Neurologische Manifestationen bei Patienten mit primärem Sjögren-Syndrom - Eine differenzialdiagnostische und therapeutische Herausforderung

Zusammenfassung

Das primäre Sjögren-Syndrom (pSS) ist eine Autoimmunerkrankung, die durch eine chronische lymphozytäre Infiltration der exokrinen Drüsen gekennzeichnet ist, welche zu einer Keratokonjunctivitis sicca sowie einer Stomatitis sicca führt. Im Rahmen von extraglandulären Manifestationen kann es zu einer Beteiligung des Nervensystems kommen. Während eine Beteiligung des peripheren Nervensystems mittlerweile allgemein anerkannt ist, wird eine Beteiligung des zentralen Nervensystems bezüglich der Frequenz, der Bedeutung sowie der Ätiologie kontrovers diskutiert. Zahlreiche Fallstudien, oft jedoch mit kleinen Fallzahlen, verdeutlichen die Vielfalt neurologischer Manifestationen. Das klinische Spektrum reicht von einer milden Neuropathie bis hin zu schwerwiegenden fokalen neurologischen Symptomen. Die Diagnose von neurologischen Manifestationen kann nur interdisziplinär durch Kooperation des Rheumatologen mit dem Neurologen erfolgen und sollte neuere laborchemische und bildgebende Verfahren nutzen. Dieser Artikel gibt eine Übersicht über etablierte und neuere Erkenntnisse bezüglich der Inzidenz, Ätiologie, Diagnostik sowie der Therapie von neurologischen Manifestationen beim pSS.

Abstract

Primary Sjögren’s syndrome (pSS) is an autoimmune disorder characterized by chronic lymphocytic infiltration of exocrine glands leading to keratoconjunctivitis sicca and Stomatitis sicca. Extraglandular manifestations may include the nervous system. While involvement of the peripheral nervous system is generally accepted, affection of the central nervous system is a matter of discussion as to frequency, significance and etiology. Various studies, although often with small numbers of cases, underline the diversity of neurologic complications of pSS. The clinical picture ranges from mild neuropathy to severe acute focal symptoms. For the diagnosis of neurologic manifestations, cooperation of specialists in rheumatology and neurology is pivotal and should include the latest laboratory and imaging techniques. This review presents both established and new knowledge of the incidence, etiology, diagnostics, and treatment of neurological manifestations in pSS.

Schlüsselwörter

Primäres Sjögren-Syndrom - neurologische Manifestationen - ZNS-Beteiligung - Polyneuropathie

Key words

Primary Sjögren’s syndrome - neurologic manifestations - CNS involvement - polyneuropathy

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