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Correspondence |

Systemic Sclerosis and Early-Onset Pulmonary Hypertension FREE TO VIEW

David Langleben, MD; Stylianos E. Orfanos, MD, PhD; for the French PAH-SSc Network
Author and Funding Information

From the Center for Pulmonary Vascular Disease (Dr Langleben), Jewish General Hospital and Lady Davis Institute for Medical Research; and the Pulmonary Hypertension Center (Dr Orfanos), Attikon Hospital.

Correspondence to: David Langleben, MD, Jewish General Hospital, 3755 Cote Ste Catherine, Montreal, QC, Canada, H3T 1E2; e-mail: david.langleben@mcgill.ca


Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be dis-cussed in this article.

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


© 2010 American College of Chest Physicians


Chest. 2010;138(1):238-239. doi:10.1378/chest.10-0501
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To the Editor:

We read with interest the report by Hachulla et al1 (November 2009) that early-onset pulmonary arterial hypertension (PAH) was frequent in patients with systemic sclerosis and was associated with severe hemodynamic abnormalities. Most of the patients had a low carbon-monoxide diffusing capacity (Dlco), and a Dlco < 35% predicted indicated a poorer prognosis. Using sensitive indicator dilution techniques in patients with systemic sclerosis, we have demonstrated the presence of pulmonary endotheliopathy and reduced functional pulmonary capillary surface area early in the disease, even before the onset of PAH.2 Although it had been postulated for some time that decreased Dlco in the absence of interstitial fibrosis was related to reduced capillary surface area available for gas diffusion and represented the presence of PAH, this relationship had not previously been directly measured. We have recently provided the first, to our knowledge, functional confirmation that decreased Dlco relates to decreased functional capillary surface area in connective tissue disease.3 Based on our data, the prognostic threshold of Dlco <35% predicted found by Hachulla and colleagues1 should represent a profound reduction in functional capillary surface area, in the range of 25% of normal.3,4 This degree of abnormality would certainly contribute to a poor prognosis. Regular screening as proposed by Hachulla and colleagues represents the only hope for early detection and possibly better therapeutic success.

Hachulla E, Launay D, Mouthon L, et al; for the French PAH-SSc Network for the French PAH-SSc Network Is pulmonary arterial hypertension really a late complication of systemic sclerosis? Chest. 2009;1365:1211-1219. [CrossRef] [PubMed]
 
Orfanos SE, Psevdi E, Stratigis N, et al. Pulmonary capillary endothelial dysfunction in early systemic sclerosis. Arthritis Rheum. 2001;444:902-911. [CrossRef] [PubMed]
 
Langleben D, Orfanos SE, Giovinazzo M, et al. Pulmonary capillary endothelial metabolic dysfunction: severity in pulmonary arterial hypertension related to connective tissue disease versus idiopathic pulmonary arterial hypertension. Arthritis Rheum. 2008;584:1156-1164. [CrossRef] [PubMed]
 
Orfanos SE, Langleben D, Khoury J, et al. Pulmonary capillary endothelium-bound angiotensin-converting enzyme activity in humans. Circulation. 1999;9912:1593-1599. [CrossRef] [PubMed]
 

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References

Hachulla E, Launay D, Mouthon L, et al; for the French PAH-SSc Network for the French PAH-SSc Network Is pulmonary arterial hypertension really a late complication of systemic sclerosis? Chest. 2009;1365:1211-1219. [CrossRef] [PubMed]
 
Orfanos SE, Psevdi E, Stratigis N, et al. Pulmonary capillary endothelial dysfunction in early systemic sclerosis. Arthritis Rheum. 2001;444:902-911. [CrossRef] [PubMed]
 
Langleben D, Orfanos SE, Giovinazzo M, et al. Pulmonary capillary endothelial metabolic dysfunction: severity in pulmonary arterial hypertension related to connective tissue disease versus idiopathic pulmonary arterial hypertension. Arthritis Rheum. 2008;584:1156-1164. [CrossRef] [PubMed]
 
Orfanos SE, Langleben D, Khoury J, et al. Pulmonary capillary endothelium-bound angiotensin-converting enzyme activity in humans. Circulation. 1999;9912:1593-1599. [CrossRef] [PubMed]
 
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