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Aktuelle Rheumatologie 2017; 42(04): 291-300
DOI: 10.1055/s-0042-124185
Übersichtsarbeit
© Georg Thieme Verlag KG Stuttgart · New York

Das Sjögren-Syndrom

Sjögren’s Syndrome
Isabella Tröster
1   Klinik für Rheumatologie und Klinische Immunologie, Universitätsklinikum Freiburg, Medizinische Fakultät der Universität Freiburg, Freiburg
,
Maike Jülich
1   Klinik für Rheumatologie und Klinische Immunologie, Universitätsklinikum Freiburg, Medizinische Fakultät der Universität Freiburg, Freiburg
,
Reinhard Voll
1   Klinik für Rheumatologie und Klinische Immunologie, Universitätsklinikum Freiburg, Medizinische Fakultät der Universität Freiburg, Freiburg
,
Florian Kollert
2   Rheumatologie und Klinische Immunologie, Universität Freiburg, Freiburg
› Author Affiliations
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Publication History

Publication Date:
30 August 2017 (online)

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Zusammenfassung

Das Sjögren-Syndrom ist eine chronische Autoimmunerkrankung mit Beteiligung der Tränen- und Speicheldrüsen. Durch lymphozytäre Infiltration und sekundäre Destruktion des exokrinen Drüsengewebes kommt es zur Sicca-Symptomatik. Es handelt sich um eine der 3 häufigsten Autoimmunerkrankungen mit einer Prävalenz von 0,03–2,1%, abhängig von Alters- und Populationsgruppen sowie der angewandten Diagnostik-Kriterien, und betrifft deutlich häufiger Frauen (ca. 10:1) [1] [2] [3]. Neben der charakteristischen Sicca-Symptomatik leiden viele Patienten unter systemischen Manifestationen wie z. B. Arthralgien, seltener Hautvaskulitis, interstitieller Lungenerkrankung oder Nierenbeteiligung.

Nicht-steroidale Antirheumatika, Kortikosteroide und Immunsuppressiva werden zur Therapie dieser Komplikationen angewandt während die Sicca-Symptomatik meist lokal-symptomatisch behandelt wird. Das Risiko der Entwicklung eines malignen Lymphoms ist beim Sjögren-Syndrom erhöht. Ein Großteil der Patienten leidet außerdem unter einer ausgeprägten Abgeschlagenheit (Fatigue) [4], die das subjektiv schwerwiegendste Symptom darstellen kann. Die Prävalenz von depressiven Störungen ist ebenfalls erhöht [5]. Auch wenn neue therapeutische Angriffspunkte und B-Zell-gerichtete Therapien, die sich derzeit in klinischer Testung befinden, auf eine Verbesserung der Behandlungsmöglichkeiten hoffen lassen, bleibt die Heterogenität des Krankheitsbildes und der Symptome eine Herausforderung in Klinik und Forschung.

Abstract

Sjögren’s syndrome is a chronic autoimmune disease involving both lacrimal and salivary glands. Lymphocytic infiltration and secondary destruction of the exocrine gland tissue leads to sicca symptoms. It is one of the 3 most common autoimmune diseases with a prevalence of 0.03–2.1%, depending on age and population groups as well as the diagnostic criteria used, and mostly affects women (ca. 10:1) [1] [2] [3].

Apart from the characteristic sicca symptoms many patients also suffer from systemic manifestations such as arthralgia or, less frequently, skin vasculitis, interstitial lung disease or kidney involvement. Sicca symptoms are usually treated locally whereas non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids and immunosuppressants are used for complications.

Sjögren’s syndrome is associated with an increased risk of developing a malignant lymphoma. In addition, most patients suffer from severe fatigue [4], which may be the most severe subjective symptom. The prevalence of depressive disorders is increased as well [5].

Even though promising new therapeutic approaches and B-cell-targeted therapies are currently under clinical investigation, the heterogeneity of the disease and its symptoms continues to be challenging for researchers and physicians alike.

Schlüsselwörter

Sjögren-Syndrom - Diagnostik - Therapie

Key words

Sjögren’s syndrome - diagnostics - therapy
 

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