Background. Recurrent antineutrophil cytoplasmic antibody (ANCA)-associated
small vessel vasculitis (ANCA-SVV) after renal transplantation has been de
scribed in case series. However, general information regarding the frequenc
y, character, and predictors of recurrent disease after transplantation is
currently lacking. We considered the rate of relapse, whether a positive AN
CA at the time of transplantation predicted relapse, and whether cyclospori
ne A prevented recurrent disease.
Methods. We performed a pooled analysis of published data, added to the exp
erience at the Universities of North Carolina (14 patients) and Lund, Swede
n (11 patients). To avoid reporting bias, only case series were included fo
r analysis. Subgroup analysis was performed by disease category (Wegener's
granulomatosis, microscopic polyangiitis, or necrotizing crescentic glomeru
lonephritis) and ANCA staining pattern.
Results. ANCA-SVV recurred in 17.3% of all patients (N = 127), in 20% of cy
closporine A-treated patients (N = 85), and in 25.6% of patients with circu
lating ANCA at the time of transplantation (N = 39). There was no statistic
ally significant difference in the relapse rate between patients treated an
d those not treated with cyclosporine A (P = 0.45), between those with and
without circulating ANCA at the time of transplant (P = 0.75), or between p
atients with Wegener's granulomatosis and those with microscopic polyangiit
is or necrotizing crescentic glomerulonephritis alone (P = 0.62).
Conclusion. There is a substantial relapse rate in the ANCA-SVV population.
Therapy with cyclosporine A does not protect against recurrent ANCA-SVV, a
nd the presence of a positive ANCA at the time of transplantation does not
preclude transplantation. These conclusions must be substantiated with a pr
ospective study of renal transplantation in patients with ANCA-SVV so as to
optimize their management.