Diagnose und therapie des Sjögren-syndroms

Abstract

Sjögren's syndrome is the most frequent collagen vascular disease affecting about 0.2 % of the population. Xerophthalmia and xerostomia are the predominant symptoms of Sjögren's syndrome. These symptoms are very common in clinical practice and therefore should be verified by specific investigations such as the Schirmer- and Saxon-test before Sjögren's syndrome is considered. La-antibodies and with less certainty Ro-antibodies are specific for Sjögren's syndrome, but both antibodies are present only in about half of the patients. If Sjögren's syndrome is suspected clinically and high titre of specific antibodies are missing and if conditions such as interstitial lung disease, elevated erythrocyte sedimentation rate, hypergammaglobulinemia, or hypocomplementemia cannot be explained by another disease, salivary gland biopsy should be performed. In up to 90 % of patients Sjögren's syndrome runs a favourable course with low disease activity. Therefore symptomatic local therapeutics and/or oral application of pilocarpine and/or non-steroidal antirheumatic drugs are usually sufficient to cope with the clinical condition. Sicca-symptoms refractory to symptomatic therapy and/or pulmonary involvement, CNS inflammation and/or polyneuropathy as well as small vessel vasculitis require immunosuppressive therapy. So far clinical trials of high quality are only available with rituximab, a B-cell depleting chimeric mouse-human antibody. Rituximab was able to improve subjective and objective parameters of salivary gland dysfunction and organ involvement albeit with the risk of developing serum sickness-like disease.