Abstract
Zusammenfassung. Das primäre Sjögren-Syndrom ist eine autoimmune Erkrankung vornehmlich der exokrinen Drüsen und äussert sich meistens in Sicca-Symptomatik der Augen und des Mundes, jedoch auch von Nase, Rachen, Vagina und Haut. Fatigue und Schmerz sind ebenfalls sehr charakteristisch. Die Erkrankung kann sich jedoch auch systemisch manifestieren, z.B. in Gelenken, Muskeln, Lungen, Nieren, Haut und Nervensystem. Gefürchtet ist die Entwicklung eines Lymphoms, das insbesondere bei Vorliegen von Anti-SSA-(Ro)-positiven Antikörpern, aber auch z.B. bei hohem ESSDAI-Wert, Komplementverbrauch, Zytopenien oder Nachweis von ektopen Keimzentren in der Speicheldrüsenbiopsie erhöht ist. Die Diagnosestellung ergibt sich in der Regel durch Objektivierung der Sicca-Symptomatik (z.B. Schirmertest) und Vorhandensein der typischen Antikörper oder bei typischer Speicheldrüsenbiopsie. Therapeutisch wird die Sicca-Symptomatik primär symptomatisch behandelt (z.B. durch Tränen- und Speichelersatzmittel), erst bei systemischen Manifestationen kommen Glukokortikoide, konventionelle DMARDs oder ggf. Biologika (in der Regel Rituximab) zum Einsatz. Bei sehr schweren Verläufen sind ggf. zusätzlich intravenöse Immunglobuline und Plasmaexchange notwendig.
Abstract. Primary Sjögren’s syndrome is an autoimmune disease that affects primarily the exocrine glands and is mainly characterized by sicca symptoms of the eyes and mouth, but also nose, throat, vagina and skin can be affected. Fatigue and pain are also very characteristic. Systemic manifestations can occur, e.g. in joints, muscles, lungs, kidneys, skin or the nervous system. A feared complication is the development of a lymphoma, the risk being especially high in case of positive anti-SSA-(Ro) antibodies, but also in case of a high ESSDAI score, hypocomplementemia, cytopenia or evidence of ectopic germinal centers in salivary gland biopsies. Diagnosis is principally made by verification of sicca symptoms (e.g. Schirmer’s test) and detection of typical antibodies or a typical gland biopsy. Sicca symptoms are primarily treated symptomatically (e.g. artificial tears or saliva). In case of systemic manifestations glucocorticoids, conventional DMARDs or biologics (mostly rituximab) are treatment options. In severe cases, intravenous immunoglobulins or plasma exchange may be required.
Résumé. Le syndrome de Sjögren primaire est une maladie auto-immune principalement des glandes exocrines qui se manifeste principalement par des symptômes de sécheresse des yeux et de la bouche, mais aussi du nez, de la gorge, du vagin et de la peau. La fatigue et la douleur sont aussi très caractéristiques. La maladie peut également se manifester de manière systémique, par exemple au niveau des articulations, des muscles, des poumons, des reins, de la peau et du système nerveux. On craint le développement de lymphomes, particulièrement en présence d’anticorps anti-SSA-(Ro) positifs, mais aussi, par exemple, en présence de valeurs élevé de l’ESSDAI, de consommation du complément, de cytopénie ou de détection de centres germinaux ectopiques dans les biopsies des glandes salivaires. Le diagnostic est généralement établi par objectivation des symptômes de la maladie (test de Schirmer par exemple) et la présence d’ anticorps typiques ou dans le cas d’une biopsie typique des glandes salivaires. Sur le plan thérapeutique, les symptômes de la sécheresse sont traités principalement de manière symptomatique (par exemple avec des substituts de larmes et de salive). Ce n’est qu’en cas de manifestations systémiques que l’on utilise des glucocorticoïdes, des DMARD classiques ou, si nécessaire, des produits biologiques (généralement le rituximab). Dans les cas très graves, des immunoglobulines intraveineuses et un échange de plasma peuvent également être nécessaires.
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