Department of Rheumatology, Brest Teaching Hospital, Brest, France; EA 2216 Immunology and Pathology, Brest University, SFR ScinBios, Labex 'Immunotherapy, Graft, Oncology', Brest, France.
EA 2216 Immunology and Pathology, Brest University, SFR ScinBios, Labex 'Immunotherapy, Graft, Oncology', Brest, France.
J Autoimmun. 2014 Jun;51:109-14. doi: 10.1016/j.jaut.2014.02.006. Epub 2014 Mar 4.
Primary Sjögren's syndrome (pSS) is one of the most frequent autoimmune systemic diseases, mainly characterized by ocular and oral dryness due to the progressive destruction of lachrymal and salivary glands by an inflammatory process. A noteworthy proportion of patients also features extraglandular manifestations, sometimes severe and life-threatening. Until now, its management relies mostly on symptomatic interventions, long-term monitoring, and, in patients with severe systemic complications, immunosuppressive drugs can be provided. However, recent years have seen great progresses in the understanding of the pathological processes of the disease. The central role of regulatory lymphocytes, the implication of the type 1 interferon pathway in some patients or the importance of epigenetics have been highlighted. New classification criteria have been recently published and have shed in light an international attempt for a better recognition of the patients, probably thanks to the development of new diagnostic procedures such as salivary gland ultrasonography. To facilitate the detection of treatment efficacy in clinical trials and to help in determining which subgroups of patients would have benefits from intensive therapies, a better definition of activity scores and the availability of new prognostic markers are urgent. Thereby, the development of future therapies should be based on specific molecular signatures that will enable a personalized management of each patient. This review focuses on the most striking advances in the fields of pathophysiology, diagnosis and treatment of pSS, which generate a great hope for pSS patients.
原发性干燥综合征（pSS）是最常见的自身免疫性系统性疾病之一，主要表现为眼干和口干，这是由于炎症过程导致泪腺和唾液腺进行性破坏所致。相当一部分患者还存在腺外表现，有时甚至严重且危及生命。迄今为止，其治疗主要依赖于对症干预、长期监测，以及在有严重全身并发症的患者中给予免疫抑制剂。然而，近年来，人们对疾病的病理过程有了更深入的了解。调节性淋巴细胞的核心作用、1 型干扰素途径在某些患者中的作用或表观遗传学的重要性已得到强调。最近公布了新的分类标准，为更好地识别患者提供了国际上的尝试，这可能要归功于新的诊断程序的发展，如唾液腺超声检查。为了在临床试验中更容易检测治疗效果，并有助于确定哪些亚组的患者将从强化治疗中获益，迫切需要更准确地定义活动评分和获得新的预后标志物。因此，未来的治疗方法应基于特定的分子特征，以便对每位患者进行个性化治疗。这篇综述重点介绍了在 pSS 的病理生理学、诊断和治疗领域的最显著进展，这为 pSS 患者带来了新的希望。
