Zusammenfassung
Das Sjögren-Syndrom (SS) ist eine chronische Autoimmunerkrankung, die durch lymphoplasmazelluläre Infiltration und Verlust des sekretorischen Epithels der exokrinen Drüsen charakterisiert ist (wobei besonders Speichel- und Tränendrüsen befallen sind) und zu Mund- und Augentrockenheit führt. Die Symptome des SS sind chronisch und manchmal verheerend mit schweren Einbußen der Lebensqualität. Obwohl das SS als klassische Autoimmunkrankheit gilt, werden Immunsuppressiva als wichtigster Bestandteil der Therapie von Autoimmunerkrankungen selten eingesetzt. Cholinergika wie Pilocarpin und Cevimeline sind die Stützen der gegenwärtigen Therapie des SS, während orales Interferon und topisches Ciclosporin als Augentropfen eine mildernde Wirkung auszuüben scheinen. Im Gegensatz dazu haben andere Immunsuppressiva wie Methotrexat, Azathioprin oder Anti-TNF-α die Symptome des mit dieser Erkrankung verbundenen Sicca-Komplexes nicht gelindert. Der Einsatz von Kortikosteroiden, Cyclophosphamid und Nucleosid-Analoga ist für den extraglandulären Befall des SS reserviert.
Abstract
Sjögren’s syndrome (SS), is a chronic autoimmune disorder, characterized by lymphocytic infiltration and destruction of the exocrine glands (mainly the salivary and the lacrimal glands) resulting in dry mouth and eyes. The symptoms of SS are chronic and sometimes devastating, compromising the quality of life at a major extent. Although SS is considered a classical autoimmune disorder, evidence for the use of immunosuppressive agents, the mainstay of therapy of diseases of autoimmune origin, is scarce. Cholinergic agents, such as pilocarpine and cevimeline are the cornerstone of current therapy of SS, while oral interferon and ciclosporine ocular drops seem to be of some benefit. In contrast, other immunosuppressive substances, such as methotrexate, azathioprine or anti-TNF agents, did not alleviate the sicca features associated with the disease. Corticosteroids, cyclophosphamide and nucleoside analogues are reserved for life threatening severe extraglandular manifestations of SS.
Schlüsselwörter
Sjögren-Syndrom (SS) - Autoimmunerkrankung - Immunsuppression - Immunmodulation
Key words
Sjögren’s Syndrome (SS) - autoimmune disease - immunosuppression - immunomodulation
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H. M. Moutsopoulos, MD
Department of Pathophysiology, Medical School, National University of Athens
M. Asias 75
11527 Athens
Griechenland
Telefon: ++ 30/2 10/7 46 25 13
Fax: ++ 30/2 10/7 46 26 46
eMail: hmoutsop@med.uoa.gr