Price Elizabeth J, Baer Alan N
Department of Rheumatology, Great Western Hospital NHS Foundation Trust, Swindon, UK.
Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Rheumatology (Oxford). 2021 Jun 18;60(6):2574-2587. doi: 10.1093/rheumatology/key363.
SS is a chronic, autoimmune disease of unknown aetiology for which there is no known curative treatment. Although dryness of the eyes and mouth are the classically described features, patients often experience drying of other mucosal surfaces and systemic manifestations, including fatigue and arthralgia. There is an association with other autoimmune diseases, especially thyroid disease, coeliac disease and primary biliary cholangitis. Systemic features may affect up to 70% and include inflammatory arthritis, skin involvement, haematological abnormalities, neuropathies, interstitial lung disease and a 5-10% lifetime risk of B cell lymphoma. Treatment should aim to empower patients to manage their condition; conserve, replace and stimulate secretions; prevent damage; and suppress underlying systemic disease activity.
干燥综合征是一种病因不明的慢性自身免疫性疾病，目前尚无已知的治愈方法。虽然眼睛和口腔干燥是其典型症状，但患者常伴有其他黏膜表面干燥及全身症状，如疲劳和关节痛。它与其他自身免疫性疾病有关，尤其是甲状腺疾病、乳糜泻和原发性胆汁性胆管炎。全身症状可能影响多达70%的患者，包括炎性关节炎、皮肤受累、血液学异常、神经病变、间质性肺病以及5%-10%的终生患B细胞淋巴瘤风险。治疗应旨在帮助患者控制病情；保存、替代和刺激分泌物；预防损伤；并抑制潜在的全身疾病活动。
