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Vaskulopathien bei Sjögren-Syndrom

Vasculopathy in Sjögren’s syndrome

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Zusammenfassung

Das Sjögren-Syndrom ist eine systemische Autoimmunerkrankung mit im Vordergrund stehender Beteiligung der exokrinen Drüsen und resultierender Sicca-Symptomatik. Darüber hinaus kommt es in etwa 40% der Fälle zu extraglandulären Manifestationen mit Beteiligung der Haut, des muskuloskelettalen Systems, des peripheren und zentralen Nervensystems sowie auch innerer Organe. Das Auftreten einer systemischen Vaskulitis bei Sjögren-Syndrom kann bei etwa 5–10% der Patienten angenommen werden. Eine klassische vaskulitische Manifestation des Sjögren-Syndroms stellen die leukozytoklastische und kryoglobulinämische Vaskulitis dar, wobei in der Pathogenese insbesondere B-Zell-vermittelte Autoimmunreaktionen bei Nachweis von Autoantikörpern gegen Ro/SS-A und La/SS-B sowie Kryoglobuline eine Rolle spielen. Im Allgemeinen können das Auftreten einer Vaskulitis bei Sjögren-Syndrom sowie der Nachweis von Kryoglobulinen, eine Komplementerniedrigung, das Auftreten eines Non-Hodgkin-Lymphoms und eine Glomerulonephritis als ungünstige prognostische Faktoren angesehen werden. Bei Vaskulitis können verschiedene immunsuppressive Therapieansätze in der Regel in Komedikation mit Glukokortikoiden eingesetzt werden. Für therapieresistente Verläufe kann darüber hinaus die Option einer B-Zell-gerichteten Therapie unter Einsatz von Rituximab erwogen werden.

Abstract

Sjögren’s syndrome is a systemic autoimmune disease with a predominant involvement of exocrine glands leading to sicca symptoms. Extraglandular involvement occurs in about 40% of patients with skin, musculoskeletal, neurological and organ manifestations. Systemic vasculitic manifestations of Sjögren’s syndrome can be assumed in approximately 5%–10% of patients. Leukocytoclastic or cryoglobulinemic vasculitis represent classic vasculitic manifestations of Sjögren’s syndrome. In the pathogenesis of vasculitis, B-cell-driven autoimmune processes play a major role by producing autoantibodies against the Ro/SS-A and La/SS-B antigens and cryoglobulins. In patients with Sjögren’s syndrome, manifestation of vasculitis, non-Hodgkin’s lymphoma and glomerulonephritis, as well as positive cryoglobulins and decreased levels of complement factors, are considered negative prognostic markers. Various immunosuppressive strategies, usually in co-medication with glucocorticoids, are used for the treatment of vasculitis in Sjögren’s syndrome. For refractory and severe manifestations, a B-cell-targeted therapy with Rituximab should be also considered.

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Authors and Affiliations

  1. Klinik für Rheumatologie und Klinische Immunologie, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland

    E. Feist

  2. Institut für Radiologie, Charité-Universitätsmedizin Berlin, Berlin, Deutschland

    K.-G. A. Hermann

  3. Institut für Pathologie, Charité-Universitätsmedizin Berlin, Berlin, Deutschland

    A. Dankof

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  1. E. Feist
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  2. K.-G. A. Hermann
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  3. A. Dankof
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Correspondence to E. Feist.

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Feist, E., Hermann, KG. & Dankof, A. Vaskulopathien bei Sjögren-Syndrom. Z. Rheumatol. 68, 305–311 (2009). https://doi.org/10.1007/s00393-008-0400-8

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