Scleroderma

Eric Hachulla, MD, PhD; David Launay, MD, PhD; Luc Mouthon, MD, PhD; Olivier Sitbon, MD, PhD; Alice Berezne, MD; Loïc Guillevin, MD; Pierre-Yves Hatron, MD; Ge´rald Simonneau, MD; Pierre Clerson, MD; and Marc Humbert, MD, PhD;

Pulmonary arterial hypertension (PAH) is a frequent complication in patients with systemic sclerosis (SSc) that has a prevalence of 8 to 12%. Within the last 2 decades, pulmonary fibrosis and PAH have become the leading causes of death in SSc patients. The estimated 3-year survival rate among patients with PAH associated with SSc is approximately 50%. The development of PAH in patients with SSc must be differentiated from that of pulmonary hypertension (PH) associated with severe pulmonary fibrosis. The latter may manifest as a significant reduction of lung volume (FVC 60% predicted and/or total lung capacity [TLC] 60%) and a modest elevation in mean pulmonary artery pressure (mPAP) of 35 mm Hg at rest.

Unlike pulmonary fibrosis, PAH is understood to occur late in the natural course of SSc and more frequently in patients with limited cutaneous SSc (lcSSc). This assertion, however, may benefit from reappraisal. Studies have shown that PAH may be similarly prevalent among patients with diffuse cutaneous SSc (dcSSc) as it is among patients with lcSSc. Robust data estimating the typical duration between the diagnosis of SSc and the diagnosis of PAH are lacking. The aim of this study was to analyze this duration and evaluate the potential relevance of “time of occurrence of PAH” in the course of SSc as a prognostic factor.

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