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Diagnosis: pauci-immune necrotizing glomerulonephritis - ANCA-associated

The microscopic features, the negative immunofluorescence and positive serum ANCA allowed us to diagnose this disease. But, what is the etiology and/or pathogenesis in this case?

In an exhaustive review of the pre-transplant clinical history we found that the patient had chronic renal failure of rapid onset, without renal biopsy. She had a c-ANCA positive test: 1:320, several months before transplantation. Extrarenal symptoms were never demonstrated. With this data we "assume" that it is an ANCA-associated vasculitis limited to the kidney, recurrent.

The patient has continued with impaired renal function despite having received cyclophosphamide. There are until now only 4 months of follow-up.

See the chapter Crescentic glomerulonephritis of our Tutorial.

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Bibliography

• Geetha D, Eirin A, True K, Valentina Irazabal M, Specks U, Seo P, Nachman P, Fervenza FC. Renal Transplantation in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Multicenter Experience. Transplantation. 2011 Apr 19. [Epub ahead of print] [PubMed link]
• Rutgers A, Sanders JS, Stegeman CA, Kallenberg CG. Pauci-immune necrotizing glomerulonephritis. Rheum Dis Clin North Am. 2010 Aug;36(3):559-72. [PubMed link]
• Berden AE, Ferrario F, Hagen EC, Jayne DR, Jennette JC, Joh K, Neumann I, Noël LH, Pusey CD, Waldherr R, Bruijn JA, Bajema IM. Histopathologic classification of ANCA-associated glomerulonephritis. J Am Soc Nephrol. 2010 Oct;21(10):1628-36. [PubMed link]
• Tabata H, Honda K, Moriyama T, Itabashi M, Taneda S, Takei T, Tanabe K, Teraoka S, Yamaguchi Y, Oda H, Nitta K. Two cases of ANCA-associated vasculitis in post-transplant kidney: relapse and de novo. Clin Transplant. 2009 Aug;23 Suppl 20:49-53. [PubMed link]

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