Pulmonary hypertension in anti-synthetase syndrome: prevalence, comparative risk across autoimmune diseases, and impact on survival

Abstract Objectives Anti-synthetase syndrome (ASyS) is a distinct entity within the myositis spectrum characterized by interstitial lung disease (ILD) which raises the concern of increased pulmonary vascular pressures. However, the prevalence of pulmonary hypertension (PH) in real-world ASyS populations remains unclear. We investigated the prevalence and impact of PH in ASyS. Methods Data were obtained from a world-wide, physician-reported multicenter registry based on physician-diagnosed ASyS and/or a positive anti-synthetase antibody. Comparative analyses and multivariable logistic regression assessed the odds of PH in ASyS relative to other disease groups. Cox proportional hazards models evaluated associations with all-cause mortality. Results A total of 1,868 ASyS and 2,043 controls from 103 centers were analyzed. Prevalence of PH was 8.9% of ASyS, which was higher than in non-ASyS myositis (3%, p < 0.001), rheumatoid arthritis (4%, p = 0.009), and interstitial pneumonia with autoimmune features (IPAF 3%, p = 0.003), but not significantly different from systemic sclerosis (SSc 13%, p = 0.07) or systemic lupus erythematosus (SLE 11%, p = 0.57). ASyS had higher odds of PH compared to non-ASyS myositis (OR 1.91) and IPAF (OR 3.72), but lower odds than SSc (OR 0.62) or SLE (OR 0.24), in multivariate models adjusted for age, sex, smoking and ILD. Five-year survival in ASyS was 94%, decreasing to 88.3% in those with PH (p = 0.001). PH remained associated with increased mortality in ASyS, independent of age, sex and ILD (HR 2.52, 95% CI 1.41–4.53). Conclusion PH occurred in 8.9% of ASyS and was independently associated with increased mortality, highlighting the importance of PH evaluation in this population.

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Lara D. Veeken
Published
2026-09-29
DOI
https://doi.org/10.1093/rheumatology/keag543
Primary Topic
Inflammatory Myopathies and Dermatomyositis
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article
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article

Pulmonary hypertension in anti-synthetase syndrome: prevalence, comparative risk across autoimmune diseases, and impact on survival

Elena Schiopu, Ana Villar, Eduardo Dourado, Caterina Vacchi et al.
Lara D. Veeken
Inflammatory Myopathies and Dermatomyositis
article

Pulmonary hypertension in anti-synthetase syndrome: prevalence, comparative risk across autoimmune diseases, and impact on survival

Elena Schiopu, Ana Villar, Eduardo Dourado, Caterina Vacchi, Esther Ebstein, Akira Yoshida, Claudia Alpini, Angela Ceribelli, Renata Miossi, Davide Rozza, Francesco Girolamo, Antonella Notarnicola, Carlo Vancheri, Íñigo Rúa‐Figueroa, Lorenzo Cavagna, Florenzo Iannone, Miguel Ángel González-Gay, Franco Franceschini, Rohit Aggarwal, James B Lilleker, Giovanni Franco, Iazsmin Bauer Ventura, Francesca Bottazzi, Christina Charles‐Schoeman, Gianluca Sambataro, Garifallia Sakellariou, Samuel Katsuyuki Shinjo, Alberto Cauli, Anamika Kumari Anuja, DevarasettiPhani Kumar, Alessandro Biglia, Giulia Dei, Christoph Fiehn, Corrado Campochiaro, Giovanni Cagnotto, Domenico Sambataro, Sangmee Sharon Bae, Marco Fornaro, Daniela Ghetie, Francesco Bonella, Farah Tamirou, Fernando Henrique Carlos de Souza, Eugenio Arrigoni, David Lacomis, Upendra Rathore, Andreina Manfredi, Sara Faghihi‐Kashani, Ilaria Cavazzana, Giovanni Barausse, Albert Gil‐Vila, Giacomo Davide De Luca, Fabián Matías Caro, Edoardo Conticini, Hector Chinoy, Elena Bravi, Chester V. Oddis, Darosa Lim, Masataka Kuwana, Domenica Federica Briganti, Fabrizio Luppi, Giovanni Zanframundo, Esther F. Vicente‐Rabaneda, Ernesto Trallero‐Araguás, Laura Bianchessi, Heather Bukiri, Jorge Rojas‐Serrano, Erkan Alpsoy, Siamak Moghadam‐Kia, Adam I. Schiffenbauer, André Pinto Saraiva, Harsha Gunawardena, Helena M. Andersson, George Schett, F. Bozán, Anna Mandel, Daphne Rivero Gallegos, Carlo Selmi, Elizabeth Volkmann, Aravinthan Loganathan, Felipe Reyes, Diana Isabel Perez Roman, Gianluca Erre, the CLASS project participating investigators, Alain Meyer, Latika Gupta, Antony Amato, Chiara Murru, Giandomenco Sebastiani
article en

Abstract

Abstract Objectives Anti-synthetase syndrome (ASyS) is a distinct entity within the myositis spectrum characterized by interstitial lung disease (ILD) which raises the concern of increased pulmonary vascular pressures. However, the prevalence of pulmonary hypertension (PH) in real-world ASyS populations remains unclear. We investigated the prevalence and impact of PH in ASyS. Methods Data were obtained from a world-wide, physician-reported multicenter registry based on physician-diagnosed ASyS and/or a positive anti-synthetase antibody. Comparative analyses and multivariable logistic regression assessed the odds of PH in ASyS relative to other disease groups. Cox proportional hazards models evaluated associations with all-cause mortality. Results A total of 1,868 ASyS and 2,043 controls from 103 centers were analyzed. Prevalence of PH was 8.9% of ASyS, which was higher than in non-ASyS myositis (3%, p < 0.001), rheumatoid arthritis (4%, p = 0.009), and interstitial pneumonia with autoimmune features (IPAF 3%, p = 0.003), but not significantly different from systemic sclerosis (SSc 13%, p = 0.07) or systemic lupus erythematosus (SLE 11%, p = 0.57). ASyS had higher odds of PH compared to non-ASyS myositis (OR 1.91) and IPAF (OR 3.72), but lower odds than SSc (OR 0.62) or SLE (OR 0.24), in multivariate models adjusted for age, sex, smoking and ILD. Five-year survival in ASyS was 94%, decreasing to 88.3% in those with PH (p = 0.001). PH remained associated with increased mortality in ASyS, independent of age, sex and ILD (HR 2.52, 95% CI 1.41–4.53). Conclusion PH occurred in 8.9% of ASyS and was independently associated with increased mortality, highlighting the importance of PH evaluation in this population.

Lara D. Veeken
Universidad de Cantabria (ES), University of Lisbon (PT), University of California, Los Angeles (US), Sanjay Gandhi Post Graduate Institute of Medical Sciences (IN), University of Pittsburgh (US), Universidade de São Paulo (BR), University of California, San Francisco (US), Università degli Studi di Enna Kore (IT), University of Pavia (IT), Logan Hospital (AU), Arthritis Australia (AU), University of Chicago (US), Ruhrlandklinik (DE), Instituto Nacional de Enfermedades Respiratorias (MX), Administração Regional de Saúde de Lisboa e Vale do Tejo (PT), Hospital Universitario Fundación Jiménez Díaz (ES), Hospital Clínico de la Universidad de Chile (CL), Policlinico San Matteo Fondazione (IT), The Royal Wolverhampton NHS Trust (GB), Wolverhampton Hospital (GB), Instituto de Investigación Sanitaria Fundación Jiménez Díaz (ES), University of Milano-Bicocca (IT), Nippon Medical School (JP)
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