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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.

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.

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