Wang L J, Wei Y L, Zhang Z L
Department of Rheumatology and Clinical Immunology, Peking University First Hospital, Beijing 100034, China.
Department of Rheumatology and Clinical Immunology, People's Hospital of Zhengzhou, Zhengzhou 450000, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2017 Dec 18;49(6):1087-1089.
Sjögren'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 Sjögren'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 Sjögren'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 Sjögren's syndrome can affect all parts of the heart, but valve involvement is relatively rare. The specific relationship between cardiac involvement and Sjögren's syndrome and the mechanisms behind these associations both need further research.
干燥综合征是一种全身性自身免疫性疾病,其特征为干燥,可累及多个器官,导致间质性肺病、肾小管酸中毒、血液系统受累、周围神经病变和肝损害,但心脏受累较为罕见。本文报告1例以主动脉瓣受累为主的干燥综合征病例。一名66岁女性因劳力性呼吸困难入院,超声心动图显示重度主动脉瓣狭窄伴中度反流。瓣膜置换术前检查发现患者红细胞沉降率升高、类风湿因子阳性,遂转诊至风湿科门诊进一步检查和治疗。进一步询问病史发现患者有眼干、口干十余年,且有明显龋齿。进一步实验室检查显示血清免疫球蛋白水平升高、抗核抗体阳性。眼科检查和唇腺活检结果也支持干燥综合征的诊断。经糖皮质激素和羟氯喹治疗10个月后,患者呼吸困难症状明显改善,类风湿因子转为阴性,红细胞沉降率恢复正常。多次超声心动图检查显示其主动脉瓣狭窄也有所改善。该患者已随访4年,病情保持稳定。干燥综合征的心脏受累可累及心脏各个部位,但瓣膜受累相对少见。心脏受累与干燥综合征之间的具体关系及其背后的机制均需进一步研究。