A Sj(o)gren's syndrome patient with prominent aortic valve involvement: a case report / 北京大学学报(医学版)

ABSTRACT

Sj(o)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(o)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 Sj(o)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(o)gren's syndrome can affect all parts of the heart,but valve involvement is relatively rare.The specific relationship between cardiac involvement and Sj(o)gren's syndrome and the mechanisms behind these associations both need further research.