Related Experiment Videos
[Pulmonary arterial hypertension and systemic diseases]
P Chérin1, J F Delfraissy, O Blétry
1Service de Médecine Interne et Réanimation Médicale, Hôpital Antoine Béclère, Clamart.
Revue Du Rhumatisme Et Des Maladies Osteo-Articulaires
|May 1, 1991
Summary
Pulmonary artery hypertension (PAHT) often accompanies systemic diseases, particularly connective tissue disorders like scleroderma. This serious complication is frequently underdiagnosed and lacks effective treatments, contributing significantly to mortality in affected patients.
Area of Science:
- Rheumatology
- Cardiology
- Pulmonology
Context:
- Systemic diseases with vascular involvement can lead to pulmonary artery hypertension (PAHT).
- Connective tissue disorders, including scleroderma, CREST syndrome, and mixed connective tissue disease, are primary associations.
- Less commonly, PAHT is linked to vasculitis of great vessels or systemic lupus erythematosus.
Purpose:
- To highlight the underrecognized incidence and clinical significance of PAHT in systemic diseases.
- To identify the various mechanisms contributing to PAHT in these conditions.
- To review the current therapeutic landscape and survival impact of PAHT.
Summary:
- PAHT is a frequent, often overlooked complication in connective tissue diseases, significantly increasing mortality.
- Mechanisms include hypoxic vasoconstriction, thromboembolism, vasomotor phenomena, and most commonly, pulmonary vasculitis.
- While rare positive outcomes occur with immunosuppressants in mixed connective tissue disease, no definitive survival-improving treatments are established.
Impact:
- Emphasizes PAHT as a critical, underdiagnosed cause of death in systemic diseases with a potentially benign clinical reputation.
- Underscores the need for improved diagnostic strategies and research into effective treatments for PAHT in this patient population.
- Informs clinicians about the serious prognosis associated with PAHT in connective tissue diseases, necessitating vigilant monitoring and management.