Systemischer Lupus erythematodes und primäres Sjögren-Syndrom

ZUSAMMENFASSUNG

Die Therapie des systemischen Lupus erythematodes (SLE) und des primären Sjögren-Syndroms (pSS) stellt eine Herausforderung dar. Dies liegt nicht nur an der Heterogenität der Ausprägung beider Erkrankungen, sondern auch an den wenigen erprobten und zugelassenen Therapiemöglichkeiten. Für die Behandlung des SLE zugelassen sind Hydroxychloroquin, Azathioprin, Belimumab und, zur Therapie der schweren Lupus-Nephritis, Cyclophosphamid; durch den gemeinsamen Bundesausschuss wurde zudem Mycophenolat-Mofetil zur Behandlung der Lupus-Nephritis genehmigt, während das bewährte Medikament Methotrexat zwar nicht zugelassen ist, jedoch standardmäßig verwendet wird. Für das pSS gibt es wenige zugelassene Optionen zur Therapie der glandulären Beteiligung. Die Behandlung extraglandulärer, systemischer Manifestationen richtet sich nach dem Organbefall. Hierfür existieren keine zugelassenen Therapieoptionen und wir bewegen uns stets im Off-Label-Bereich. Ziel der vorliegenden Arbeit ist es, die möglichen Therapieoptionen des SLE und pSS zu erläutern und ihren Stellenwert in der Behandlung einzelner Manifestationen darzulegen. Zudem wollen wir dem behandelnden Rheumatologen Hinweise geben, wann ein Off-Label-Use für beide Erkrankungen sinnvoll sein kann.

SUMMARY

The treatment of patients with systemic lupus erythematosus (SLE) and primary Sjögren’s syndrome (pSS) is challenging. This is not only the result of the heterogeneity of manifestations of both diseases but also due to a lack of approved therapeutic options. Approved for the treatment of SLE are hydroxychloroquine, azathioprine, belimumab, and for the treatment of severe lupus nephritis, cyclophosphamide; mycophenolate mofetil can be prescribed for the treatment of lupus nephritis according to the “Gemeinsamer Bundesausschuss”. Methotrexate is not approved for the treatment of SLE but used frequently, nevertheless. Only few approved drugs exist for the treatment of glandular manifestations of pSS. The treatment of extraglandular systemic manifestations depends on the organ involved, however, there are no approved drugs, and we are always treating off-label. It is the aim of this article to outline the therapeutic options for SLE and pSS and their relevance for the treatment of individual manifestations. In addition, we would like to provide the treating rheumatologists with information on when off-label treatment is warranted for both diseases.

Schlüsselwörter

Systemischer Lupus erythematodes - primäres Sjögren-Syndrom - Pharmakotherapie - Immunsuppressiva - Off-Label-Use

Keywords

Systemic lupus erythematosus - primary Sjögren’s syndrome - pharmacotherapy - immunosuppressants - off-label use

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