Chest
Volume 140, Issue 4, October 2011, Pages 1016-1024
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Original Research
Pulmonary Vascular Disease
Clinical Characteristics and Survival in Systemic Sclerosis-Related Pulmonary Hypertension Associated With Interstitial Lung Disease

https://doi.org/10.1378/chest.10-2473Get rights and content

Background

Pulmonary hypertension (PH) complicating systemic sclerosis (SSc)-related interstitial lung disease (ILD) is usually associated with a poor prognosis. However, data are either lacking or scarce on prognostic factors in this condition. The objectives of this study were to compare the survival of patients with ILD-associated PH (PH-ILD) or pulmonary arterial hypertension (PAH) and to determine whether the severity of PH has prognostic value in SSc-associated PH-ILD.

Methods

Consecutive patients with SSc and PH-ILD (n = 47) or PAH (n = 50) confirmed by right-sided heart catheterization were included in a cross-sectional analysis. PH was classified as mild (mean pulmonary arterial pressure [mPAP] ≤ 35 mm Hg) or moderate to severe (mPAP > 35 mm Hg).

Results

As compared with patients with PAH, subjects with PH-ILD were younger, were more frequently men with a history of smoking, had more frequently diffuse SSc, less frequently anticentromere antibodies, and a lower FVC/diffusing capacity of lung for carbon monoxide (Dlco) ratio. They had a worse prognosis than patients with PAH (3-year survival of 47% vs 71%, respectively; P = .07). Patients with mild PH-ILD had similar poor outcomes when compared with those with moderate to severe PH-ILD. Pericardial effusion (hazard ratio [HR], 2.44; P = .04) and lower Dlco (HR, 0.96; P = .01) were the only independent factors predictive of a poor survival in the PH-ILD group.

Conclusions

Patients with SSc with PH-ILD had a different phenotype and a worse prognosis than those with SSc and PAH. Lower Dlco and presence of pericardial effusion were predictive of a poor outcome in PH-ILD, whereas mPAP seemed to have no prognostic significance.

Section snippets

Materials and Methods

Data were retrospectively retrieved from clinical files of patients followed up in six specialist centers for SSc and PH in France (Antoine-Béclère Hospital, Clamart; Claude-Huriez Hospital, Lille; Cochin Hospital [Internal Medicine and Rheumatology], Paris; Foch Hospital, Suresne; Louis-Pradel Hospital, Lyon). All consecutive patients with SSc and precapillary PH seen in Antoine-Béclère Hospital (n = 46), Claude-Huriez Hospital (n = 23), and Cochin Hospital (Internal Medicine, n = 8) between

Clinical, Functional, and Hemodynamic Characteristics in the PH-ILD Group Compared With the PAH Group

Data from 47 patients with SSc with PH-ILD were retrospectively collected and compared with 50 patients with SSc with PAH. Patients with PH-ILD differed from patients with PAH (Table 1); there was a higher proportion of men and diffuse SSc and a lower percentage of patients with anticentromere antibodies in the PH-ILD group. Patients in the PH-ILD group tended to be younger at PH diagnosis and to more frequently have antitopoisomerase 1 antibodies. Concerning the hemodynamic data, cardiac index

Discussion

PH in patients with SSc can be classified either as an isolated PAH or as a PH-ILD.1 However, the difficulty is that SSc is frequently associated with ILD and pulmonary vascular disease. Therefore, in a patient with SSc with both PH and ILD, it can be difficult to firmly establish whether the patient has a PAH independent from ILD, a PH-ILD, or the combination of PH-ILD and a pulmonary vasculopathy. Usually, both in clinical trials and in previous studies focusing on PH in SSc-related ILD as

Acknowledgments

Author contributions: Dr Launay had full access to all the data in the study and had final responsibility for the decision to submit for publication.

Dr Launay: contributed to the study design; collection, analysis, and interpretation of data; drafting and critical review of the manuscript; and reading and approving the final version.

Dr Humbert: contributed to the collection of data, drafting and critical review of the manuscript, and reading and approving the final version.

Dr Berezne:

References (35)

  • S Trad et al.

    Pulmonary arterial hypertension is a major mortality factor in diffuse systemic sclerosis, independent of interstitial lung disease

    Arthritis Rheum

    (2006)
  • R Condliffe et al.

    Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era

    Am J Respir Crit Care Med

    (2009)
  • B Chang et al.

    Scleroderma patients with combined pulmonary hypertension and interstitial lung disease

    J Rheumatol

    (2003)
  • E Weitzenblum et al.

    Pulmonary hypertension in chronic obstructive pulmonary disease and interstitial lung diseases

    Semin Respir Crit Care Med

    (2009)
  • A Chaouat et al.

    Pulmonary hypertension in COPD

    Eur Respir J

    (2008)
  • B Chang et al.

    Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma

    J Rheumatol

    (2006)
  • A Chaouat et al.

    Severe pulmonary hypertension and chronic obstructive pulmonary disease

    Am J Respir Crit Care Med

    (2005)
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      PH and ILD are the most common pulmonary manifestations in SSc and also the leading cause of death. The exact prevalence of group 3 PH in SSc is unknown, but some studies estimate 22% of patients with SSc meet criteria.33 The presence of even mild PH in SSc-ILD results in a 5-fold increased risk of death with a 3-year survival of 39%.33,34

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    Funding/Support: This study was supported by a research grant from Pfizer.

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

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