[Fatal pulmonary hypertension in Sharp syndrome (M.C.T.D.) with pulmonary fibrosis].
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.
Remacle
P
P
Service de Pneumologie, Centre Hospitalier Universitaire de Tivoli, La Louvière, Belgium.
Bruart
J
J
Henneghien
C
C
Schreiber
S
S
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
D003520:Cyclophosphamide; D011239:Prednisolone
Hypertension pulmonaire fatale dans un syndrome de Sharp (M.C.T.D.) avec fibrose pulmonaire.