Division of Rheumatology, The Children's Hospital of Philadelphia and Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Curr Opin Rheumatol. 2013 Sep;25(5):651-7. doi: 10.1097/BOR.0b013e328363ed23.
Sjögren syndrome is a chronic autoimmune disease affecting lacrimal and salivary glands that often is accompanied by extraglandular disease manifestations. Although common in adults, the prevalence and prognosis of childhood Sjögren syndrome are unknown, in part due to lack of child-specific diagnostic and classification criteria. This review discusses difficulties in diagnosing childhood Sjögren syndrome and highlights recent findings in Sjögren syndrome treatment and pathogenesis from studies in adults and animal models over the past 18 months.
Studies of rituximab show some therapeutic potential in adult Sjögren syndrome, whereas newer modalities including gene therapy and mesenchymal stem cell transfer are promising. The pathogenesis of Sjögren syndrome is emerging, including roles of T and B lymphocytes, autoantibodies, interferons, and glandular epithelial cells. Specific recent notable findings in Sjögren syndrome pathogenesis include identification of a type II interferon signature in salivary glands of Sjögren syndrome patients, characterization of salivary gland-infiltrating T-cell subsets, and characterization of antimuscarinic acetylcholine receptor type 3 autoantibodies.
Childhood Sjögren syndrome is a poorly defined and underdiagnosed autoimmune disease that requires child-specific criteria in order to study disease burden and prognosis. Studies in adults and animal models continue to elucidate new potential diagnostic and therapeutic targets, which may be relevant for childhood Sjögren syndrome.
干燥综合征是一种慢性自身免疫性疾病，影响泪腺和唾液腺，常伴有外分泌腺疾病表现。尽管在成人中很常见，但儿童干燥综合征的患病率和预后尚不清楚，部分原因是缺乏儿童特异性的诊断和分类标准。本文讨论了儿童干燥综合征诊断中的困难，并重点介绍了过去 18 个月中成人和动物模型研究中干燥综合征治疗和发病机制的最新发现。
利妥昔单抗治疗成人干燥综合征具有一定的疗效，而基因治疗和间充质干细胞移植等新方法也很有前途。干燥综合征的发病机制正在逐步阐明，包括 T 和 B 淋巴细胞、自身抗体、干扰素和腺上皮细胞的作用。干燥综合征发病机制中的一些最近的显著发现包括：在干燥综合征患者的唾液腺中鉴定出 II 型干扰素特征，鉴定出唾液腺浸润性 T 细胞亚群，以及鉴定出抗毒蕈碱乙酰胆碱受体 3 自身抗体。
儿童干燥综合征是一种定义不明确且诊断不足的自身免疫性疾病，需要有儿童特异性标准来研究疾病负担和预后。成人和动物模型的研究继续阐明新的潜在诊断和治疗靶点，这些靶点可能与儿童干燥综合征有关。
