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Case 163
Diagnosis
 
     
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Diagnosis: Pauci-Immune ANCA-associated Vasculitis

In some cases of ANCA-associated GN, it is possible to find arterial or arteriolar vasculitis in renal biopsy. The usual clinical correlate of crescent formation and arterits is rapidly progressive GN (RPGN). 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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