Zusammenfassung
Das primäre Sjögren-Syndrom ist eine entzündlich-rheumatische, autoimmune Systemerkrankung, die charakteristischerweise die exokrine Funktion von Tränen- und Speicheldrüsen beeinträchtigt. Die Ansichten über die Prävalenz und Inzidenz der Erkrankungen gehen teilweise erheblich auseinander. Der erste Teil der vorliegenden Arbeit fasst die bekannten epidemiologischen Daten zusammen. Wir versuchen anhand der 10 wichtigsten Publikationen, die Studien nach einem eigens entwickelten Punktesystem zu bewerten. Der zweite Teil des Artikels diskutiert die wissenschaftliche Evidenz über die Prognose des primären Sjögren-Syndroms. Dabei werden insbesondere zwei neuere Studien, eine griechische retrospektive und eine prospektive schwedische Studie, analysiert. Wir werden zeigen, dass sich in den Studienpopulationen keine signifikant erhöhte Mortalität (bezogen auf alle Todesursachen) ergeben hat, eine Subgruppe mit niedrigen C3 und/oder C4 hat ein erhöhtes Risiko, an einem Non-Hodgkin-Lymphom zu versterben.
Abstract
The primary Sjögren’s syndrome is an autoimmun, systemic inflammatory disease with characteristic deterioration of the exocrine function of the lachrymal and salivary glands. The opinions about prevalence and incidence of the disease are conflicting. The first part of the article will focus on the known epidemiologic data. It was shown to characterize the quality of the 10 most important studies dealing with this aspect by using a self developed scoring system. The second part of this paper will discuss the scientific evidence of prognosis of primary Sjögren’s syndrome with special regard to two big newer studies in a Greek (retrospective) and a Swedish (prospective) cohort. We will show that in contrast to earlier studies there was no statistical increase in all-cause mortality in the study populations.
Schlüsselwörter
Primäres Sjögren-Syndrom - Epidemiologie - Mortalität - Non-Hodgkin-Lymphom - Prognose
Key words
Primary Sjögren’s syndrome - epidemiology - mortality - Non-Hodgkin-Lymphoma - prognosis
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Rolf Manthorpe
Sjögren’s Syndrome Research Centre, The house doctors
Jägersro vägen 40
SE-212 37 Malmö
Schweden
Email: Manthorpe@inet.uni2.dk
