Anti-synthetase Syndrome (ASSD) Related Interstitial Lung Disease (ILD) in Comparison to Non-ASSD Related ILDs: Analysis from the “Classification Criteria for Anti-synthetase Syndrome (CLASS)” Project Database

Sangmee Bae¹, Francisca Bozan², Daphne Rivero Gallegos³, Giovanni Zanframundo⁴, Sara Faghihi-Kashani⁵, iazsmin Ventura⁶, Eduardo Dourado⁷, Aravinthan Loganathan⁸, Gianluca sambataro⁹, Akira Yoshida¹⁰, Francesco Bonella¹¹, Tamera J Corte¹², Tracy J Doyle¹³, david fiorentino¹⁴, Miguel Angel Gonzalez-Gay¹⁵, marie Hudson¹⁶, Masataka Kuwana¹⁷, Antonella Notarnicola¹⁸, Andrew Mammen¹⁹, Neil McHugh²⁰, Frederick Miller²¹, Carlomaurizio Montecucco²², Chester Oddis²³, Jorge Rojas-Serrano²⁴, Jens Schmidt²⁵, Carlo Scire²⁶, Albert Selva-O’Callaghan²⁷, Victoria Werth²⁸, Lorenzo Cavagna²⁹ and Rohit Aggarwal³⁰, ¹UCLA, Los Angeles, CA, ²Hospital Clinico Universidad de Chile, Santiago, Chile, ³INER, Ciudad de México, Mexico State, Mexico, ⁴Università di Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy, Milano, Italy, ⁵Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, San Francisco, CA, ⁶Section of Rheumatology, University of Chicago, Chicago, IL, ⁷Unidade Local de Saúde da Região de Aveiro, Aveiro, Portugal, ⁸RUH, Middle Park, Queensland, Australia, ⁹University of Catania, Catania, Italy, ¹⁰Nippon Medical School Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan, 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, ¹⁸Karolinska University Hospital and Karolinska Institutet, Stockholm, Stockholms Lan, Sweden, ¹⁹NIH, Bethesda, MD, ²⁰University of Bath, Bath, United Kingdom, ²¹NIH, NIEHS, Chapel Hill, NC, ²²IRCCS policlinico S. Matteo foundation, University of Pavia, Pavia, Italy, 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, ²⁷Internal Medicine Department, Universitat Autònoma de Barcelona, Barcelona, Spain, ²⁸University of Pennsylvania, Wynnewood, PA, ²⁹University of Pavia, Pavia, Italy, ³⁰Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA

Meeting: ACR Convergence 2024

Keywords: interstitial lung disease, Myopathies, Myositis

Session Information

Date: Sunday, November 17, 2024

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

Session Type: Poster Session B

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

Background/Purpose: Anti-synthetase syndrome (ASSD) is a subset of idiopathic inflammatory myopathy characterized by autoantibodies directed against aminoacyl tRNA synthetases. Interstitial lung disease (ILD) can be the first and most prominent feature of ASSD. Our current study aims to describe the characteristics of ASSD-related interstitial lung disease (ASSD-ILD) in comparison to non-ASSD-related ILDs.

Methods: We utilized the Classification Criteria for Anti-synthetase Syndrome (CLASS) project database, which has physician-reported data from 92 centers across 30 countries, to identify patients with ASSD-ILD (cases) and ILD related to ASSD mimicking conditions (controls). Demographic, clinical, radiographic, and physiological data were compared between cases and controls using chi-square tests for categorical variables and Student’s t-test for continuous variables. The association of cases (versus controls) with all-cause mortality was analyzed using logistic regression adjusted for follow-up time.

Results: A total of 2501 patients with ILD were identified, 1667 cases and 834 controls (Table 1). The comparator group included patients with dermatomyositis (15%), systemic sclerosis (18%), rheumatoid arthritis (14%), interstitial pneumonia with autoimmune features without anti-synthetase antibodies (IPAF, 20%), idiopathic pulmonary fibrosis (8%) and other mimicking conditions (24%). ASSD-ILD patients were younger at disease onset and included fewer Hispanics.

ASSD-ILD cases were more likely to present with acute progressive disease (within 3 months) and were more often symptomatic with cough and dyspnea. The controls were more likely to present with chronic, asymptomatic disease.

The pulmonary function tests at diagnosis showed significantly lower forced vital capacity and diffusing capacity of carbon monoxide in ASSD-ILD compared to controls. The most common ILD pattern on high-resolution chest computed tomography (HRCT) at the time of diagnosis was the nonspecific interstitial pneumonia (NSIP) pattern for both groups. Organizing pneumonia (OP) was more common in ASSD-ILD, while the usual interstitial pneumonia (UIP) pattern was more common in controls. ASSD-ILD HRCT scans had more ground glass, while controls had more reticulation and honeycombing.

ASSD-ILD patients had significantly higher rates of hospitalization for respiratory failure. Survival data was available in 65% of the dataset (60% of cases and 74% in controls) over a follow-up time of 4 [2-8] years, median [interquartile range]. All-cause mortality was 7.7% in ASSD-ILD patients and 4.3% in controls (p=0.004). ASSD-ILD had an increased odds for death compared to controls, which remained significant after adjustment for follow-up time (OR [95%CI] 2.36 [1.42-3.93], p=0.0004).

Conclusion: Patients with ASSD-ILD were more likely to have symptomatic ILD and a more acute presentation, and had higher rates of hospitalization compared to patients with non-ASSD-related ILD. Higher odds of all-cause mortality in ASSD-ILD cases compared to comparators highlight the importance of early recognition and the need for future work to identify poor prognostic factors in these patients.

Data is presented as n(%) or mean±SD. P value calculated with chi-square test or Student’s t-test
Abbreviations: NYHA, New York Heart Association functional class; HRCT, high resolution computed tomography; UIP, Usual interstitial pneumonia; NSIP, Nonspecific interstitial pneumonia; OP, organizing pneumonia; DAD, diffuse alveolar damage; LIP, lymphoid interstitial pneumonia; FVC, forced vital capacity; FEV1, forced expiratory volume in 1 second; DLCO, diffusing capacity for carbon monoxide; ICU, intensive care unit

Disclosures: S. Bae: None; F. Bozan: None; D. Rivero Gallegos: None; G. Zanframundo: None; S. Faghihi-Kashani: None; i. Ventura: None; E. Dourado: Bial, 6; A. Loganathan: None; G. sambataro: Boehringer-Ingelheim, 6; A. Yoshida: 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. Notarnicola: Boehringer-Ingelheim, 1, 6; A. Mammen: None; N. McHugh: None; F. Miller: None; C. Montecucco: None; 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: AbbVie/Abbott, 5, Eli Lilly, 5, Galapagos, 5; A. Selva-O’Callaghan: 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, Bozan F, Rivero Gallegos D, Zanframundo G, Faghihi-Kashani S, Ventura i, Dourado E, Loganathan A, sambataro G, Yoshida A, Bonella F, J Corte T, J Doyle T, fiorentino d, Gonzalez-Gay M, Hudson m, Kuwana M, Notarnicola A, Mammen A, McHugh N, Miller F, Montecucco C, Oddis C, Rojas-Serrano J, Schmidt J, Scire C, Selva-O’Callaghan A, Werth V, Cavagna L, Aggarwal R. Anti-synthetase Syndrome (ASSD) Related Interstitial Lung Disease (ILD) in Comparison to Non-ASSD Related ILDs: Analysis from the “Classification Criteria for Anti-synthetase Syndrome (CLASS)” Project Database [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/anti-synthetase-syndrome-assd-related-interstitial-lung-disease-ild-in-comparison-to-non-assd-related-ilds-analysis-from-the-classification-criteria-for-anti-synthetase-syndrome-class/. Accessed .

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