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Pulmonary arterial hypertension complicating connective tissue diseases
Seminars in Respiratory and Critical Care Medicine, 08/06/09

Hassoun PM et al. – Pulmonary hypertension (PAH) is a common complication of connective tissue diseases (CTD), particularly systemic sclerosis (SSc). This article reviews the clinical features associated with it and available thearapies.

Risk factors for development of PAH in SSc:
- Limited systemic sclerosis
- Late age of onset
- Raynaud phenomenon
- Reduction in diffusing capacity for carbon monoxide (DLCO)
- A forced vital capacity (FVC):DLCO ratio of <1.6
- Elevation of serum N-terminal pro-brain natriuretic peptide (N-TproBNP) levels
- Antibodies (e.g., anti-U3 RNP)

THERAPY- Evidence of chronically impaired endothelial function affecting vascular tone and remodeling has been the basis for current therapy of PAH:

Vasodilator therapy using high-dose calcium channel blockers is an effective long-term therapy (<7% of IPAH pts) who demonstrate acute vasodilation

Other available therapies: antiinflammatory drugs, prostaglandins, endothelin receptor antagonists, phosphodiesterase inhibitors, combination therapy, anticoagulation, and tyrosine kinase inhibitors

Modern therapy for PAH appears to be of limited value in SSc-PAH; available markers of disease severity or response to therapy are either limited or lacking. Therefore, there is an urgent need to identify potential genetic causes and novel physiological, molecular, and imaging biomarkers that will allow a better understanding of the underlying pathogenesis and serve as reliable tools to monitor therapy in this devastating syndrome.

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