Systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease

Impact of pulmonary arterial hypertension therapies

Jérôme Le Pavec, Reda E. Girgis, Noah Lechtzin, Stephen Mathai, David Launay, Laura Hummers, Ari Zaiman, Olivier Sitbon, Gérald Simonneau, Marc Humbert, Paul M Hassoun

Research output: Contribution to journalArticle

Abstract

Objective Precapillary pulmonary hypertension (PH) is an important cause of death in patients with systemic sclerosis (SSc). It can occur in isolation (pulmonary arterial hypertension [PAH]) or in association with interstitial lung disease (ILD). Importantly, the outcomes and efficacy of PAH therapies in patients with SSc-related PH complicating ILD (PH-ILD) remain unknown. This study was undertaken to evaluate our experience with PH-ILD with regard to the efficacy and safety of PAH therapies in this patient cohort. Methods We conducted a retrospective analysis of consecutive SSc patients from 2 large referral centers who had PH-ILD confirmed by right-sided heart catheterization and who received targeted PAH therapies. World Health Organization (WHO) functional class, 6-minute walk distance, and hemodynamic parameters were assessed at baseline and after a mean ± SD of 7.7 ± 6.2 months of treatment for PAH. Kaplan-Meier and Cox proportional hazards models were used to analyze survival and to identify prognostic factors. Results Seventy patients were included in the study. No significant changes were observed in WHO functional class, 6-minute walk distance, or hemodynamic parameters after therapy. The 1-, 2-, and 3-year survival estimates were 71%, 39%, and 21%, respectively. In the multivariate model, worsening oxygenation during followup and reduced renal function were the only significant risk factors for death. Conclusion This study represents the largest series to date in which the impact of PAH therapies in SSc-related PH-ILD was examined. In this cohort, PAH therapies were associated with no clear benefits. Deterioration in oxygenation was an important determinant of long-term survival. Prospective clinical trials focusing on this group of patients are warranted.

Original languageEnglish (US)
Pages (from-to)2456-2464
Number of pages9
JournalArthritis and Rheumatism
Volume63
Issue number8
DOIs
StatePublished - Aug 2011

Fingerprint

Systemic Scleroderma
Interstitial Lung Diseases
Pulmonary Hypertension
Therapeutics
Hemodynamics
Survival
Cardiac Catheterization
Survival Analysis
Proportional Hazards Models
Cause of Death
Referral and Consultation
Clinical Trials

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy
  • Rheumatology
  • Pharmacology (medical)

Cite this

Systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease : Impact of pulmonary arterial hypertension therapies. / Le Pavec, Jérôme; Girgis, Reda E.; Lechtzin, Noah; Mathai, Stephen; Launay, David; Hummers, Laura; Zaiman, Ari; Sitbon, Olivier; Simonneau, Gérald; Humbert, Marc; Hassoun, Paul M.

In: Arthritis and Rheumatism, Vol. 63, No. 8, 08.2011, p. 2456-2464.

Research output: Contribution to journalArticle

Le Pavec, Jérôme ; Girgis, Reda E. ; Lechtzin, Noah ; Mathai, Stephen ; Launay, David ; Hummers, Laura ; Zaiman, Ari ; Sitbon, Olivier ; Simonneau, Gérald ; Humbert, Marc ; Hassoun, Paul M. / Systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease : Impact of pulmonary arterial hypertension therapies. In: Arthritis and Rheumatism. 2011 ; Vol. 63, No. 8. pp. 2456-2464.
@article{02d2acef93734ce58716717230b5cb84,
title = "Systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease: Impact of pulmonary arterial hypertension therapies",
abstract = "Objective Precapillary pulmonary hypertension (PH) is an important cause of death in patients with systemic sclerosis (SSc). It can occur in isolation (pulmonary arterial hypertension [PAH]) or in association with interstitial lung disease (ILD). Importantly, the outcomes and efficacy of PAH therapies in patients with SSc-related PH complicating ILD (PH-ILD) remain unknown. This study was undertaken to evaluate our experience with PH-ILD with regard to the efficacy and safety of PAH therapies in this patient cohort. Methods We conducted a retrospective analysis of consecutive SSc patients from 2 large referral centers who had PH-ILD confirmed by right-sided heart catheterization and who received targeted PAH therapies. World Health Organization (WHO) functional class, 6-minute walk distance, and hemodynamic parameters were assessed at baseline and after a mean ± SD of 7.7 ± 6.2 months of treatment for PAH. Kaplan-Meier and Cox proportional hazards models were used to analyze survival and to identify prognostic factors. Results Seventy patients were included in the study. No significant changes were observed in WHO functional class, 6-minute walk distance, or hemodynamic parameters after therapy. The 1-, 2-, and 3-year survival estimates were 71{\%}, 39{\%}, and 21{\%}, respectively. In the multivariate model, worsening oxygenation during followup and reduced renal function were the only significant risk factors for death. Conclusion This study represents the largest series to date in which the impact of PAH therapies in SSc-related PH-ILD was examined. In this cohort, PAH therapies were associated with no clear benefits. Deterioration in oxygenation was an important determinant of long-term survival. Prospective clinical trials focusing on this group of patients are warranted.",
author = "{Le Pavec}, J{\'e}r{\^o}me and Girgis, {Reda E.} and Noah Lechtzin and Stephen Mathai and David Launay and Laura Hummers and Ari Zaiman and Olivier Sitbon and G{\'e}rald Simonneau and Marc Humbert and Hassoun, {Paul M}",
year = "2011",
month = "8",
doi = "10.1002/art.30423",
language = "English (US)",
volume = "63",
pages = "2456--2464",
journal = "Arthritis and Rheumatology",
issn = "2326-5191",
publisher = "John Wiley and Sons Ltd",
number = "8",

}

TY - JOUR

T1 - Systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease

T2 - Impact of pulmonary arterial hypertension therapies

AU - Le Pavec, Jérôme

AU - Girgis, Reda E.

AU - Lechtzin, Noah

AU - Mathai, Stephen

AU - Launay, David

AU - Hummers, Laura

AU - Zaiman, Ari

AU - Sitbon, Olivier

AU - Simonneau, Gérald

AU - Humbert, Marc

AU - Hassoun, Paul M

PY - 2011/8

Y1 - 2011/8

N2 - Objective Precapillary pulmonary hypertension (PH) is an important cause of death in patients with systemic sclerosis (SSc). It can occur in isolation (pulmonary arterial hypertension [PAH]) or in association with interstitial lung disease (ILD). Importantly, the outcomes and efficacy of PAH therapies in patients with SSc-related PH complicating ILD (PH-ILD) remain unknown. This study was undertaken to evaluate our experience with PH-ILD with regard to the efficacy and safety of PAH therapies in this patient cohort. Methods We conducted a retrospective analysis of consecutive SSc patients from 2 large referral centers who had PH-ILD confirmed by right-sided heart catheterization and who received targeted PAH therapies. World Health Organization (WHO) functional class, 6-minute walk distance, and hemodynamic parameters were assessed at baseline and after a mean ± SD of 7.7 ± 6.2 months of treatment for PAH. Kaplan-Meier and Cox proportional hazards models were used to analyze survival and to identify prognostic factors. Results Seventy patients were included in the study. No significant changes were observed in WHO functional class, 6-minute walk distance, or hemodynamic parameters after therapy. The 1-, 2-, and 3-year survival estimates were 71%, 39%, and 21%, respectively. In the multivariate model, worsening oxygenation during followup and reduced renal function were the only significant risk factors for death. Conclusion This study represents the largest series to date in which the impact of PAH therapies in SSc-related PH-ILD was examined. In this cohort, PAH therapies were associated with no clear benefits. Deterioration in oxygenation was an important determinant of long-term survival. Prospective clinical trials focusing on this group of patients are warranted.

AB - Objective Precapillary pulmonary hypertension (PH) is an important cause of death in patients with systemic sclerosis (SSc). It can occur in isolation (pulmonary arterial hypertension [PAH]) or in association with interstitial lung disease (ILD). Importantly, the outcomes and efficacy of PAH therapies in patients with SSc-related PH complicating ILD (PH-ILD) remain unknown. This study was undertaken to evaluate our experience with PH-ILD with regard to the efficacy and safety of PAH therapies in this patient cohort. Methods We conducted a retrospective analysis of consecutive SSc patients from 2 large referral centers who had PH-ILD confirmed by right-sided heart catheterization and who received targeted PAH therapies. World Health Organization (WHO) functional class, 6-minute walk distance, and hemodynamic parameters were assessed at baseline and after a mean ± SD of 7.7 ± 6.2 months of treatment for PAH. Kaplan-Meier and Cox proportional hazards models were used to analyze survival and to identify prognostic factors. Results Seventy patients were included in the study. No significant changes were observed in WHO functional class, 6-minute walk distance, or hemodynamic parameters after therapy. The 1-, 2-, and 3-year survival estimates were 71%, 39%, and 21%, respectively. In the multivariate model, worsening oxygenation during followup and reduced renal function were the only significant risk factors for death. Conclusion This study represents the largest series to date in which the impact of PAH therapies in SSc-related PH-ILD was examined. In this cohort, PAH therapies were associated with no clear benefits. Deterioration in oxygenation was an important determinant of long-term survival. Prospective clinical trials focusing on this group of patients are warranted.

UR - http://www.scopus.com/inward/record.url?scp=79961114967&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=79961114967&partnerID=8YFLogxK

U2 - 10.1002/art.30423

DO - 10.1002/art.30423

M3 - Article

VL - 63

SP - 2456

EP - 2464

JO - Arthritis and Rheumatology

JF - Arthritis and Rheumatology

SN - 2326-5191

IS - 8

ER -