Recurrent Pauci-immune Necrotizing Crescentic Glomerulonephritis in a Kidney Transplant Patient – Corrected Proof

Heidi M. Schaefer, Anthony Langone, J. Harold Helderman, Agnes B. Fogo — Sun, 27 Sep 2009 07:00:00 +0000

Glomerulonephritis is the primary cause of end-stage renal disease (ESRD) in a substantial proportion of patients and includes antineutrophil cytoplasmic antibody (ANCA)-associated small-vessel vasculitis. Although recognition and treatment of ANCA-associated vasculitis (AAV) has improved, the diagnosis can be difficult to make. In 1 study, the diagnosis was missed (before ANCA testing was performed) in 43% of patients. It is estimated that 20%-40% of patients with AAV will progress to kidney failure requiring replacement therapy. It is important to be aware of the diagnosis before transplant to provide patient counseling and monitor allograft function closely in the postoperative period because the relapse rate can be significant. In a pooled analysis by Nachman et al, the overall recurrence rate was 17%. We present a case of a patient with reported “focal sclerosing nephropathy” and an acute increase in serum creatinine level shortly after transplant who was noted to have crescentic glomerulonephritis with the absence of immune complexes on allograft biopsy. This case shows the importance of confirming the cause of ESRD before transplant and the role of allograft biopsy in identifying the causes of decreased kidney function.

Kidney Function » Heidi M. Schaefer, Anthony Langone, J. Harold Helderman, Agnes B. Fogo

Recurrent Pauci-immune Necrotizing Crescentic Glomerulonephritis in a Kidney Transplant Patient – Corrected Proof