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Sjögren-Syndrom

Aktuelle Aspekte aus rheumatologischer Sicht

Sjögren’s syndrome

Current aspects from a rheumatological point of view

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Zusammenfassung

Das Sjögren-Syndrom ist eine autoimmune Erkrankung der exokrinen Drüsen mit den Leitsymptomen Keratokonjunktivitis und Stomatitis sicca auf Grundlage einer komplexen Pathogenese. Die Prävalenz beträgt ca. 0,5–1%, wobei ein primäres Sjögren-Syndrom von einem sekundären Sjögren-Syndrom bei anderen Autoimmunerkrankungen differenziert wird. Die Diagnose wird durch die subjektiven Beschwerden und den objektiven Nachweis von Sicca-Symptomen, Anti-Ro(SS-A)/La(SS-B)-Antikörpern und/oder einer fokalen lymphozytären Infiltration der Drüsengewebe gestellt. Neben der typischen Sicca-Symptomatik, die symptomatisch durch Substitutions- und Stimulationstherapie behandelt wird, weisen einige Patienten extraglanduläre Manifestationen auf. Dominierend finden sich Beschwerden am Bewegungsapparat und an inneren Organen, die durch den Rheumatologen behandelt werden. Die Indikation zur Basistherapie wird individuell gestellt; deren Wirksamkeit ist jedoch nicht durch Studien belegt. Etwa 5–10% der Patienten mit einem primären Sjögren-Syndrom entwickeln ein Non-Hodgkin-Lymphom der B-Zell-Reihe. Die Erkrankung erfordert eine interdisziplinäre Betreuung mit Augen-, Zahn-, HNO- und anderen Kollegen in Abhängigkeit vom klinischen Bild.

Abstract

Sjögren’s syndrome is an autoimmune disease of the exocrine glands characterized by the leading symptoms of keratoconjunctivitis and stomatitis sicca based on a complex pathogenesis. The prevalence is about 0.5–1%; primary Sjögren’s syndrome is differentiated from secondary Sjögren’s syndrome associated with other autoimmune disorders. The diagnosis is established by the presence of subjective complaints and objective evidence of sicca symptoms, anti-Ro(SSA)/La(SSB) antibodies, and/or focal lymphocytic infiltration of the glandular tissue. In addition to the typical sicca symptomatology, which is managed symptomatically by substitution and stimulation therapy, some patients exhibit extraglandular manifestations. Complaints involving the musculoskeletal system and inner ear dominate and are treated by the rheumatologist. The indication for base therapy is tailored to individual needs, but the efficacy of this approach has not been established in studies. About 5–10% of the patients with primary Sjögren’s syndrome develop a B-cell non-Hodgkin’s lymphoma. The disease requires interdisciplinary management including, among others, ophthalmologists, dentists, and otorhinolaryngologists, depending on the clinical picture.

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  1. Reha-Zentrum Bad Aibling, Deutsche Rentenversicherung Bund, Rheumazentrum – AHB, Kolbermoorer Straße 56, 83043 , Bad Aibling, Deutschland

    C. Tomiak

  2. Charite Universitätsmedizin Berlin & Deutsches Rheumaforschungszentrum Berlin, Berlin, Deutschland

    T. Dörner

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  1. C. Tomiak
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  2. T. Dörner
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Tomiak, C., Dörner, T. Sjögren-Syndrom. Z. Rheumatol. 65, 505–519 (2006). https://doi.org/10.1007/s00393-006-0101-0

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