• A Björk, J Mofors, and M Wahren-Herlenius.
• From the, Division of Rheumatology, Department of Medicine, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.
• J. Intern. Med. 2020 May 1; 287 (5): 475-492.

AbstractPrimary Sjögren's syndrome (SS) is a systemic autoimmune disease in which exocrine organs, primarily the salivary and lacrimal glands, are targets of chronic inflammation, leading to severe dryness of eyes and mouth. Fatigue and arthralgia are also common, and extraglandular manifestations involving the respiratory, nervous and vascular systems occur in a subset of patients. Persistent activation of the type I interferon system, and autoreactive B and T cells with production of disease-associated autoantibodies are central to the pathogenesis. Genetic polymorphisms that associate with an increased risk of SS have been described, though the risk-increase contributed by the respective variant is generally low. It is thus becoming increasingly clear that genetics cannot alone account for the development of SS and that other, presumably exogenous, factors must play a critical role. Relatively few studies have investigated exposure to potential risk factors prior to SS disease onset. Rather, many factors have been studied in prevalent cases. In this review, we summarize current literature on exogenous factors in the pathogenesis of SS including infections, hormones, smoking, solvents and additional compounds. We delineate for which factors there is current evidence of increased disease risk, and for which our present knowledge is confined to suggesting their role in SS pathogenesis. Finally, we outline future perspectives in the continued search for environmental risk factors for SS, a research area of great importance considering the possibilities for preventive measures.© 2020 The Association for the Publication of the Journal of Internal Medicine.

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