s36_ab9

Churg Strauss syndrome - Successful induction of remission with methotrexate and unexpected high cardiac and pulmonary relapse ratio during maintenance treatment

C. Metzler, B. Hellmich, A. Gause, W.L. Gross, K. de Groot¹

Poliklinik für Rheumatologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck and Rheumaklinik Bad Bramstedt GmbH, Lübeck; ¹Klinik für Nephrologie, Medizinische Hochschule, Hannover, Germany

ABSTRACT
Objective
To examine the safety and efficacy of methotrexate (MTX) plus low-dose prednisolone for induction of remission in non life- or organ-threatening courses and for remission main-tenance in Churg-Strauss syndrome (CSS).

Methods
In an open-label study 11 patients were treated with MTX for induction of remission at initial diagnosis and relapse. Twenty-five patients received MTX for maintenance of remission. Primary endpoints were the achievement of remission and the incidence of relapses, respectively. Doses of concomitant prednisolone (PRD) and side effects were secondary end-points.

Results
Induction of remission was achieved in 8/11 patients with MTX/PRD. Median time to remission was 5 months (range 2 - 9). Remission was maintained in 12 of 23 with available long-term follow-up (median 48 months). Eleven patients experienced 8 major and 3 minor relapses with a median time from remission to first relapse of 9 months. With MTX, the median cumulative PRD dose during the induction phase was 6.2 g. In the maintenance phase PRD could be reduced by 53% in responders. Apart from one case of MTX-induced pneumonitis, ad-verse events were confined to mild/moderate episodes of infection and leucopenia. No opportunistic infections occurred, neither did steroid-specific adverse events

Conclusions
MTX is safe and effective for the induction of remission in non-life-threatening CSS. It allows a considerable reduction of PRD and thus avoidance of PRD-related adverse events. However, the ability of MTX to prevent relapses in CSS appears limited. The identification of an optimal maintenance regimen and prognostic factors for treatment response requires trials with larger patient numbers.

Key words
Churg-Strauss syndrome, methotrexate, remission, relapse.

Please address correspondence and reprint requests to: Claudia Metzer, MD, Universitätsklinikum Schleswig-Holstein,

Campus Lübeck Poliklinik für Rheumatologie, Ratzeburger Allee 160, D-23538 Lübeck, Germany.
E-mail: metzler@rheuma-zentrum.de