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YAO Hong-liang, ZHU Ke-jian. Department of Dermatology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310016, China Corresponding author: ZHU Ke-jian, Email: zhukejiansrrsh@126.com ��Abstract�� Sjogren′s syndrome ��SS�� is a chronic autoimmune inflammatory disease characterized by predominant infiltration of lymphocytes in exocrine glands as well as presence of several autoantibodies in sera. Primary SS ��pSS�� is the condition in the absence of other definitively diagnosed autoimmune diseases. The pathogenesis of pSS remains unclear. Currently, pSS is considered to be induced by multiple environmental factors and endogenous antigens, which trigger a self-perpetuating inflammatory response in susceptible individuals, lead to abnormal cellular and humoral immunity followed by the impairment of exocrine glands, production of autoantibodies and damage to target organs. Although no ideal therapy is available, biological agents have offered new clues to the treatment of pSS. ��Key words�� Sjogren's syndrome; Diagnosis; Therapy