Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Via Roma 67, 56126 Pisa, Italy.
Fondazione Policlinico Universitario Campus Bio-Medico, Via Alvaro del Portillo, 200, 00128 Roma, Italy; Research Unit of Immuno-Rheumatology, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Via Alvaro del Portillo, 21, 00128 Roma, Italy.
Autoimmun Rev. 2024 Jan;23(1):103425. doi: 10.1016/j.autrev.2023.103425. Epub 2023 Aug 25.
Sjӧgren's disease (SjD) is a systemic autoimmune disorder characterized by the chronic inflammation and dysfunction of exocrine glands, mainly salivary glands, causing dryness of the eyes and of the mouth. The disease may affect different organs and tissues with complex and heterogeneous clinical presentation, usually with sicca symptoms, profound fatigue, chronic pain, major organ involvement, and lymphomas. SjD diagnosis is based on the combination of clinical, serological, and functional tests with histological biomarkers. Minor salivary gland biopsy (mSGB) represents the cornerstone for the diagnosis of SjD, allowing the study of the characteristic focal infiltration of B- and T lymphocytes. Besides, mSGB might also have a prognostic role, being the infiltrates more complex in patients with severe SjD. But biopsy, so far, is not mandatory for SjD and mSG ultrasound and peripheral biomarkers might replace its role in the future. Another important aspect of SjD is the presence of autoantibodies, although 20 to 30% of patients are "seronegative" for specific autoantibodies (ANA, antiRo/SSA, antiLa/SSB). The characteristics of this subset of patients are currently under evaluation and "new" autoantibodies and biomarkers might be necessary for better patient's stratification and follow-up.
干燥综合征（SjD）是一种系统性自身免疫性疾病，其特征为外分泌腺（主要为唾液腺）的慢性炎症和功能障碍，导致眼干和口干。该疾病可能会影响不同的器官和组织，具有复杂且异质的临床表现，通常伴有干燥症状、极度疲劳、慢性疼痛、重要器官受累和淋巴瘤。SjD 的诊断基于临床、血清学和功能测试与组织学生物标志物的结合。唇腺活检（mSGB）是 SjD 诊断的基石，可用于研究特征性的 B 细胞和 T 淋巴细胞灶性浸润。此外，mSGB 可能还具有预后作用，在 SjD 严重的患者中浸润更为复杂。但是，活检目前并非 SjD 的强制性检查，mSG 超声和外周生物标志物可能会在未来取代其作用。SjD 的另一个重要方面是自身抗体的存在，尽管 20%至 30%的患者针对特定自身抗体（ANA、抗 Ro/SSA、抗 La/SSB）呈“血清阴性”。目前正在对这组患者的特征进行评估，可能需要“新”的自身抗体和生物标志物来更好地对患者进行分层和随访。
