Risk factors for relapse in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis: Tools for treatment decisions?

J.S.F. Sanders1, P.M. Stassen2, A.P. van Rossum1, C.G.M. Kallenberg1, C.A. Stegeman2

1Department of Clinical Immunology and 2Department of Nephrology, University Hospital Groningen, The Netherlands.

ABSTRACT
Current treatment based on the use of cyclophosphamide and corticosteroids has changed anti-neutrophil cytoplasmic antibody (ANCA)-associated vas-culitides from highly fatal into more chronic relapsing diseases. Relapses are a major problem in these diseases and cause increased morbidity and mortality. Current clinical research mainly focuses on achieving control of active disease while minimizing treat-mentrelated toxicity. Risks for long-term relapse and their sequelae have been less thoroughly studied. It is noteworthy that, besides treatment, several other factors have been associated with the occurrence of relapses. Thus, compared to MPO-ANCA positive patients, patients with PR3-ANCA associated vasculitis run a significantly increased risk of experiencing relapses. ANCA-status during follow-up, levels of T cell activation, genetic background, and infectious and other exogenous factors have been linked to relapse as well. With a few exceptions, these associations are merely descriptive and not pathophysiologically proven. Furthermore, data on adapting treatment in accordance with risk factors for relapse are scarce. We review here the risk factors for relapse in ANCA-associated vasculitis, their potential pathogenic implications, and their possible role in preventive strategies and adaptations of current treatment policies.

Key words
ANCA-associated vasculitis, Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, relapses, prevention.


Please address correspondence and reprint requests to: J.S.F. Sanders, Department of Internal Medicine, University Hospital Groningen, Hanzeplein 1, 9713 GZ Groningen The Netherlands.
E-mail: j.sanders@int.azg.nl

Clin Exp Rheumatol 2004; 22 (Suppl. 36): S94-S101.
© Copyright Clinical and Experimental Rheumatology 2004.