Cardiac Manifestations in Patients with Anti-Synthetase Syndrome: Analysis from the “Classification Criteria for Anti-synthetase Syndrome (CLASS)” Project Database

Sangmee Bae¹, Gianluca sambataro², iazsmin Ventura³, Francisca Bozan⁴, Eduardo Dourado⁵, Sara Faghihi-Kashani⁶, Aravinthan Loganathan⁷, Daphne Rivero Gallegos⁸, Akira Yoshida⁹, Giovanni Zanframundo¹⁰, Francesco Bonella¹¹, Tamera J Corte¹², Tracy J Doyle¹³, david fiorentino¹⁴, Miguel Angel Gonzalez-Gay¹⁵, marie Hudson¹⁶, Masataka Kuwana¹⁷, Andrew Mammen¹⁸, Neil McHugh¹⁹, Frederick Miller²⁰, Carlomaurizio Montecucco²¹, Antonella Notarnicola²², Chester Oddis²³, Jorge Rojas-Serrano²⁴, Jens Schmidt²⁵, Carlo A. Scire²⁶, Albert Gil-Vila²⁷, Victoria Werth²⁸, Lorenzo Cavagna²⁹ and Rohit Aggarwal³⁰, ¹UCLA, Los Angeles, CA, ²University of Catania, Catania, Italy, ³Section of Rheumatology, University of Chicago, Chicago, IL, ⁴Hospital Clinico Universidad de Chile, Santiago, Chile, ⁵Unidade Local de Saúde da Região de Aveiro, Aveiro, Portugal, ⁶Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, San Francisco, CA, ⁷Royal National Hospital for Rheumatic Diseases, Bath, United Kingdom, ⁸INER, Ciudad de México, Mexico State, Mexico, ⁹Nippon Medical School Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan, ¹⁰Università di Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy, Milano, Italy, ¹¹Center for interstitial and rare lung diseases, Ruhrlandklinik, University of Duisburg-Essen, Essen, Germany, ¹²Royal Prince Alfred Hospital, University of Sydney, Sydney, Australia, ¹³Brigham and Women's Hospital, West Roxbury, MA, ¹⁴Department of Dermatology, Stanford University School of Medicine, Stanford, CA, Palo Alto, CA, ¹⁵University of Cantabria, Fundación Jimenez Díaz, Madrid, Madrid, Spain, ¹⁶McGill University, Montreal, QC, Canada, ¹⁷Department of Allergy and Rheumatology, Nippon Medical School, Tokyo, Japan, Tokyo, Japan, ¹⁸NIH, Bethesda, MD, ¹⁹University of Bath, Bath, United Kingdom, ²⁰NIH, NIEHS, Chapel Hill, NC, ²¹IRCCS policlinico S. Matteo foundation, University of Pavia, Pavia, Italy, ²²Karolinska University Hospital and Karolinska Institutet, Stockholm, Stockholms Lan, Sweden, ²³Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, ²⁴National Institute of Respiratory Diseases, Ismael Cosío Villegas, Mexico City, Mexico, ²⁵University Medical Center Goettingen, Göttingen, Germany, ²⁶University of Milano Bicocca, Milan, Italy, ²⁷Universitat Autònoma of Barcelona, Barcelona, Spain, ²⁸University of Pennsylvania, Wynnewood, PA, ²⁹University of Pavia and Fondazione IRCCS Policlinico San Matteo Hospital of Pavia, Pavia, Pavia, Italy, ³⁰Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA

Meeting: ACR Convergence 2024

Keywords: autoimmune diseases, Cardiovascular, Myositis, pulmonary

Session Information

Date: Saturday, November 16, 2024

Title: Muscle Biology, Myositis & Myopathies – Basic & Clinical Science Poster I

Session Type: Poster Session A

Session Time: 10:30AM-12:30PM

Background/Purpose: The prevalence of cardiac manifestations has not been comprehensively described in anti-synthetase syndrome (ASSD). In the current study, we report the prevalence of cardiac involvement in ASSD patients compared to non-ASSD IIM and other mimicking conditions.

Methods: We utilized the CLASS (Classification Criteria of Anti-Synthetase Syndrome) project database, comprised of physician-reported retrospective data from 92 centers around the world. Cardiac involvement variables included myocarditis, pericarditis, arrhythmias, nonischemic cardiomyopathy, ischemic cardiomyopathy, and pulmonary hypertension (PH). Information on interstitial lung disease (ILD) and lung physiology including forced vital capacity (FVC) and diffusing capacity (DLCO) were also analyzed. Prevalence was calculated among patients with data entered, excluding those with missing data.

Results: We included 1953 ASSD cases and 2093 controls (914 non-ASSD IIM controls). The comparator group included 44% non-ASSD IIM (28% dermatomyositis, 9% polymyositis, 7% other IIM), 11% rheumatoid arthritis, 11% systemic sclerosis, 8% interstitial pneumonia with autoimmune features, 5% systemic lupus erythematosus, 5% Sjogren’s, and 16% others. The cohort mean age was 52 years and 29% were male which were similar in ASSD and controls (Table 1). ASSD patients had a higher proportion of ever smokers and higher mean body mass index.

Cardiac involvement was reported in 15% of ASSD cases, which was higher compared to all controls and compared to non-ASSD IIM controls (Table 1). The most frequently reported cardiac manifestation in ASSD was PH, with a prevalence of 8.4%. PH in ASSD cases was mostly group 3 PH due to ILD, which was different from controls in which group 1 PH was more common. These results were attributable to the high prevalence of ILD (86%) in ASSD cases vs controls. Pulmonary function test results showed significantly lower FVC and DLCO in ASSD compared to controls.

The prevalences of arrhythmias (2.9%), nonischemic cardiomyopathy (2%), myocarditis (1.8%), pericarditis (1.8%), and ischemic cardiomyopathy (0.8%) in ASSD were not significantly different compared to all controls. Compared to non-ASSD IIM controls, ASSD cases had a significantly lower prevalence of arrhythmias. The most common method for the detection of arrhythmias was electrocardiogram (67%), for myocarditis, cardiac magnetic resonance imaging (58%), and for pericarditis and cardiomyopathy, echocardiogram (31-75%).

We also analyzed cardiac manifestations in ASSD cases by antibody subgroups. Anti-Jo-1 patients did not have significant differences in their cardiac manifestations compared to non-Jo-1 patients (Table 2). Anti-PL-7 patients had a numerically greater proportion of pericarditis.

Conclusion: The prevalence of cardiac involvement was 15% in patients with ASSD. PH (mostly group 3 with concomitant ILD) was the most common manifestation. Arrhythmias, cardiomyopathy, and pericarditis were seen in less than 3% of ASSD cases, which was not different from controls. The prevalence of cardiac involvement was similar in anti-Jo-1 and non-Jo-1 antibody subgroups.

Values are n(%) or mean±SD. All values calculated after excluding missing data for any given variable. Prevalence of each variable is calculated within the total of the category (ex. myocarditis 13% of ASSD patients with cardiac involvement)
*marked group with significantly higher value between ASSD vs All control (p<0.05)
†marked group with significantly higher value between ASSD vs non-ASS IIM (p<0.05)
aGroup 1 primary pulmonary artery hypertension, Group 3 secondary pulmonary hypertension due to ILD, Other PH includes pulmonary embolism, due to heart disease including systolic or diastolic dysfunction
bIntermediate or high risk of PH according to 2015 ERS/ESC guidelines
cThe most abnormal or severe New York Heart Association funcitonal class patient ever had
Abbreviation: PAP, pulmonary artery pressure; PFT, pulmonary function test; FVC, forced vital capacity; DLCO, diffusing capacity using carbon monoxide; NYHA, New York Heart Association functional class

Among the cases, 89% had antibody results reported and were included in this table.
Values are n(%) or mean±SD. All % calculated after excluding missing data for any given variable. Prevalence of each variable is calculated within the total of the category
P value calculated comparing Anti Jo_1 patients to all non-Jo_1 patients

Disclosures: S. Bae: None; G. sambataro: Boehringer-Ingelheim, 6; i. Ventura: None; F. Bozan: None; E. Dourado: Bial, 6; S. Faghihi-Kashani: None; A. Loganathan: None; D. Rivero Gallegos: None; A. Yoshida: None; G. Zanframundo: None; F. Bonella: None; T. J Corte: 4D, 2, 5, Avalyn Therapeutics, 1, 2, 5, Boehringer-Ingelheim, 1, 2, 5, 6, 12, Support for attending meetings and/or travel, Bridge Biotherapeutics, 1, 2, 5, Bristol-Myers Squibb (BMS), 1, 2, 5, 6, 12, Support for attending meetings and/or travel, Cincera, 2, DevPro, 1, 2, Endeavour BioMedicine, 1, 2, Pharmaxis, 2, 5, Pliant, 1, 2, 5, Roche, 1, 2, 5, 6; T. J Doyle: Bayer, 5, Sanofi, 3; d. fiorentino: Argenyx, 2, biogen, 2, bus, 2, johnson & johnson, 2, kyverna, 2, 5, Pfizer, 2, Priovant, 5, 12, gift, Serono, 5, usb, 2; M. Gonzalez-Gay: None; m. Hudson: AstraZeneca, 5, Boehringer-Ingelheim, 5, Bristol-Myers Squibb(BMS), 5, Pfizer, 5; M. Kuwana: Asahi Kasei Pharma, 6, AstraZeneca, 2, Boehringer Ingelheim, 2, 6, Chugai, 2, 6, GSK, 2, MBL, 9, Ono Pharmaceuticals, 6; A. Mammen: None; N. McHugh: None; F. Miller: None; C. Montecucco: None; A. Notarnicola: Boehringer-Ingelheim, 1, 6; C. Oddis: Abcuro, 5, Alexion, 5, Argenx, 5, Boehringer Ingelheim, 5, CSL Behring, 5, EMD Serono, 5, Janssen, 5, Pfizer, 5, Priovant, 5; J. Rojas-Serrano: None; J. Schmidt: CSL Behring, 2, 6, 12, Support for attending meetings and/or travel, Grifols, 2, Janssen, 2, Kezar, 5, Takeda, 2, 6, UCB, 6; C. Scire: None; A. Gil-Vila: None; V. Werth: AbbVie/Abbott, 2, Alpine immune sciences, 2, Amgen, 1, 5, anaptysbio, 2, AstraZeneca, 2, 5, Biogen, 2, 5, BMS, 2, 5, Cabaletta Bio, 2, Calyx, 2, Caribou, 2, Corbus, 5, CSL Behring, 2, 5, Cugene, 2, Evommune, 2, Gilead, 2, 5, GSK, 2, Horizon, 2, 5, Immunovant, 2, Innovaderm, 2, Janssen, 2, Lilly, 2, Merck, 2, Nuvig Pharmaceuticals, 2, Pfizer, 2, 5, Priovant, 5, Regeneron, 1, 5, Rome Pharmaceuticals, 2, 5, Sanofi, 2, Takeda, 2, UCB, 2, Ventus, 2, 5, Viela, 5, Xencor, 2; L. Cavagna: None; R. Aggarwal: Alexion, 2, ANI Pharmaceuticals, 2, Argenx, 2, AstraZeneca, 2, Boehringer-Ingelheim, 2, 5, Bristol-Myers Squibb(BMS), 2, 5, CabalettaBio, 2, Capella Bioscience, 2, Capstanx, 2, Corbus, 2, CSL Behring, 2, EMD Serono, 2, 5, Galapagos, 2, Horizon Therapeutics, 2, I-Cell, 2, Immunovant, 2, Janssen, 1, 2, 5, Kezar, 2, Kyverna, 2, Lilly, 2, Mallinckrodt, 5, Manta Medicines Corporation, 2, Merck, 2, Novartis, 2, Nuvig Therapeutics, 2, Octapharma, 2, Pfizer, 2, 5, Q32, 5, Roivant, 2, Sanofi, 2, Teva, 2.

To cite this abstract in AMA style:

Bae S, sambataro G, Ventura i, Bozan F, Dourado E, Faghihi-Kashani S, Loganathan A, Rivero Gallegos D, Yoshida A, Zanframundo G, Bonella F, J Corte T, J Doyle T, fiorentino d, Gonzalez-Gay M, Hudson m, Kuwana M, Mammen A, McHugh N, Miller F, Montecucco C, Notarnicola A, Oddis C, Rojas-Serrano J, Schmidt J, Scire C, Gil-Vila A, Werth V, Cavagna L, Aggarwal R. Cardiac Manifestations in Patients with Anti-Synthetase Syndrome: Analysis from the “Classification Criteria for Anti-synthetase Syndrome (CLASS)” Project Database [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/cardiac-manifestations-in-patients-with-anti-synthetase-syndrome-analysis-from-the-classification-criteria-for-anti-synthetase-syndrome-class-project-database/. Accessed .

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