Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 May 12;25(1):77.
doi: 10.1186/s13075-023-03059-x.

Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis

Jessica L Fairley  1   2 , Dylan Hansen  2 , Laura Ross  1   2 , Susanna Proudman  3   4 , Joanne Sahhar  5   6 , Gene-Siew Ngian  5   6 , Jennifer Walker  4 , Lauren V Host  7 , Kathleen Morrisroe  1   2 , Diane Apostolopoulous  5   6 , Nava Ferdowsi  2 , Michelle Wilson  2   6 , Maryam Tabesh  2 , Wendy Stevens  2 , Mandana Nikpour  8   9 ; Australian Scleroderma Interest Group
Collaborators, Affiliations

Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis

Jessica L Fairley et al. Arthritis Res Ther. .

Abstract

Objectives: To describe the clinical phenotype and prognosis of people in the Australian Scleroderma (SSc) Cohort Study with pulmonary arterial hypertension (PAH) with or without interstitial lung disease (ILD).

Methods: Participants meeting ACR/EULAR criteria for SSc were divided into four mutually exclusive groups: those meeting criteria for PAH (PAH-only), ILD (ILD-only), concurrent PAH and ILD (PAH-ILD) or neither PAH nor ILD (SSc-only). Logistic or linear regression analyses were used for associations between clinical features, health-related quality of life (HRQoL) and physical function. Survival analysis was performed using Kaplan-Meier estimates and Cox-regression modelling.

Results: Of 1561 participants, 7% fulfilled criteria for PAH-only, 24% ILD-only, 7% PAH-ILD and 62% SSc-only. People with PAH-ILD were more frequently male, with diffuse skin involvement, higher inflammatory markers, older age of SSc onset and higher frequency of extensive ILD than the cohort overall (p < 0.001). People of Asian race more frequently developed PAH-ILD (p < 0.001). People with PAH-ILD or PAH-only had worse WHO functional class and 6-min-walk-distance than ILD-only (p < 0.001). HRQoL scores were worst in those with PAH-ILD (p < 0.001). Survival was reduced in the PAH-only and PAH-ILD groups (p < 0.01). Multivariable hazard modelling demonstrated the worst prognosis in extensive ILD and PAH (HR = 5.65 95% CI 3.50-9.12 p < 0.01), followed by PAH-only (HR = 4.21 95% CI 2.89-6.13 p < 0.01) and PAH with limited ILD (HR = 2.46 95% CI 1.52-3.99 p < 0.01).

Conclusions: The prevalence of concurrent PAH-ILD in the ASCS is 7%, with poorer survival in those patients with PAH-ILD compared to ILD or SSc alone. The presence of PAH confers a poorer overall prognosis than even extensive ILD; however, further data are required to better understand the clinical outcomes of this high-risk patient group.

Keywords: Interstitial lung disease; Pulmonary hypertension; Quality of life; Survival.

PubMed Disclaimer

Conflict of interest statement

The Australian Scleroderma Interest Group received research support grants from Actelion. SP has received honoraria from Janssen. JS and JW have received honoraria from Boehringer Ingelheim Pty Ltd. WS has received consultancies from Jansenn and Boehringer-Ingelheim. MN has received honoraria or consultancies from Janssen, AstraZeneca, GlaxoSmithKlein, Boehringer-Ingelheim and Bristol-Myers Squibb. JF has received conference sponsorship from Pfizer and honoraria from Boehringer-Ingelheim. LH has been a paid speaker for the Limbic publication.

Figures

Fig. 1
Fig. 1
Key findings. 6MWD, six-minute walk distance; C/I, contraindication; dcSSc, diffuse cutaneous systemic sclerosis; ILD, interstitial lung disease; PAH, pulmonary arterial hypertension); PH, pulmonary hypertension; RHC, right heart catheter; SSc, systemic sclerosis; WHO, World Health Organization; WSPH, World Society of Pulmonary Hypertension
Fig. 2
Fig. 2
All-cause mortality from SSc onset. ILD, interstitial lung disease; PAH, pulmonary arterial hypertension; SSc, systemic sclerosis
Fig. 3
Fig. 3
All-cause mortality from ILD onset in those with ILD. ILD, interstitial lung disease; PAH, pulmonary arterial hypertension; SSc, systemic sclerosis. ILD severity is defined as limited if < 20% involvement on HRCT or 20‒30% HRCT involvement with FVC ≥ 70%, or extensive if > 30% HRCT involvement, or 20‒30% HRCT involvement and FVC < 70%
See this image and copyright information in PMC

References

    1. Abu-Shakra M, Lee P. Mortality in systemic sclerosis: a comparison with the general population. J Rheumatol. 1995;22(11):2100–2102. - PubMed
    1. Hinchcliff M, Khanna S, Hsu VM, Lee J, Almagor O, Chang RW, et al. Survival in systemic sclerosis-pulmonary arterial hypertension by serum autoantibody status in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) Registry. Semin Arthritis Rheum. 2015;45(3):309–314. doi: 10.1016/j.semarthrit.2015.06.011. - DOI - PMC - PubMed
    1. Coghlan JG, Denton CP, Grunig E, Bonderman D, Distler O, Khanna D, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73(7):1340–1349. doi: 10.1136/annrheumdis-2013-203301. - DOI - PMC - PubMed
    1. Schurawitzki H, Stiglbauer R, Graninger W, Herold C, Polzleitner D, Burghuber OC, et al. Interstitial lung disease in progressive systemic sclerosis: high-resolution CT versus radiography. Radiology. 1990;176(3):755–759. doi: 10.1148/radiology.176.3.2389033. - DOI - PubMed
    1. Bussone G, Mouthon L. Interstitial lung disease in systemic sclerosis. Autoimmun Rev. 2011;10(5):248–255. doi: 10.1016/j.autrev.2010.09.012. - DOI - PubMed

Publication types