Department of Internal Medicine.
Department of Rheumatology, CHU Rennes, University of Rennes 1.
Rheumatology (Oxford). 2023 Feb 6;62(SI):SI1-SI11. doi: 10.1093/rheumatology/keac412.
SSc is an auto-immune disease characterized by life-threatening manifestations such as lung fibrosis or pulmonary arterial hypertension. Symptoms with a detrimental impact on quality of life are also reported and sicca syndrome (xerostomia, xeropthalmia) is present in up to 80% of patients with SSc. Sicca syndrome can occur in the absence of overlap with Sjögren's disease and recent studies highlight that fibrosis of minor and major salivary glands, directly linked to the pathogenesis of SSc, could be a major contributor of xerostomia in SSc. This narrative review provides an overview of the clinical presentation, diagnostic strategies, management and future perspectives on sicca syndrome in patients with SSc.
硬皮病是一种自身免疫性疾病，其特征是危及生命的表现，如肺纤维化或肺动脉高压。也有报道称，会出现对生活质量有不利影响的症状，并且多达 80%的硬皮病患者存在干燥综合征（口干、眼干）。干燥综合征可在不与干燥综合征重叠的情况下发生，最近的研究强调，与硬皮病发病机制直接相关的小唾液腺和大唾液腺纤维化，可能是硬皮病患者口干的主要原因。本综述概述了硬皮病患者干燥综合征的临床表现、诊断策略、治疗方法和未来展望。
