SSc-PAH patients are at risk of disease progression in contemporary clinical practice

<bold>Background:</bold> The effect of upfront treatment for systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is not clear. <bold>Objectives:</bold> To assess the impact of upfront treatment on progressive SSc-PAH. <bold>Methods:</bold> We included treatment-naïve SSc-PAH patients according to the new hemodynamic definition<sup>1</sup> from the EUSTAR registry (CP122). Progressive PAH was defined as death, atrial septostomy, lung transplantation, initiation of IV/SC prostanoids or long-term oxygen therapy, or “other worsening” (≥15% worsening of 6MWD and worsening of symptoms or new PAH treatment) within 36 months. Risk was assessed using COMPERA 2.0. Cox regression was used to assess impact on PAH progression. <bold>Results:</bold> Of 243 SSc-PAH patients, 114 (38%) progressed within 36 months. Patients who progressed had higher baseline risk profiles and worse hemodynamics, while 29% received upfront combination therapy (Fig. 1A). Multivariable analysis showed that SSc patients with a higher risk profile, lower DLCO, higher mPAP and PVR, and lower PAWP had more progressive PAH, while upfront treatment did not significantly reduce progression (Fig. 1B). <bold>Conclusions:</bold> Despite a higher baseline risk profile and worse hemodynamics in progressive SSc-PAH patients, a small proportion received upfront combination therapy. Therapeutic optimization aligned with treatment guidelines is warranted. <bold>References:</bold> <sup>1</sup>Humbert et al., Eur Heart J, 2022 <fig><object-id>erj;64/suppl_68/PA2493/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>

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Published
2024-09-14
DOI
https://doi.org/10.1183/13993003.congress-2024.pa2493
Primary Topic
Systemic Sclerosis and Related Diseases
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article

SSc-PAH patients are at risk of disease progression in contemporary clinical practice

Anna‐Maria Hoffmann‐Vold, Helle Bitter, Ivan Castellvi, Øyvind Molberg et al.
Systemic Sclerosis and Related Diseases
article

SSc-PAH patients are at risk of disease progression in contemporary clinical practice

Anna‐Maria Hoffmann‐Vold, Helle Bitter, Ivan Castellvi, Øyvind Molberg, H. J. Bjørkekjær, Yoshiya Tanaka, Alexandra Balbir‐Gurman, Gabriela Riemekasten, Cathrine Brunborg, Sverre Høie, Kaspar Broch, Øyvind Midtvedt, Paolo Airó, Marco Matucci‐Cerinic, Marie‐Elise Truchetet, Patricia E. Carreira, Cosimo Bruni, Elise Siegert, Oliver Distler, Mickaël Martin, Håvard Fretheim, Carmen Pilar Simeón‐Aznar, Armando Gabrielli, Christopher P. Denton, Ulf Müller-Ladner, Eric Hachulla
article en

Abstract

<bold>Background:</bold> The effect of upfront treatment for systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is not clear. <bold>Objectives:</bold> To assess the impact of upfront treatment on progressive SSc-PAH. <bold>Methods:</bold> We included treatment-naïve SSc-PAH patients according to the new hemodynamic definition<sup>1</sup> from the EUSTAR registry (CP122). Progressive PAH was defined as death, atrial septostomy, lung transplantation, initiation of IV/SC prostanoids or long-term oxygen therapy, or “other worsening” (≥15% worsening of 6MWD and worsening of symptoms or new PAH treatment) within 36 months. Risk was assessed using COMPERA 2.0. Cox regression was used to assess impact on PAH progression. <bold>Results:</bold> Of 243 SSc-PAH patients, 114 (38%) progressed within 36 months. Patients who progressed had higher baseline risk profiles and worse hemodynamics, while 29% received upfront combination therapy (Fig. 1A). Multivariable analysis showed that SSc patients with a higher risk profile, lower DLCO, higher mPAP and PVR, and lower PAWP had more progressive PAH, while upfront treatment did not significantly reduce progression (Fig. 1B). <bold>Conclusions:</bold> Despite a higher baseline risk profile and worse hemodynamics in progressive SSc-PAH patients, a small proportion received upfront combination therapy. Therapeutic optimization aligned with treatment guidelines is warranted. <bold>References:</bold> <sup>1</sup>Humbert et al., Eur Heart J, 2022 <fig><object-id>erj;64/suppl_68/PA2493/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>

University of Verona (IT), Marche Polytechnic University (IT), Hebron University (PS), Oslo University Hospital (NO), Good Samaritan Hospital (US), Université de Bordeaux (FR), Vita-Salute San Raffaele University (IT), Inserm (FR), University of Occupational and Environmental Health Japan (JP), Université de Lille (FR), The Royal Free Hospital (GB), Rambam Health Care Campus (IL), Hospital de Sant Pau (ES), Hospital of Southern Norway (NO), Centre Hospitalier Universitaire de Lille (FR), Université de Poitiers (FR), Research Institute Hospital 12 de Octubre (ES), Sørlandet Hospital Arendal (NO), University Hospital of Zurich (CH), Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia (IT), Azienda Ospedaliero-Universitaria Careggi (IT), Rappaport Family Institute for Research in the Medical Sciences (IL), Vall d'Hebron Hospital Universitari (ES), Kerckhoff Klinik (DE), University College London (GB), University of Lübeck (DE)
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Openalex Percentile: Top 68%
Systemic Sclerosis and Related Diseases
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