Immunsuppressive Therapie der Sarkoidose

Zusammenfassung

Die Sarkoidose ist eine granulomatöse Systemerkrankung, die nahezu jedes Organsystem betreffen kann. Behandelt wird die Sarkoidose nur bei bestimmten Organmanifestationen oder progredientem Verlauf. Zum Einsatz kommen vor allem Immunsuppressiva. Die Therapie der ersten Wahl sind Kortikosteroide unabhängig von der Art der Organmanifestation. Daneben werden in der Literatur nahezu alle vorhandenen Immunsuppressiva auch in der Therapie der Sarkoidose beschrieben. Verwendet wurden unter anderem MTX, Leflunomid, Azathioprin, Cyclosporin, Cyclophosphamid, Mycophenolat-Mofetil, Thalidomid, (Hydroxy-)Chloroquin, Pentoxyphyllin, Ursodeoxycholsäure sowie TNF-α-Blocker und Rituximab. Eingesetzt wurden Immunsuppressiva vor allem zur Einsparung von Kortikosteroiden, aber auch bei therapierefraktären oder lebensbedrohlichen Verläufen der Sarkoidose. Die Auswahl des Immunsuppressivums richtet sich dabei nach dem betroffenen Organ. Die Evidenz der meisten Therapien beschränkt sich allerdings auf retrospektive Fallserien und Einzelfallberichte. Nur für wenige Therapien wurden prospektive Studien durchgeführt, die jedoch überwiegend nur eine geringe Patientenzahl eingeschlossen haben. Evidenzgrade der Stufen I und II werden fast ausschließlich für Therapien der pulmonalen und kutanen Sarkoidose erreicht.

Abstract

Sarcoidosis is a granulomatous disease potentially affecting every organ system of the body. Treatment is only warranted in cases with certain organ manifestations or in cases with progressive disease. Immunosuppressants are the main players in therapy whereas glucocorticoids are the first line therapy independant from the affected site. Besides steroids virtually all available immunosuppressive agents are discussed in literature such as methotrexate, leflunomide, azathioprine, cyclosporine, cyclophosphamide, mycophenlat mofetil, thalidomide, hydroxychloroquine, pentoxyphylline, ursodeoxycholic acid as well as TNF-alpha inhibitors and rituximab. The main indication for the use of immunosuppressants is their steroid-sparing effect, but also cases of refractory or life threatening courses of disease. The choice of drugs is guided by the type of organ manifestation. The current evidence of most treatment options is based on retrospective case series and single case reports. Only few treatment recomendations are derived from prospective studies most of which having included only small numbers of patients. Evidence levels I and II currently are allmost exclusively available for pulmonary and cutanous sarcoidosis.

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