Abstract

Primary Sjögren's syndrome (pSS) is an autoimmune disease affecting the salivary and lachrymal glands. The patients complain of symptoms of dry eyes and dry mouth, but up to 50 % may additionally develop extraglandular manifestations such as arthritis, vasculitis, polyneuropathy, pulmonary fibrosis or interstitial nephritis. Most therapeutic studies on the glandular manifestations of pSS failed to meet their primary endpoints, possibly since many of the patients had already advanced and irreversible disease.In recent years several important advances have been made. The role of B and T lymphocytes in the pathogenesis of pSS has been identified. The disease activity scores ESSDAI and ESSPRI were developed and can now be used as endpoints in therapeutical studies. Since then, numerous promising new drugs, mostly affecting B and T lymphocytes, have been studied in clinical trials. New classification criteria have been proposed, which will be an important tool in making a diagnosis in an early disease stage, in which the glandular function may still be normal. It is very likely therefore, that we will soon have efficient therapies, which will be able to stop the disease progression in an early stage of pSS.

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