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See Latest ArticlesPulmonary arterial hypertension in connective tissue diseases
Current Respiratory Medicine Reviews, 05/15/09
Bodini BD et al. - Article reviews the characteristic features, diagnosis, symptoms and treatment options for pulmonary arterial hypertension (PAH) occurring in patients with connective tissue disease:
- Features and diagnosis:
- Progressive obliteration of the small pulmonary vasculature
- Increased vascular resistance and elevated pulmonary artery pressures
- Elevated pressures in the pulmonary circulation resulting in right heart failure and premature death
- PAH can also occur in connective tissue diseases: systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis
- PAH may occur in association with left heart disease, interstitial fibrosis or as a result of an isolated pulmonary arteriopathy
- Symptoms and clinical presentation are very similar to idiopathic PAH but mortality is higher
- Diagnostic tools:
- A 2-D echoDoppler examination, further confirmed by right heart catheterization
- Treatment options:
- Intravenous epoprostenol therapy can improve exercise capacity and symptoms in pts with systemic sclerosis
- Endothelin receptor antagonists (bosentan and sitaxentan), the phosphodyesterase-type-5 inhibitor (sildenafil) and subcutaneous treprostinil have shown favourable results
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