Clinical characteristics and therapeutic management of adult patients with Sjögren’s syndrome

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  • Azuma Naoto
    Division of Rheumatology, Department of Internal Medicine, Hyogo College of Medicine

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  • 成人のシェーグレン症候群の特徴と治療

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Abstract

<p>　Sjögren’s syndrome (SS) is a chronic inflammatory autoimmune disease primarily characterized by lymphocyte-mediated destruction of the exocrine glands, resulting in dry eye and dry mouth. The inflammatory process can affect any extraglandular organ. Therefore, in addition to the common dryness signs and symptoms, systemic manifestations may occur during the evolution of the disease. SS is associated with other autoimmune rheumatic diseases, such rheumatoid arthritis and systemic lupus erythematosus. When extraglandular manifestations developed, the distinction between SS-induced and other connective tissue disease-induced may be difficult in some patients. Thus, SS has various clinical conditions. The therapeutic management for patients with SS is based on the management of both sicca and systemic manifestations. The management of extraglandular manifestations must be tailored to the organs involved.<br>　Recently, new wave in management of SS has emerged. First, SS has been targeted for medical expenses subsidy as specified incurable disease from January 2015 in Japan, if patients satisfy the diagnostic and disease severity criteria. Then, new ACR-EULAR classification criteria were published in 2016. Finally, in 2017, the evidence-based practice guideline for SS was published by the research team of Ministry of Health, Labour and Welfare. Adequate understanding of diagnostic criteria, disease activity assessment indexes, characterization of glandular and extraglandular manifestations and therapeutic effects of medications including biologics, is necessary for accurately grasping clinical conditions and therapeutic planning. In addition, there are also necessary to perform research appropriately.</p>

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