SummarySjogren's syndrome (SS) is an autoimmune disease characterized by dryness of the mouth and the eyes. Systemic involvement in SS is well known, however, obvious cardiac manifestations, particularly significant valve disorders, are extremely rare and only three cases of significant valve disease associated with SS that required surgical intervention have been previously described. We report a case of aortic stenosis (AS) associated with SS in an elderly patient. The diagnosis of primary SS had been made based on clinical features, positive ocular signs, and positive serologic findings. Echocardiography showed severe calcification, elevated mean pressure gradient (57 mmHg), and a small orifice area (0.45 cm 2 ) of the aortic valve. At surgery, severe calcification of the aortic cusps and the annulus was the mechanism of AS, and the aortic valve was replaced with a bioprosthetic valve. Valve pathology showed nodular calcification and hyaline degeneration, but lymphocyte infiltration was not evident. The etiologic relation of SS to the valve lesions is not clear pathologically in this case, however, chronic inflammation related to immunologic reactions in SS could have some effect on exacerbation for degeneration of the valve tissue. (Int Heart J 2016; 57: 251-253) Key words: Autoimmune disease, Bioprosthetic valve, Heart valve disease, Mechanical valve, Valve replacement S jogren's syndrome (SS) is an autoimmune disease characterized by keratoconjunctivitis sicca (dry eye) and xerostomia (dry mouth) that is the result of lymphocyte-mediated destruction of exocrine glands, and mostly affects middle-aged women. The systemic manifestations of SS such as articular, lung, kidney, vascular, and gastrointestinal involvement are well known, however, obvious cardiac manifestations are rare, although clinically silent involvements are fairly common on echocardiography.1) To our knowledge, only a few cases of clinically significant cardiac valve disease associated with SS that required surgical intervention have been described in the literature.
2-4)In this paper, we report a case of aortic stenosis (AS) associated with SS, and discuss prosthetic valve selection in valve surgery for patients associated with an autoimmune disorder.The current study was approved by our institutional Research Ethics Board at the Munakata Suikokai General Hospital, and written informed consent was obtained from the patient for publication of this case report and any accompanying images.
Case ReportA 70-year-old woman was admitted to our hospital for nocturnal dyspnea and chest pain. She had never smoked and had no history of rheumatic fever or diabetes mellitus, but she was on medication for hypertension and dyslipidemia for more than 10 years. At the age of 66 the patient had been diagnosed with Castleman's disease (hyaline-vascular type) after lymph node biopsy, and primary SS based on clinical features (dry mouth and eyes), ocular signs (a Shirmer's test; ≤ 3 mm in 5 minutes, rose Bengal score ≥ 4), and serological findings ( Table).5...