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NDT Plus Advance Access published online on February 2, 2009
NDT Plus, doi:10.1093/ndtplus/sfp006
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© The Author [2009]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Make the grade for Wegener's granulomatosis after kidney transplantation

Lioba Schewior1, Duska Dragun1, Birgit Rudolph2 and Elke Schaeffner1

1 Department of Nephrology and Intensive Care Medicine, Charité Campus Virchow
2 Department of Pathology, Charité Campus Mitte, Berlin, Germany

Correspondence: Duska Dragun, Department of Nephrology and Intensive Care Medicine, Charité Hospital, Campus Virchow Klinikum, Augustenburger Platz 1, 13353 Berlin, Germany. Tel: +49-30-450-653485; Fax: +49-30-450-553909; E-mail: duska.dragun{at}charite.de


  Abstract

Antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) is a well-described cause of multiple organ involvement including rapidly progressive pauci-immune crescentic glomerulonephritis. Kidney transplantation (KTx) is considered the treatment of choice in patients with end-stage renal disease (ESRD) due to AAV. Patient and graft survival in AAV after KTx is favourable and comparable with other non-diabetic causes of ESRD. While relapse of AAV is high in dialysis patients (up to 50%), it decreases after KTx (8.6–22.2%). Yet, relapse may occur at any time after KTx and transplant involvement has been documented in at least 25 cases. Therapeutic guidelines for the management of AAV after KTx do not exist and clinical management is a controversial discussion. We present two unusual cases of young males with smouldering AAV who recently underwent KTx at our hospital. Case 1 experienced repeated relapses after KTx and was finally successfully treated with rituximab. Case 2 received rituximab pre-emptively before living kidney donation and remained free of flairs. Prompted by theses two cases, we reviewed the literature focusing on the right point of time for transplantation, risk assessment, role of antineutrophil cytoplasmic antibodies, clinical presentation of flairs and immunosuppression in smouldering Wegener's granulomatosis (WG) and in relapse, including individualized treatment with rituximab.

Key Words: ANCA • kidney transplantation • relapse • rituximab • vasculitis

Received for publication May 18, 2008. Accepted for publication January 6, 2009.


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