Goules Andreas V, Tzioufas Athanasios G
Department of Pathophysiology, School of Medicine, University of Athens, Mikras Asias Str 75, 115 27, Athens, Greece.
Department of Medicine, Johns Hopkins University, Baltimore, MD, USA.
Immunol Res. 2017 Feb;65(1):331-344. doi: 10.1007/s12026-016-8844-4.
Primary Sjögren's syndrome is a complex, autoimmune disease with distinct clinical phenotypes and variable outcomes. The systemic form of the disease is characterized by immune complex-mediated manifestations and is complicated by lymphoma as a result of a polyclonal B cell hyperactivity that is evolving into B cell malignancy. In the past decades, well-established clinical and serological markers have been described in the literature to identify high-risk patients and predict lymphoma development. However, specific biological treatments have proven ineffective to control the disease. Significant research effort has been made to reveal the major underlying biological events in this subgroup and identify biomarkers for early diagnosis, prognosis and response to treatment. In this review, we summarize the current data for the proposed histological, molecular and genetic biomarkers.
原发性干燥综合征是一种复杂的自身免疫性疾病，具有独特的临床表型和多样的预后。该疾病的系统性形式以免疫复合物介导的表现为特征，并因多克隆B细胞过度活跃演变为B细胞恶性肿瘤而并发淋巴瘤。在过去几十年中，文献中已描述了成熟的临床和血清学标志物，用于识别高危患者并预测淋巴瘤的发生。然而，特定的生物治疗已被证明无法有效控制该疾病。人们已付出巨大研究努力来揭示该亚组中的主要潜在生物学事件，并确定早期诊断、预后及治疗反应的生物标志物。在本综述中，我们总结了目前关于所提出的组织学、分子和遗传生物标志物的数据。
