Praktisches Management der Sjögren-Erkrankung im Zeitalter neuer Therapieoptionen

Sjögren's disease is a chronic inflammatory autoimmune disease, which mostly affects women who suffer particularly from dryness of mucous membranes, especially in the eyes and the mouth, tiredness and potentially also extraglandular manifestations such as joint and muscle pain. In the initial diagnostic work up serological alterations especially autoantibodies against SS-A(Ro)/SS-B(La) and rheumatoid factors play an important role. The pathogenesis is not yet finally clarified but an important role of B cells can be assumed. To date mostly symptomatic treatment options to improve glandular functional deficits with respect to the sicca symptoms are available. Based on most recent publications there is justifiable hope that this could soon change, especially with respect to extraglandular manifestations. For this reason, it is particularly important that validated outcome parameters are available to enable proper evaluation of new treatment options. In this article the EULAR instruments Sjögren's syndrome disease activity index (ESSDAI) for documenting extraglandular manifestations and Sjögren's syndrome patient reported index (ESSPRI) as patient-reported outcome (PRO) have been translated and are here presented in detail. Both also represent the cornerstones of possible outcome instruments (STAR or CRESS) of the second generation, which are currently undergoing validation.