Zusammenfassung
Das primäre Sjögren-Syndrom (PSS) ist eine nicht ganz seltene Autoimmunerkrankung mit einer Prävalenz zwischen 0,2 % und 2 % und gekennzeichnet durch Xerophthalmie mit verminderter Tränendrüsensekretion (pathologischer Schirmer-Test), Xerostomie mit verminderter Speicheldrüsensekretion (verminderte Nuklidaufnahme in der Speicheldrüsenszintigraphie), SS-A (Ro) oder SS-B(La)-Antikörpern und dem histopathologischen Nachweis mononukleärer Zellen in der Speicheldrüsenbiopsie. Daneben kommt es beim Sjögren-Syndrom in einem nicht unerheblichen Anteil zu neurologischen Manifestationen, die das periphere (sensible Polyneuropathie, autonome Polyneuropathie, Hirnnervenneuropathie) und zentrale Nervensystem (Myelitis, chronische Myelopathie, Optikusneuritis, zerebrale Ischämien, psychiatrische Symptome) betreffen. Da die neurologischen Befunde zunächst ganz im Vordergrund des klinischen Bildes stehen können, ist die Differenzialdiagnose eines PSS häufig zu berücksichtigen.
Abstract
The primary Sjögren-syndrome (PSS) is a chronic autoimmune disorder with a prevalence of 0.2 % and 2 % that is manifestated clinically by xerophthalmia with reduced lacrimation (pathological Schirmer's test), xerostomia with reduced salivary flow (pathological salivary scintigraphy), antibodies to Ro/SSA and La/SSB, labial salivary gland biopsy with mononuclear infiltrations. In a considerable percentage, PSS is associated with neurological complications which may affect the peripheral nervous system (sensory polyneuropathy, autonomic polyneuropathy, cranial nerve neuropathy) and to a lesser extent the central nervous system (acute myelitis, progressive myelopathy, optic neuritis, ischemic infarction, psychiatric disturbances). Since neurological findings may dominate the clinical picture, PSS should be considered frequently as differential diagnosis.
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PD Dr. Peter P. Urban
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