title: [Fatal pulmonary hypertension in Sharp syndrome (M.C.T.D.) with pulmonary fibrosis].
abstract: We have followed a case of Mixed Connective Tissue Disease (M.C.T.D.) during 5 years. A progressive pulmonary hypertension inducing cor pulmonary failure terminated in a fatal issue. The prognosis of M.C.T.D. previously considered good is sometimes more severe in spite of immunosuppressive therapy.
authors.affiliation: Service de Pneumologie, Centre Hospitalier Universitaire de Tivoli, La Louvière, Belgium.
authors.forename: P
authors.initials: P
authors.lastname: Remacle
authors.forename: J
authors.initials: J
authors.lastname: Bruart
authors.forename: C
authors.initials: C
authors.lastname: Henneghien
authors.forename: S
authors.initials: S
authors.lastname: Schreiber
mesh_terms: D003520:Cyclophosphamide / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000209:etiology*; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000150:complications* / Q000188:drug therapy / Q000401:mortality; D011239:Prednisolone / Q000008:administration & dosage; D011660:Pulmonary Heart Disease / Q000209:etiology*; D012129:Respiratory Function Tests
chemical_list: D003520:Cyclophosphamide; D011239:Prednisolone
vernacular_title: Hypertension pulmonaire fatale dans un syndrome de Sharp (M.C.T.D.) avec fibrose pulmonaire.