Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

Kathleen Morrisroe; Wendy Stevens; Molla Huq; David Prior; Joanne Sahhar; Gene Siew Ngian; David Celermajer; Jane Zochling; Susanna Proudman; Mandana Nikpour; Australian Scleroderma Interest Group (ASIG); Jane Zochling

doi:10.1186/s13075-017-1341-x

Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

Kathleen Morrisroe, Wendy Stevens, Molla Huq, David Prior, Joanne Sahhar, Gene Siew Ngian, David Celermajer, Jane Zochling, Susanna Proudman, Mandana Nikpour, Australian Scleroderma Interest Group (ASIG), Jane Zochling

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Abstract

Background: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). We sought to determine survival, predictors of mortality, and health-related quality of life (HRQoL) related to PAH in a large SSc cohort with PAH. Methods: We studied consecutive SSc patients with newly diagnosed (incident) World Health Organization (WHO) Group 1 PAH enrolled in a prospective cohort between 2009 and 2015. Survival methods were used to determine age and sex-adjusted standardised mortality ratio (SMR) and years of life lost (YLL), and to identify predictors of mortality. HRQoL was measured using the Short form 36 (SF-36) instrument. Results: Among 132 SSc-PAH patients (112 female (85%); mean age 62 ± 11 years), 60 (45.5%) died, with a median (±IQR) survival time from PAH diagnosis of 4.0 (2.2-6.2) years. Median (±IQR) follow up from study enrolment was 3.8 (1.6-5.8) years. The SMR for patients with SSc-PAH was 5.8 (95% CI 4.3-7.8), with YLL of 15.2 years (95% CI 12.3-18.1). Combination PAH therapy had a survival advantage (p < 0.001) compared with monotherapy, as did anticoagulation compared with no anticoagulation (p < 0.003). Furthermore, combination PAH therapy together with anticoagulation had a survival benefit compared with monotherapy with or without anticoagulation and combination therapy without anticoagulation (hazard ratio 0.28, 95% CI 0.1-0.7). Older age at PAH diagnosis (p = 0.03), mild co-existent interstitial lung disease (ILD) (p = 0.01), worse WHO functional class (p = 0.03) and higher mean pulmonary arterial pressure at PAH diagnosis (p = 0.001), and digital ulcers (p = 0.01) were independent predictors of mortality. Conclusions: Despite the significant benefits conferred by advanced PAH therapies suggested in this study, the median survival in SSc PAH remains short at only 4 years.

Original language	English
Article number	122
Number of pages	10
Journal	Arthritis Research and Therapy
Volume	19
DOIs	https://doi.org/10.1186/s13075-017-1341-x
Publication status	Published - 2 Jun 2017

Bibliographical note

Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver(http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

Keywords

Pulmonary arterial hypertension
quality of life
systemic sclerosis
predictors of mortality

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10.1186/s13075-017-1341-xLicence: CC BY

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Morrisroe, K., Stevens, W., Huq, M., Prior, D., Sahhar, J., Ngian, G. S., Celermajer, D., Zochling, J., Proudman, S., Nikpour, M., Australian Scleroderma Interest Group (ASIG), & Zochling, J. (2017). Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension. Arthritis Research and Therapy, 19, Article 122. https://doi.org/10.1186/s13075-017-1341-x

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title = "Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension",

abstract = "Background: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). We sought to determine survival, predictors of mortality, and health-related quality of life (HRQoL) related to PAH in a large SSc cohort with PAH. Methods: We studied consecutive SSc patients with newly diagnosed (incident) World Health Organization (WHO) Group 1 PAH enrolled in a prospective cohort between 2009 and 2015. Survival methods were used to determine age and sex-adjusted standardised mortality ratio (SMR) and years of life lost (YLL), and to identify predictors of mortality. HRQoL was measured using the Short form 36 (SF-36) instrument. Results: Among 132 SSc-PAH patients (112 female (85%); mean age 62 ± 11 years), 60 (45.5%) died, with a median (±IQR) survival time from PAH diagnosis of 4.0 (2.2-6.2) years. Median (±IQR) follow up from study enrolment was 3.8 (1.6-5.8) years. The SMR for patients with SSc-PAH was 5.8 (95% CI 4.3-7.8), with YLL of 15.2 years (95% CI 12.3-18.1). Combination PAH therapy had a survival advantage (p < 0.001) compared with monotherapy, as did anticoagulation compared with no anticoagulation (p < 0.003). Furthermore, combination PAH therapy together with anticoagulation had a survival benefit compared with monotherapy with or without anticoagulation and combination therapy without anticoagulation (hazard ratio 0.28, 95% CI 0.1-0.7). Older age at PAH diagnosis (p = 0.03), mild co-existent interstitial lung disease (ILD) (p = 0.01), worse WHO functional class (p = 0.03) and higher mean pulmonary arterial pressure at PAH diagnosis (p = 0.001), and digital ulcers (p = 0.01) were independent predictors of mortality. Conclusions: Despite the significant benefits conferred by advanced PAH therapies suggested in this study, the median survival in SSc PAH remains short at only 4 years.",

keywords = "Pulmonary arterial hypertension, quality of life, systemic sclerosis, predictors of mortality",

author = "Kathleen Morrisroe and Wendy Stevens and Molla Huq and David Prior and Joanne Sahhar and Ngian, {Gene Siew} and David Celermajer and Jane Zochling and Susanna Proudman and Mandana Nikpour and {Australian Scleroderma Interest Group (ASIG)} and Catherine Hill and Sue Lester and Peter Nash and Gian Ngian and Maureen Rischmueller and Janet Roddy and Joanne Sahhar and Gemma Strickland and Vivek Thakkar and Jenny Walker and Jane Zochling",

note = "Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver(http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.",

year = "2017",

month = jun,

day = "2",

doi = "10.1186/s13075-017-1341-x",

language = "English",

volume = "19",

journal = "Arthritis Research and Therapy",

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publisher = "BioMed Central Ltd.",

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Morrisroe, K, Stevens, W, Huq, M, Prior, D, Sahhar, J, Ngian, GS, Celermajer, D, Zochling, J, Proudman, S, Nikpour, M, Australian Scleroderma Interest Group (ASIG) & Zochling, J 2017, 'Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension', Arthritis Research and Therapy, vol. 19, 122. https://doi.org/10.1186/s13075-017-1341-x

TY - JOUR

T1 - Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

AU - Morrisroe, Kathleen

AU - Stevens, Wendy

AU - Huq, Molla

AU - Prior, David

AU - Sahhar, Joanne

AU - Ngian, Gene Siew

AU - Celermajer, David

AU - Zochling, Jane

AU - Proudman, Susanna

AU - Nikpour, Mandana

AU - Australian Scleroderma Interest Group (ASIG)

AU - Hill, Catherine

AU - Lester, Sue

AU - Nash, Peter

AU - Ngian, Gian

AU - Rischmueller, Maureen

AU - Roddy, Janet

AU - Sahhar, Joanne

AU - Strickland, Gemma

AU - Thakkar, Vivek

AU - Walker, Jenny

AU - Zochling, Jane

N1 - Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver(http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

PY - 2017/6/2

Y1 - 2017/6/2

N2 - Background: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). We sought to determine survival, predictors of mortality, and health-related quality of life (HRQoL) related to PAH in a large SSc cohort with PAH. Methods: We studied consecutive SSc patients with newly diagnosed (incident) World Health Organization (WHO) Group 1 PAH enrolled in a prospective cohort between 2009 and 2015. Survival methods were used to determine age and sex-adjusted standardised mortality ratio (SMR) and years of life lost (YLL), and to identify predictors of mortality. HRQoL was measured using the Short form 36 (SF-36) instrument. Results: Among 132 SSc-PAH patients (112 female (85%); mean age 62 ± 11 years), 60 (45.5%) died, with a median (±IQR) survival time from PAH diagnosis of 4.0 (2.2-6.2) years. Median (±IQR) follow up from study enrolment was 3.8 (1.6-5.8) years. The SMR for patients with SSc-PAH was 5.8 (95% CI 4.3-7.8), with YLL of 15.2 years (95% CI 12.3-18.1). Combination PAH therapy had a survival advantage (p < 0.001) compared with monotherapy, as did anticoagulation compared with no anticoagulation (p < 0.003). Furthermore, combination PAH therapy together with anticoagulation had a survival benefit compared with monotherapy with or without anticoagulation and combination therapy without anticoagulation (hazard ratio 0.28, 95% CI 0.1-0.7). Older age at PAH diagnosis (p = 0.03), mild co-existent interstitial lung disease (ILD) (p = 0.01), worse WHO functional class (p = 0.03) and higher mean pulmonary arterial pressure at PAH diagnosis (p = 0.001), and digital ulcers (p = 0.01) were independent predictors of mortality. Conclusions: Despite the significant benefits conferred by advanced PAH therapies suggested in this study, the median survival in SSc PAH remains short at only 4 years.

AB - Background: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). We sought to determine survival, predictors of mortality, and health-related quality of life (HRQoL) related to PAH in a large SSc cohort with PAH. Methods: We studied consecutive SSc patients with newly diagnosed (incident) World Health Organization (WHO) Group 1 PAH enrolled in a prospective cohort between 2009 and 2015. Survival methods were used to determine age and sex-adjusted standardised mortality ratio (SMR) and years of life lost (YLL), and to identify predictors of mortality. HRQoL was measured using the Short form 36 (SF-36) instrument. Results: Among 132 SSc-PAH patients (112 female (85%); mean age 62 ± 11 years), 60 (45.5%) died, with a median (±IQR) survival time from PAH diagnosis of 4.0 (2.2-6.2) years. Median (±IQR) follow up from study enrolment was 3.8 (1.6-5.8) years. The SMR for patients with SSc-PAH was 5.8 (95% CI 4.3-7.8), with YLL of 15.2 years (95% CI 12.3-18.1). Combination PAH therapy had a survival advantage (p < 0.001) compared with monotherapy, as did anticoagulation compared with no anticoagulation (p < 0.003). Furthermore, combination PAH therapy together with anticoagulation had a survival benefit compared with monotherapy with or without anticoagulation and combination therapy without anticoagulation (hazard ratio 0.28, 95% CI 0.1-0.7). Older age at PAH diagnosis (p = 0.03), mild co-existent interstitial lung disease (ILD) (p = 0.01), worse WHO functional class (p = 0.03) and higher mean pulmonary arterial pressure at PAH diagnosis (p = 0.001), and digital ulcers (p = 0.01) were independent predictors of mortality. Conclusions: Despite the significant benefits conferred by advanced PAH therapies suggested in this study, the median survival in SSc PAH remains short at only 4 years.

KW - Pulmonary arterial hypertension

KW - quality of life

KW - systemic sclerosis

KW - predictors of mortality

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UR - http://purl.org/au-research/grants/NHMRC/1113954

UR - http://purl.org/au-research/grants/NHMRC/1071735

U2 - 10.1186/s13075-017-1341-x

DO - 10.1186/s13075-017-1341-x

M3 - Article

C2 - 28576149

AN - SCOPUS:85020014395

SN - 1478-6354

VL - 19

JO - Arthritis Research and Therapy

JF - Arthritis Research and Therapy

M1 - 122

ER -

Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

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