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[Pulmonary hypertension associated with systemic sclerosis].
Olivier Sanchez1, Hilario Nunes, Olivier Sitbon
1Service de Pneumologie et Réanimation Respiratoire, UPRES EA 2705 Maladies vasculaires pulmonaires , Hôpital Antoine-Béclère, 157, rue de la Porte-de-Trivaux, 92140 Clamart, France.
Annales De Medecine Interne
|September 10, 2002
Summary
Pulmonary hypertension, a severe complication of scleroderma and CREST syndrome, is often diagnosed early. Current treatments like epoprostenol and immunosuppressants show limited efficacy, necessitating novel therapeutic approaches.
Area of Science:
- Cardiology
- Rheumatology
- Pulmonology
Context:
- Pulmonary hypertension (PH) is a life-threatening complication of scleroderma, particularly CREST syndrome.
- Early diagnosis is crucial, with dyspnea as a primary symptom.
Purpose:
- To analyze literature and report clinical experience on PH in scleroderma.
- To evaluate diagnostic tools and treatment efficacy.
Summary:
- Echocardiography aids detection; right-heart catheterization confirms diagnosis and assesses vasoreactivity (e.g., with nitric oxide).
- Patients with connective tissue diseases may have milder hemodynamics due to earlier diagnosis.
- Fewer patients show acute vasodilator response, indicating early irreversible pulmonary vascular lesions.
- Continuous intravenous epoprostenol and immunosuppressive therapies show limited survival benefits.
- Novel therapies like prostacyclin analogues and endothelin receptor antagonists are under investigation.
Impact:
- Highlights the challenges in treating PH associated with scleroderma.
- Emphasizes the need for further research into effective therapies for this patient population.