Zhang W, Chen Z, Li X M, Gao J, Zhao Y
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology,State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education,Beijing 100730, China.
Department of Rheumatology and Immunology, the First Affiliated Hospital of University of Science and Technology of China, Anhui Provincial Hospital, Hefei 230001, China.
Zhonghua Nei Ke Za Zhi. 2023 Sep 1;62(9):1059-1067. doi: 10.3760/cma.j.cn112138-20221027-00797.
Sjögren's syndrome (SS) is a chronic systemic autoimmune disease characterized by lymphocyte proliferation and progressive exocrine gland damage. In addition to the impairment of salivary and lacrimal gland function, SS can present with multi-system and multi-organ involvement, accompanied by autoantibodies in serum and hyperimmunoglobulinemia. SS can be divided into primary and secondary forms based on the absence or presence, respectively, of concurrent connective tissue diseases such as systemic lupus erythematosus and rheumatoid arthritis. Based on evidence and guidelines from China and other countries, the Chinese Rheumatology Association drafted standardized criteria for the diagnosis and treatment of primary SS. The objectives were to standardize the detection and interpretation of key indicators for the diagnosis of SS, including serum anti-SSA antibody and labial gland pathology, suggest the use of the widely accepted European League Against Rheumatism (EULAR)-SS disease activity index for the evaluation of the disease, and standardize the rational management of SS patients with topical and systemic therapies.
干燥综合征(SS)是一种慢性全身性自身免疫性疾病,其特征为淋巴细胞增殖和进行性外分泌腺损伤。除了唾液腺和泪腺功能受损外,SS还可出现多系统和多器官受累,并伴有血清自身抗体和高免疫球蛋白血症。根据是否并发系统性红斑狼疮和类风湿关节炎等结缔组织病,SS可分为原发性和继发性两种形式。基于中国和其他国家的证据及指南,中华医学会风湿病学分会起草了原发性SS的诊断和治疗标准。目的是规范SS诊断关键指标的检测和解读,包括血清抗SSA抗体和唇腺病理,建议使用广泛认可的欧洲抗风湿病联盟(EULAR)-SS疾病活动指数评估疾病,并规范SS患者局部和全身治疗的合理管理。