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Original Research: PULMONARY HYPERTENSION |

Is Pulmonary Arterial Hypertension Really a Late Complication of Systemic Sclerosis?

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; Gérald Simonneau, MD; Pierre Clerson, MD; Marc Humbert, MD, PhD; for the French PAH-SSc Network
Author and Funding Information

Affiliations: From the Department of Internal Medicine (Drs. Hachulla, Launay, and Hatron), University of Lille 2, Lille, France; the National Reference Center for Scleroderma (Drs. Hachulla, Launay, and Hatron), Hôpital Claude Huriez, Lille, France; the Department of Internal Medicine (Drs. Mouthon, Berezne, and Guillevin), Université Paris Descartes, Unité Propre de Recherche de l'Enseignement Supérieur (UPRES) EA 4058, Paris, France; the National Reference Center for Vasculitis and Scleroderma (Drs. Mouthon, Berezne, and Guillevin), Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France; the Respiratory Department (Drs. Sitbon, Simonneau, and Humbert), Université Paris-Sud 11, Paris, France; the National Reference Center for Pulmonary Hypertension (Drs. Sitbon, Simonneau, and Humbert), Hôpital Antoine Béclère, Assistance Publique Hôpitaux de Paris, Clamart, France; and Orgamétrie Biostatistiques (Dr. Clerson), Roubaix, France.

Correspondence to: Eric Hachulla, MD, PhD, Department of Internal Medicine, National Reference Center for Scleroderma, Claude Huriez Hospital, University of Lille, 59037 Lille cedex, France; e-mail: ehachulla@chru-lille.fr

*A list of the French PAH-SSc Network Investigators is located in the Appendix.


The work was performed in France at Béclère Hospital, Clamart; Huriez Hospital, Lille; and Cochin Hospital, Paris.

Funding/Support: This study was supported by a research grant from Actelion Pharmaceuticals France.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).


© 2009 American College of Chest Physicians


Chest. 2009;136(5):1211-1219. doi:10.1378/chest.08-3042
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Background:  Pulmonary arterial hypertension (PAH) is a frequent cause of morbidity and mortality in patients with systemic sclerosis (SSc). PAH is generally considered to be a late complication of limited cutaneous SSc. This study identified and investigated a subset of SSc patients with early-onset PAH.

Methods:  Clinical and hemodynamic data at the time of diagnosis were collected retrospectively for 78 consecutive patients with PAH associated with SSc. PAH diagnosed within 5 years of the first non-Raynaud phenomenon symptom of SSc was considered to be an early-onset complication. PAH diagnosed > 5 years following SSc diagnosis was considered to be a late complication.

Results:  PAH occurred a mean (± SD) duration of 6.3 ± 6.6 years after the diagnosis of SSc (median delay, 4.0 years; 95% CI, 2.88 to 6.0 years). Early-onset PAH was diagnosed in 43 patients (55.1%), and late-onset PAH was diagnosed in 35 patients (44.9%). Patients with early-onset PAH were older at SSc diagnosis than patients with late-onset PAH (mean age, 58.0 ± 12.5 vs 46.6 ± 12.9 years, respectively; p = 0.0002). No differences in age at the time of PAH diagnosis, or in SSc subtype (limited vs diffuse; anticentromere vs anti-Scl70 antibodies), were observed between onset subgroups. At diagnosis, early-onset PAH was more severe than late-onset PAH, with a lower cardiac index (2.4 ± 0.6 vs 2.8 ± 0.6 L/min/m2, respectively; p = 0.005) and greater total pulmonary resistance (1,708 ± 777 vs 1,341 ± 530 dyne · s · cm−5/m2, respectively; p = 0.02). Mortality at 3 and 5 years was comparable between subgroups.

Conclusions:  In contrast to the expected scenario, early-onset PAH occurred in approximately half of SSc patients. Early-onset PAH was as frequent among patients with diffuse SSc as those with limited SSc. Annual screening for PAH should be implemented immediately after SSc diagnosis for all patients.

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