This article appears in the following Rheumatology issue: Update in systemic sclerosis [View the issue table of contents]
Outcome measures in pulmonary arterial hypertension associated with systemic sclerosis
1Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland, 2Department of Pneumology, University Hospital Gasthuisberg, Leuven, Belgium, 3Department of Respiratory Medicine, VU University Medical Center, Amsterdam, The Netherlands, 4Department of Respiratory Medicine, Hanover Medical School, Hanover, Germany and 5Department of Cardiology, Erasme Hospital, Free University of Brussels, Brussels, Belgium.
Correspondence to: R. Naeije, Department of Pathophysiology, Erasme Campus, CP 604, 808, Lennik road, B-1070 Brussels, Belgium. E-mail: rnaeije{at}ulb.ac.be
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Abstract |
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SSc is complicated in 
10% of the patients by pulmonary arterial hypertension (PAH), a rare dyspnoea–fatigue syndrome caused by an increase in pulmonary vascular resistance. The prognosis of SSc-PAH is particularly poor, with estimated survival rates of 50% at 2 yrs without pulmonary circulation-targeted therapies. Prostacyclins, endothelin receptor antagonists and phosphodiesterase-5 inhibitors have been shown to be efficacious in PAH, with persistent long-term benefit and approximate doubling of survival rate, and these encouraging results appear transposable to the SSc-PAH subcategory. However, PAH as well as SSc-PAH remain incurable, with insufficient functional improvement in many patients. More progress is needed, and this will require more effective drugs and adapted outcome measures.
KEY WORDS: Pulmonary arterial hypertension, Systemic sclerosis, Outcome measure, Exercise capacity
Submitted 1 May 2008; Accepted 3 July 2008