Prognose des primären Sjögren-Syndroms unter besonderer Berücksichtigung des Lymphomrisikos

Zusammenfassung

Das primäre Sjögren-Syndrom ist eine chronische Autoimmunepithelitis, die Speichel-, Tränen- und andere exokrine Drüsen befällt. Extraglanduläre Manifestationen sind häufig, jedoch selten bedrohlich. Die Sicca-Symptomatik ist häufig chronisch progredient, wobei die Progredienz am stärksten im Zeitraum von der Erstmanifestation bis zur Diagnosestellung zu sein scheint. Das klinische Gesamtbild wie auch die individuelle serologische Konstellation einschließlich der Manifestationen ist sehr variabel und hat sich in Langzeituntersuchungen vom Zeitpunkt der Diagnosestellung an als sehr stabil erwiesen, sie verändern sich kaum im Vergleich zum Zeitpunkt der Diagnosestellung. Letzteres erlaubt möglicherweise eine Risikoabschätzung bereits bei Diagnosestellung. Die Lebenserwartung der Erkrankten ist mit Ausnahme einer kleinen Patientengruppe nicht reduziert. Das primäre Sjögren-Syndrom prädisponiert mehr als alle anderen Kollagenosen zu Malignomen des hämatopoetischen Systems, insbesondere treten gehäuft Non-Hodgkin-Lymphome auf. Die vorliegende Arbeit beleuchtet unterschiedliche Aspekte des Langzeitverlaufs im Hinblick auf Prognose, Lebenserwartung und Lymphomgefährdung. Daraus abgeleitet werden eine Risikoeinschätzung und die resultierende Intensität der Langzeitüberwachung der Patienten vorgeschlagen.

Abstract

Primary Sjögren’s syndrome is a chronic autoimmune epithelitis which affects salivary, lacrimal and other exocrine glands. Extraglandular manifestations are common but seldom dangerous and threatening. The sicca symptoms are often slowly chronic-progressive whereby the progress seems to be most striking during the period between first manifestation and confirmation of diagnosis. The clinical course as well as the serological constellations and the extraglandular manifestations seem to be rather variable but stable during the long-term follow-up from the time of diagnostic confirmation onwards with no drastic changes compared with the status when the diagnosis was fixed. This probably allows an initial prognostic stratification at the time of the first consultation. The life expectancy of patients with Sjögren’s syndrome is, except for a minority of patients, as good as that of the age-adjusted population. In comparison with other collagen diseases, the predisposition for the development of non-Hodgkin’s lymphoma is strikingly high in primary Sjögren’s syndrome. This paper discusses different aspects of prognosis with special consideration of the life expectancy and lymphoma development. Finally, a practical mode of long-term medical care for patients with Sjögren’s syndrome is proposed with special regard to risk stratification and intensity of long-term monitoring.

Schlüsselwörter

Sjögren-Syndrom - Lymphom - Prognose - Lymphomscreening - Exokrine Funktion

Key words

Sjögren’s syndrome - lymphoma - prognosis - screening for lymphoma - exocrine function

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