Manoussakis M N, Moutsopoulos H M
Department of Pathophysiology, National University of Athens, School of Medicine, Greece.
Lupus. 1996 Jun;5 Suppl 1:S28-30.
Sjögren's syndrome (SS) is a chronic autoimmune rheumatic disorder characterized by lymphocytic infiltration and destruction of exocrine glands, mainly of salivary and lacrimal glands, leading to dryness of mouth and eyes. It can occur either alone (primary SS) or in association with almost every systemic autoimmune rheumatic SS). Usually, SS patients have slowly progressive disease confined in exocrine glands, however, in approximately one third of primary SS patients the disorder presents a systemic and progressive course with involvement of diverse extraglandular sites and in a small but significant number of patients with lymphoid neoplasia development. Although the aetiology of SS remains unknown, chronic immune system stimulation is thought to play a central role in the pathogenesis of the disorder, as illustrated by several indices of immunological hyperactivity, including various autoantibodies, polyclonal hypergammaglobulinemia and circulating paraproteins. To date, treatment of SS remains largely empirical and symptomatic, and no clinical trial has been proved capable to change the course of the disease. Hydroxychloroquine has been successfully applied for the treatment of arthralgias, myalgias and general constitutive symptoms of SS patients. In an initial small open study, hydroxychloroquine administration has been shown to improve features of immunological hyperreactivity, such as hypergammaglobulinaemia and autoantibody levels. However, large prospective double-blind studies are still needed to assess the long-term efficacy of hydroxychloroquine in preventing extraglandular involvement and lymphoma development in primary SS patients.
干燥综合征（SS）是一种慢性自身免疫性风湿性疾病，其特征是淋巴细胞浸润并破坏外分泌腺，主要是唾液腺和泪腺，导致口干和眼干。它可以单独出现（原发性SS），也可与几乎所有系统性自身免疫性风湿性疾病相关（继发性SS）。通常，SS患者的疾病进展缓慢，局限于外分泌腺，然而，在大约三分之一的原发性SS患者中，该疾病呈现系统性和进行性病程，累及不同的腺外部位，并且在少数但数量可观的患者中会发生淋巴样肿瘤。尽管SS的病因尚不清楚，但慢性免疫系统刺激被认为在该疾病的发病机制中起核心作用，多种免疫功能亢进指标可以证明这一点，包括各种自身抗体、多克隆高球蛋白血症和循环副蛋白。迄今为止，SS的治疗在很大程度上仍然是经验性和对症性的，尚无临床试验被证明能够改变疾病进程。羟氯喹已成功应用于治疗SS患者的关节痛、肌痛和一般全身症状。在一项初步的小型开放性研究中，已表明给予羟氯喹可改善免疫功能亢进的特征，如高球蛋白血症和自身抗体水平。然而，仍需要大型前瞻性双盲研究来评估羟氯喹在预防原发性SS患者腺外受累和淋巴瘤发生方面的长期疗效。
