title: [Primary Sjögren's syndrome with pulmonary hypertension].
abstract: 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.
authors.affiliation: Division of Pulmonology, NTT Kanto Teishin Hospital, Tokyo, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Usui
authors.forename: C
authors.initials: C
authors.lastname: Anzai
authors.forename: K
authors.initials: K
authors.lastname: Sano
authors.forename: S
authors.initials: S
authors.lastname: Kumazaki
authors.forename: T
authors.initials: T
authors.lastname: Ishihara
mesh_terms: D000743:Anemia, Hemolytic / Q000150:complications; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D003520:Cyclophosphamide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000209:etiology*; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage; D011928:Raynaud Disease / Q000150:complications; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone; D008775:Methylprednisolone