Update zum Sjögren-Syndrom: Diagnostik & Therapie

Zusammenfassung

Mit einer Prävalenz von circa 1:100 bis 1:400 ist das Sjögren-Syndrom die häufigste Kollagenose und betrifft hauptsächlich Frauen. Bei der Erkrankung steht die Destruktion der exokrinen Drüsen im Vordergrund und führt so regelhaft zur Sicca-Symptomatik. In über 50% der Fälle kommt es auch zu extraglandulären Manifestationen. Insbesondere Fatigue und Arthralgien aber auch verschiedene systemische Organmanifestationen wie Polyneuropathien oder interstitielle Lungenerkrankungen können auftreten. PatientInnen mit bestimmten Risikofaktoren haben eine Wahrscheinlichkeit von fünf Prozent ein Lymphom zu entwickeln. Die Diagnostik orientiert sich an den aktuellen ACR/EULAR-Klassifikationskriterien, der Speicheldrüsensonographie, sowie diversen Biomarkern, insbesondere den Antikörpern gegen SSA/Ro, in Verbindung mit der klinischen Symptomatik. Es gibt bislang keine zugelassene systemische immunsuppressive Therapie. Die European League against rheumatism (EULAR) hat Therapieempfehlungen veröffentlicht, die sich an den Empfehlungen für die Therapie des Systemischen Lupus Erythematodes orientieren und je nach klinischer Ausprägung und Manifestation variieren. Verschiedene, durchaus vielversprechende Studien zu unterschiedlichen therapeutischen Ansätzen werden aktuell durchgeführt.

Abstract

With a prevalence of 1:100 to 1:400, Sjögren’s syndrome is the most common connective tissue disease, and it mainly affects women. The disease is characterised by a destruction of the exocrine glands, which usually leads to sicca symptoms. Extraglandular manifestations occur in more than 50% of cases. These include fatigue and arthralgia as well as various systemic organ manifestations such as polyneuropathy or interstitial lung disease. Patients with certain risk factors have a five per cent chance of developing lymphoma. The diagnosis is based on the current ACR/EULAR classification criteria, salivary gland sonography and various biomarkers, in particular anti-SSA/Ro antibodies, in conjunction with clinical symptoms. There is currently no approved systemic immunosuppressive therapy. The European League Against Rheumatism (EULAR) has published treatment recommendations based on the recommendations for the treatment of systemic lupus erythematosus, which vary according to clinical severity and manifestation. Several promising trials of different therapeutic approaches are currently ongoing.

Schlüsselwörter

Sjögren-Krankheit - Sicca-Symptomatik - Speicheldrüsensonographie - Biologicals - Mesenchymale Stammzelltherapie

Key words

Sjögrenʼs disease - Sonography of the salivary gland - Mesenchymal stem cell therapy - Sicca symptoms - Biologics

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