以主动脉瓣膜病变为突出表现的干燥综合征1例报道

A Sjgren's syndrome patient with prominent aortic valve involvement:a case report

干燥综合征（Sj9gren’s syndrome,SS）是一种以泪腺和唾液腺慢性炎症为主要特征的系统性自身免疫性疾病,上皮炎症是其最基本的病理改变。在干燥综合征病程中,心脏损害并不多见,而以主动脉瓣狭窄为主要表现的干燥综合征更是罕见。本文报道1例以主动脉瓣狭窄为突出表现的干燥综合征患者。

Sjgren＇s syndrome is a systemic autoimmune disease which is characterized by xerosis,and multiple organs can be affected,causing interstitial lung disease,renal tubular acidosis,blood system involvement,peripheral neuropathy,and liver damage,but cardiac involvement is rare. Here we report a case of Sjgren＇s syndrome with prominent aortic valve involvement. A 66-year-old woman was admitted for exertional dyspnea,and the ultrasonic cardiogram showed severe aortic stenosis with moderate regurgitation. Preoperative examination for valve replacement found that the patient had elevated erythrocyte sedimentation rate and positive rheumatoid factor,so she was referred to the rheumatology outpatient department for further examination and treatment. Further questions about medical history found that the patient had dry eyes and dry mouth for more than ten years,and had obvious caries. Further laboratory examination showed elevated serum immunoglobulin levels and positive anti-nuclear antibody. The findings of ophthalmologic examination and labial gland biopsy also supported the diagnosis of Sj9 gren ＇s syndrome. After treatment of glucocorticoids and hydroxychloroquine for ten months,her dyspnea symptoms were obviously improved in the patient,and the rheumatoid factor had become negative while her erythrocyte sedimentation rate normal. Repeated ultrasonic cardiogram examination showed that her aortic stenosis was also improved. The patient has been followed up for four years,and her condition kept stable. Cardiac involvement in Sjgren＇s syndrome can affect all parts of the heart,but valve involvement is relatively rare. The specific relationship between cardiac involvement and Sjgren ＇s syndrome and the mechanisms behind these associations both need further research.