A 50-year-old woman was admitted to our hospital because of dyspnea on exertion. A chest X-ray film showed cardiomegaly and echocardiography revealed right-heart overload. Right heart catheterization studies revealed pulmonary hypertension and no signs of pulmonary embolism or vasculitis. The patient had complained of Raynaud's phenomenon since 6 years before admission. She did not notice eye or mouth dryness, but examination of a lip-biopsy specimen showed infiltration of lymphocytes into the salivary glands. Laboratory findings showed hypergammaglobulinemia and hemolytic anemia. Primary Sjögren's syndrome with pulmonary hypertension was diagnosed. Her general condition improved with steroid pulse therapy followed by oral administration of corticosteroids and cyclophosphamide.
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