Chest
Original ResearchPulmonary Vascular DiseaseClinical Characteristics and Survival in Systemic Sclerosis-Related Pulmonary Hypertension Associated With Interstitial Lung Disease
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:
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Funding/Support: This study was supported by a research grant from Pfizer.
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