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

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 e...
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Autor*in:	Fairley, Jessica L. [verfasserIn] Hansen, Dylan Ross, Laura Proudman, Susanna Sahhar, Joanne Ngian, Gene-Siew Walker, Jennifer Host, Lauren V. Morrisroe, Kathleen Apostolopoulous, Diane Ferdowsi, Nava Wilson, Michelle Tabesh, Maryam Stevens, Wendy Nikpour, Mandana

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2023

Schlagwörter:	Pulmonary hypertension Interstitial lung disease Survival Quality of life

Anmerkung:	© The Author(s) 2023

Übergeordnetes Werk:	Enthalten in: Arthritis Research & Therapy - London : BioMed Central, 1999, 25(2023), 1 vom: 12. Mai
Übergeordnetes Werk:	volume:25 ; year:2023 ; number:1 ; day:12 ; month:05

Links:	Volltext

DOI / URN:	10.1186/s13075-023-03059-x

Katalog-ID:	SPR052440842

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100	1		\|a Fairley, Jessica L. \|e verfasserin \|4 aut
245	1	0	\|a Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis
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520			\|a 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.
650		4	\|a Pulmonary hypertension \|7 (dpeaa)DE-He213
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700	1		\|a Sahhar, Joanne \|4 aut
700	1		\|a Ngian, Gene-Siew \|4 aut
700	1		\|a Walker, Jennifer \|4 aut
700	1		\|a Host, Lauren V. \|4 aut
700	1		\|a Morrisroe, Kathleen \|4 aut
700	1		\|a Apostolopoulous, Diane \|4 aut
700	1		\|a Ferdowsi, Nava \|4 aut
700	1		\|a Wilson, Michelle \|4 aut
700	1		\|a Tabesh, Maryam \|4 aut
700	1		\|a Stevens, Wendy \|4 aut
700	1		\|a Nikpour, Mandana \|4 aut
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allfields	10.1186/s13075-023-03059-x doi (DE-627)SPR052440842 (SPR)s13075-023-03059-x-e DE-627 ger DE-627 rakwb eng Fairley, Jessica L. verfasserin aut Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 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. Pulmonary hypertension (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Survival (dpeaa)DE-He213 Quality of life (dpeaa)DE-He213 Hansen, Dylan aut Ross, Laura aut Proudman, Susanna aut Sahhar, Joanne aut Ngian, Gene-Siew aut Walker, Jennifer aut Host, Lauren V. aut Morrisroe, Kathleen aut Apostolopoulous, Diane aut Ferdowsi, Nava aut Wilson, Michelle aut Tabesh, Maryam aut Stevens, Wendy aut Nikpour, Mandana aut Enthalten in Arthritis Research & Therapy London : BioMed Central, 1999 25(2023), 1 vom: 12. Mai (DE-627)326646418 (DE-600)2041668-4 1478-6354 nnns volume:25 year:2023 number:1 day:12 month:05 https://dx.doi.org/10.1186/s13075-023-03059-x kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 25 2023 1 12 05
spelling	10.1186/s13075-023-03059-x doi (DE-627)SPR052440842 (SPR)s13075-023-03059-x-e DE-627 ger DE-627 rakwb eng Fairley, Jessica L. verfasserin aut Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 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. Pulmonary hypertension (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Survival (dpeaa)DE-He213 Quality of life (dpeaa)DE-He213 Hansen, Dylan aut Ross, Laura aut Proudman, Susanna aut Sahhar, Joanne aut Ngian, Gene-Siew aut Walker, Jennifer aut Host, Lauren V. aut Morrisroe, Kathleen aut Apostolopoulous, Diane aut Ferdowsi, Nava aut Wilson, Michelle aut Tabesh, Maryam aut Stevens, Wendy aut Nikpour, Mandana aut Enthalten in Arthritis Research & Therapy London : BioMed Central, 1999 25(2023), 1 vom: 12. Mai (DE-627)326646418 (DE-600)2041668-4 1478-6354 nnns volume:25 year:2023 number:1 day:12 month:05 https://dx.doi.org/10.1186/s13075-023-03059-x kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 25 2023 1 12 05
allfields_unstemmed	10.1186/s13075-023-03059-x doi (DE-627)SPR052440842 (SPR)s13075-023-03059-x-e DE-627 ger DE-627 rakwb eng Fairley, Jessica L. verfasserin aut Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 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. Pulmonary hypertension (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Survival (dpeaa)DE-He213 Quality of life (dpeaa)DE-He213 Hansen, Dylan aut Ross, Laura aut Proudman, Susanna aut Sahhar, Joanne aut Ngian, Gene-Siew aut Walker, Jennifer aut Host, Lauren V. aut Morrisroe, Kathleen aut Apostolopoulous, Diane aut Ferdowsi, Nava aut Wilson, Michelle aut Tabesh, Maryam aut Stevens, Wendy aut Nikpour, Mandana aut Enthalten in Arthritis Research & Therapy London : BioMed Central, 1999 25(2023), 1 vom: 12. Mai (DE-627)326646418 (DE-600)2041668-4 1478-6354 nnns volume:25 year:2023 number:1 day:12 month:05 https://dx.doi.org/10.1186/s13075-023-03059-x kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 25 2023 1 12 05
allfieldsGer	10.1186/s13075-023-03059-x doi (DE-627)SPR052440842 (SPR)s13075-023-03059-x-e DE-627 ger DE-627 rakwb eng Fairley, Jessica L. verfasserin aut Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 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. Pulmonary hypertension (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Survival (dpeaa)DE-He213 Quality of life (dpeaa)DE-He213 Hansen, Dylan aut Ross, Laura aut Proudman, Susanna aut Sahhar, Joanne aut Ngian, Gene-Siew aut Walker, Jennifer aut Host, Lauren V. aut Morrisroe, Kathleen aut Apostolopoulous, Diane aut Ferdowsi, Nava aut Wilson, Michelle aut Tabesh, Maryam aut Stevens, Wendy aut Nikpour, Mandana aut Enthalten in Arthritis Research & Therapy London : BioMed Central, 1999 25(2023), 1 vom: 12. Mai (DE-627)326646418 (DE-600)2041668-4 1478-6354 nnns volume:25 year:2023 number:1 day:12 month:05 https://dx.doi.org/10.1186/s13075-023-03059-x kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 25 2023 1 12 05
allfieldsSound	10.1186/s13075-023-03059-x doi (DE-627)SPR052440842 (SPR)s13075-023-03059-x-e DE-627 ger DE-627 rakwb eng Fairley, Jessica L. verfasserin aut Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 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. Pulmonary hypertension (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Survival (dpeaa)DE-He213 Quality of life (dpeaa)DE-He213 Hansen, Dylan aut Ross, Laura aut Proudman, Susanna aut Sahhar, Joanne aut Ngian, Gene-Siew aut Walker, Jennifer aut Host, Lauren V. aut Morrisroe, Kathleen aut Apostolopoulous, Diane aut Ferdowsi, Nava aut Wilson, Michelle aut Tabesh, Maryam aut Stevens, Wendy aut Nikpour, Mandana aut Enthalten in Arthritis Research & Therapy London : BioMed Central, 1999 25(2023), 1 vom: 12. Mai (DE-627)326646418 (DE-600)2041668-4 1478-6354 nnns volume:25 year:2023 number:1 day:12 month:05 https://dx.doi.org/10.1186/s13075-023-03059-x kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 25 2023 1 12 05
language	English
source	Enthalten in Arthritis Research & Therapy 25(2023), 1 vom: 12. Mai volume:25 year:2023 number:1 day:12 month:05
sourceStr	Enthalten in Arthritis Research & Therapy 25(2023), 1 vom: 12. Mai volume:25 year:2023 number:1 day:12 month:05
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	Pulmonary hypertension Interstitial lung disease Survival Quality of life
isfreeaccess_bool	true
container_title	Arthritis Research & Therapy
authorswithroles_txt_mv	Fairley, Jessica L. @@aut@@ Hansen, Dylan @@aut@@ Ross, Laura @@aut@@ Proudman, Susanna @@aut@@ Sahhar, Joanne @@aut@@ Ngian, Gene-Siew @@aut@@ Walker, Jennifer @@aut@@ Host, Lauren V. @@aut@@ Morrisroe, Kathleen @@aut@@ Apostolopoulous, Diane @@aut@@ Ferdowsi, Nava @@aut@@ Wilson, Michelle @@aut@@ Tabesh, Maryam @@aut@@ Stevens, Wendy @@aut@@ Nikpour, Mandana @@aut@@
publishDateDaySort_date	2023-05-12T00:00:00Z
hierarchy_top_id	326646418
id	SPR052440842
language_de	englisch
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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. 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author	Fairley, Jessica L.
spellingShingle	Fairley, Jessica L. misc Pulmonary hypertension misc Interstitial lung disease misc Survival misc Quality of life Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis
authorStr	Fairley, Jessica L.
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topic_title	Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis Pulmonary hypertension (dpeaa)DE-He213 Interstitial lung disease (dpeaa)DE-He213 Survival (dpeaa)DE-He213 Quality of life (dpeaa)DE-He213
topic	misc Pulmonary hypertension misc Interstitial lung disease misc Survival misc Quality of life
topic_unstemmed	misc Pulmonary hypertension misc Interstitial lung disease misc Survival misc Quality of life
topic_browse	misc Pulmonary hypertension misc Interstitial lung disease misc Survival misc Quality of life
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title	Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis
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title_full	Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis
author_sort	Fairley, Jessica L.
journal	Arthritis Research & Therapy
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author_browse	Fairley, Jessica L. Hansen, Dylan Ross, Laura Proudman, Susanna Sahhar, Joanne Ngian, Gene-Siew Walker, Jennifer Host, Lauren V. Morrisroe, Kathleen Apostolopoulous, Diane Ferdowsi, Nava Wilson, Michelle Tabesh, Maryam Stevens, Wendy Nikpour, Mandana
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author-letter	Fairley, Jessica L.
doi_str_mv	10.1186/s13075-023-03059-x
title_sort	clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis
title_auth	Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis
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. © The Author(s) 2023
abstractGer	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. © The Author(s) 2023
abstract_unstemmed	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. © The Author(s) 2023
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title_short	Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis
url	https://dx.doi.org/10.1186/s13075-023-03059-x
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author2	Hansen, Dylan Ross, Laura Proudman, Susanna Sahhar, Joanne Ngian, Gene-Siew Walker, Jennifer Host, Lauren V. Morrisroe, Kathleen Apostolopoulous, Diane Ferdowsi, Nava Wilson, Michelle Tabesh, Maryam Stevens, Wendy Nikpour, Mandana
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