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[Pulmonary hypertension associated with connective tissue diseases]
O Sanchez1, M Humbert, O Sitbon
1Service de pneumologie et réanimation respiratoire, UPRES EA 2705 Maladies vasculaires pulmonaires, hôpital Antoine-Béclère, 157, rue de la Porte de Trivaux, 92141 Clamart, France.
La Revue De Medecine Interne
|February 28, 2002
Summary
Pulmonary hypertension, a complication of connective tissue diseases, may be diagnosed earlier, leading to less severe symptoms. Immunosuppressive therapy shows promise in rare cases, but its role requires further definition.
Area of Science:
- Cardiology
- Rheumatology
- Pulmonology
Context:
- Pulmonary hypertension (PH) is a severe complication of connective tissue diseases (CTDs), particularly scleroderma and CREST syndrome.
- Dyspnea is a common and severe symptom, often detected early via echocardiography.
- Right heart catheterization is crucial for confirming PH diagnosis and assessing vasoreactivity.
Purpose:
- To analyze existing literature on PH in CTDs.
- To report clinical experiences from a pulmonary vascular diseases center regarding this complication.
Summary:
- PH in CTDs may be less severe due to earlier diagnosis, with a lower rate of acute vasodilator response indicating early irreversible vascular lesions.
- Continuous intravenous epoprostenol therapy shows limited efficacy and does not improve survival in CTD-associated PH.
- Dramatic improvements have been observed in rare cases following immunosuppressive therapy.
Impact:
- Newer treatments including prostacyclin analogs and endothelin receptor antagonists are under investigation.
- The precise role of immunosuppressive therapy in managing CTD-associated PH needs further clarification.