Dong Lingli, Chen Yu, Masaki Yasufumi, Okazaki Toshiro, Umehara Hisanori
Department of Hematology and Immunology, Tongji Hospital, Huazhong University of Science and Technology, Wuhan, Hubei, 430030, China.
Curr Immunol Rev. 2013 Feb;9(1):13-22. doi: 10.2174/1573395511309010003.
Primary Sjögren's syndrome (pSS) is a systemic as well as an organ-specific autoimmune disease characterized by lymphocytic infiltration of the glandular epithelial tissue. SS patients have been reported to be at highest risk of developing lymphoproliferative neoplasms, when compared with patients with other rheumatoid diseases. Factors such as cytokine stimulation, environmental factors, viral infection and genetic events as well as vitamin deficiency may contribute to the development of lymphoma. Over the past few decades, numerous efforts have been made to assess the relationship between lymphoma and SS. These include epidemiological surveys, molecular biologic assessments of clonality and well-linked register cohort studies evaluating the predictive value of clinical, laboratory and histological findings. Nevertheless, the mechanisms and factors predictive of lymphoma development in pSS patients remain to be defined. This review summarizes updated knowledge on the incidence of and risk factors for lymphoma development in pSS patients, as well as discussing the most recent findings on the development and treatment of lymphoma in pSS patients and the possible mechanism of lymphoma development.
原发性干燥综合征（pSS）是一种全身性以及器官特异性自身免疫性疾病，其特征为腺上皮组织的淋巴细胞浸润。据报道，与其他类风湿性疾病患者相比，SS患者发生淋巴增殖性肿瘤的风险最高。细胞因子刺激、环境因素、病毒感染、遗传事件以及维生素缺乏等因素可能促使淋巴瘤的发生。在过去几十年中，人们为评估淋巴瘤与SS之间的关系付出了诸多努力。这些努力包括流行病学调查、克隆性的分子生物学评估以及关联良好的登记队列研究，以评估临床、实验室和组织学检查结果的预测价值。然而，pSS患者淋巴瘤发生的机制和预测因素仍有待明确。本综述总结了pSS患者淋巴瘤发生率及发生风险因素的最新知识，同时讨论了pSS患者淋巴瘤发生与治疗的最新研究结果以及淋巴瘤发生的可能机制。
