i.e. pauci-immune), associated with circulating ANCA in 80–90% of cases. It may occur in isolation as a renal-limited phenomenon, although more frequently it develops as a feature of multisystem autoimmune or rheumatic diseases. The usual clinical correlate of glomerular necrosis and crescent formation is rapidly progressive GN (RPGN), i.e. an abrupt decline in renal function that occurs over days to weeks, in association with abnormal urinary findings of haematuria and proteinuria. Historical cohorts suggest that if left untreated, necrotizing and crescentic GN has an invariably poor outcome, with the majority of patients progressing to end-stage renal disease (ESRD) or death within months. Immunosuppressive therapy has improved renal and patient survival; however, early detection and instigation of appropriate therapy is essential to prevent irreversible kidney damage and poor long-term outcomes. (The previous paragraph taken from: McAdoo SP, et al. Necrotizing and crescentic glomerulonephritis presenting with preserved renal function in patients with underlying multisystem autoimmune disease: a retrospective case series. Rheumatology (Oxford). 2015;54(6):1025-32. [PubMed link])

See the chapter: Crescentic Glomerulonephritis of our Tutorial.

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References

  • Chen A, Lee K, Guan T, He JC, Schlondorff D. Role of CD8+ T cells in crescentic glomerulonephritis. Nephrol Dial Transplant. 2019 Mar; doi: 10.1093/ndt/gfz043. [Epub ahead of print]. [PubMed link]
  • Bjørneklett R, Bostad L, Fismen AS. Prognosis and Histological Classification in Elderly Patients with ANCA-Glomerulonephritis: A Registry-Based Cohort Study. Biomed Res Int. 2018;2018:7581567. [PubMed link]
  • Jennette JC, Nachman PH. ANCA Glomerulonephritis and Vasculitis. Clin J Am Soc Nephrol. 2017;12(10):1680-1691. [PubMed link]
  • Webster P, Pusey C. Glomerular disease: Crescentic glomerulonephritis: beyond the immune system. Nat Rev Nephrol. 2017;13(4):198-200. [PubMed link]
  • Fogo AB, Lusco MA, Najafian B, Alpers CE. AJKD Atlas of Renal Pathology: Pauci-immune Necrotizing Crescentic Glomerulonephritis. Am J Kidney Dis. 2016;68(5):e31-e32. [PubMed link]
  • Rampelli SK, Rajesh NG, Srinivas BH, Harichandra Kumar KT, Swaminathan RP, Priyamvada PS. Clinical spectrum and outcomes of crescentic glomerulonephritis: A single center experience. Indian J Nephrol. 2016;26(4):252-6. [PubMed link]
  • McAdoo SP, Tanna A, Randone O, Tam FW, Tarzi RM, Levy JB, Griffith M, Lightstone L, Cook HT, Cairns T, Pusey CD. Necrotizing and crescentic glomerulonephritis presenting with preserved renal function in patients with underlying multisystem autoimmune disease: a retrospective case series. Rheumatology (Oxford). 2015;54(6):1025-32. [PubMed link]

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