0
Correspondence |

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

Aranzazu Campo, MD; Stephen C. Mathai, MD; Reda E. Girgis, MB BCh, FCCP; Paul M. Hassoun, MD, FCCP; for the French PAH-SSc Network
Author and Funding Information

From the Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University School of Medicine.

Correspondence to: Paul M. Hassoun, MD, FCCP, Division of Pulmonary and Critical Care Medicine, 1830 E Monument St, Fifth Floor, Baltimore, MD 21205; e-mail: phassoun@jhmi.edu


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 discussed 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(2):461-462. doi:10.1378/chest.10-0485
Text Size: A A A
Published online

To the Editor:

We read with great interest the report by Hachulla et al in CHEST (May 2009)1 regarding the time of presentation of pulmonary arterial hypertension (PAH) during the course of scleroderma disease (SSc). In their cohort of 78 patients with SSc-associated PAH (PAH-SSc), the authors found the following: (1) 55% of the patients had early-onset PAH (ie, within the first 5 years of SSc-related non-Raynaud symptoms); (2) These patients were older at the time of SSc diagnosis and presented with more severe hemodynamics, although functional class (FC) and mortality outcomes were similar to those of patients with late-onset PAH; (3) Diffusing capacity of the lung for carbon monoxide (Dlco) < 35% and FC class 4 were the only predictors for survival in this cohort; and (4) There was a relatively high proportion (22%) of patients with diffuse subtype SSc (dSSc).

We recently described survival outcomes of a cohort of 76 patients with PAH-SSc followed at our center.2 Although the proportion of early-onset PAH in our cohort was lower (36%), 25% of these patients were diagnosed with PAH within the first 3 years and 11% within the same year of SSc diagnosis. Similar to findings by Hachulla et al,1 our patients with early-onset PAH were older at the time of SSc presentation compared with patients developing PAH later (59 vs 43 years; P < .01); however, there were no differences in hemodynamics or survival between the groups. Nonetheless, findings from both studies reinforce the need for early screening for PAH in these patients.

In addition, in the study by Hachulla et al,1 Dlco < 35% and FC were the only predictors for survival. This finding may be biased by inclusion of patients with more than mild interstitial lung disease (ILD), because criteria for excluding ILD were based only on FVC or total lung capacity < 60% without consideration of radiologic findings.3 ILD clearly has an impact on survival in SSc,3 in particular when complicated by pulmonary hypertension, as recently demonstrated.4,5 Thus, one cannot exclude the fact that poorer outcomes related to Dlco < 35% might reflect more significant ILD than suggested by pulmonary function tests alteration alone. In this context, the relatively high proportion of patients with dSSc (22%), compared with other studies of PAH-SSc (8%-13%)6,7 with stricter criteria for ILD exclusion, may indicate inclusion of patients with significant ILD (more common with this SSc subtype). In summary, we wish to emphasize the need for a rigorous phenotypic characterization in order to identify the exact factors responsible for very poor outcomes in PAH-SSc, while avoiding confounders.

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]
 
Campo A, Mathai SC, Le Pavec J, et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am J Respir Crit Care Med. In press.
 
Goh NSL, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;17711:1248-1254. [CrossRef] [PubMed]
 
Mathai SC, Hummers LK, Champion HC, et al. Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung disease. Arthritis Rheum. 2009;602:569-577. [CrossRef] [PubMed]
 
Condliffe R, Kiely DG, Peacock AJ, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;1792:151-157. [CrossRef] [PubMed]
 
Fisher MR, Mathai SC, Champion HC, et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum. 2006;549:3043-3050. [CrossRef] [PubMed]
 
Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med. 2000;1326:425-434. [PubMed]
 

Figures

Tables

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]
 
Campo A, Mathai SC, Le Pavec J, et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am J Respir Crit Care Med. In press.
 
Goh NSL, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008;17711:1248-1254. [CrossRef] [PubMed]
 
Mathai SC, Hummers LK, Champion HC, et al. Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases: impact of interstitial lung disease. Arthritis Rheum. 2009;602:569-577. [CrossRef] [PubMed]
 
Condliffe R, Kiely DG, Peacock AJ, et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med. 2009;1792:151-157. [CrossRef] [PubMed]
 
Fisher MR, Mathai SC, Champion HC, et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum. 2006;549:3043-3050. [CrossRef] [PubMed]
 
Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med. 2000;1326:425-434. [PubMed]
 
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543