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[Fatal pulmonary hypertension in Sharp syndrome (M.C.T.D.) with pulmonary fibrosis]
P Remacle1, J Bruart, C Henneghien
1Service de Pneumologie, Centre Hospitalier Universitaire de Tivoli, La Louvière, Belgium.
Revue Medicale De Bruxelles
|January 1, 1990
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.
Insights
This case study highlights a severe progression of Mixed Connective Tissue Disease (MCTD) over five years. Despite immunosuppressive therapy, the patient developed fatal pulmonary hypertension and cor pulmonale, indicating a poorer prognosis than typically expected for MCTD.
Area of Science:
- Rheumatology
- Cardiology
- Pulmonology
Background:
- Mixed Connective Tissue Disease (MCTD) is an autoimmune disorder with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis.
- The prognosis for MCTD has generally been considered favorable, particularly with early diagnosis and treatment.