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Pathogenese und Diagnostik des Sjögren-Syndroms

Pathogenesis and diagnosis of Sjögren’s syndrome

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Zusammenfassung

Das Sjögren-Syndrom ist eine häufige Autoimmunerkrankung. Mehrere genetische Risikofaktoren wie STAT-4, ILT6 und der Haplotyp HLA-B8/DR3 konnten identifiziert werden. Zusätzlich wird das Erkrankungsrisiko durch Umweltfaktoren beeinflusst, möglicherweise durch chronische Virusinfekte. In der Pathophysiologie der Erkrankung infiltrieren T- und B-Zellen die Speichel- und Tränendrüsen der Patienten. Durch die Zerstörung der Drüsenzellen durch zytotoxische T-Zellen, Bildung von Zytokinen und Autoantikörpern, kommt es zu einer verminderten Sekretion der Drüsen mit Mund- und Augentrockenheit. Diese wird von den Patienten oft nicht bemerkt. Im Vordergrund können daher extraglanduläre Manifestationen wie Vaskulitis, Polyneuropathie oder Arthritis stehen.

In der Diagnostik des Sjögren-Syndroms muss zunächst die verminderte Drüsenfunktion verifiziert werden, z. B. mit Hilfe des Schirmer-Tests und des Saxon-Tests. Die Bestätigung des Sjögren-Syndroms als Ursache der Trockenheit erfolgt anschließend durch Nachweis von Autoantikörpern gegen Ro (SS-A) und La (SS-B) und/oder durch eine Speicheldrüsenbiopsie.

Abstract

Sjögren’s syndrome is a common autoimmune disorder. Several genetic risk factors such as STAT-4, ILT6 and the haplotype HLA-B8/DR3 have been identified. In addition, there are environmental risk factors, possibly chronic viral infections. In the pathophysiology of Sjögren’s syndrome T and B cells infiltrate the salivary and lacrimal glands. As a consequence of the destruction of glandular cells by cytotoxic T cells, production of cytokines and autoantibodies inhibiting glandular function, the production of saliva and tears is decreased. The feeling of dry eyes and mouth is frequently not noticed by the patients. Therefore, Sjögren’s syndrome should also be considered when extraglandular manifestations such as vasculitis, polyneuropathy or arthritis occur, even when the patients do not complain of dry eyes and mouth.

Establishing the diagnosis of Sjögren’s syndrome requires verification of reduced glandular function, for example using Schirmer’s test and the Saxon test. The confirmation of Sjögren’s syndrome as a cause of sicca syndrome is subsequently performed by the detection of autoantibodies against Ro (SS-A) and La (SS-B) and/or by a salivary gland biopsy.

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  1. Klinik für Immunologie und Rheumatologie, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    T. Witte

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Witte, T. Pathogenese und Diagnostik des Sjögren-Syndroms. Z. Rheumatol. 69, 50–56 (2010). https://doi.org/10.1007/s00393-009-0519-2

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