ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 0330

Baseline Interstitial Lung Disease (ILD) Characteristics and Outcomes in Patients with Anti-synthetase Syndrome Related ILD (ASSD-ILD): Analysis from the “Classification Criteria for Anti-synthetase Syndrome (CLASS)” Project Database

Francisca Bozan1, Sangmee Bae2, Daphne Rivero Gallegos3, Giovanni Zanframundo4, Sara Faghihi-Kashani5, Iazsmin Bauer Ventura6, Eduardo Dourado7, Darosa Lim8, Aravinthan Loganathan9, Gianluca sambataro10, Akira Yoshida11, Francesco Bonella12, Tamera J Corte13, Tracy J Doyle14, david fiorentino15, Miguel Angel Gonzalez-Gay16, marie Hudson17, Masataka Kuwana18, Antonella Notarnicola19, Andrew Mammen20, Neil McHugh21, Frederick Miller22, Carlomaurizio Montecucco23, Chester Oddis24, Jorge Rojas-Serrano25, Jens Schmidt26, Carlo A. Scire27, Ana Villar28, Victoria Werth29, Rohit Aggarwal30 and Lorenzo Cavagna31, 1Hospital Clinico Universidad de Chile, Santiago, Chile, 2UCLA, Los Angeles, CA, 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, 6University of Chicago, Chicago, IL, 7Unidade Local de Saúde da Região de Aveiro, Aveiro, Portugal, 8University of Pennsylvania, Philadelphia, PA, 9RUH, Middle Park, Queensland, Australia, 10University of Catania, Catania, Italy, 11Nippon Medical School Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan, 12Center for interstitial and rare lung diseases, Ruhrlandklinik, University of Duisburg-Essen, Essen, Germany, 13Royal Prince Alfred Hospital, University of Sydney, Sydney, Australia, 14Brigham and Women's Hospital, West Roxbury, MA, 15Department of Dermatology, Stanford University School of Medicine, Stanford, CA, Palo Alto, CA, 16University of Cantabria, Fundación Jimenez Díaz, Madrid, Madrid, Spain, 17McGill University, Montreal, QC, Canada, 18Department of Allergy and Rheumatology, Nippon Medical School, Tokyo, Japan, Tokyo, Japan, 19Karolinska University Hospital and Karolinska Institutet, Stockholm, Stockholms Lan, Sweden, 20NIH, Bethesda, MD, 21University of Bath, Bath, United Kingdom, 22NIH, NIEHS, Chapel Hill, NC, 23IRCCS policlinico S. Matteo foundation, University of Pavia, Pavia, Italy, 24Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, 25National Institute of Respiratory Diseases, Ismael Cosío Villegas, Mexico City, Mexico, 26University Medical Center Goettingen, Göttingen, Germany, 27University of Milano Bicocca, Milan, Italy, 28Systemic Autoimmune Diseases Unit, Internal Medicine Department, Vall d'Hebron University Hospital, Universitat Autònoma of Barcelona, Barcelona, Spain, Barcelona, Spain, 29University of Pennsylvania, Wynnewood, PA, 30Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, 31University of Pavia and Fondazione IRCCS Policlinico San Matteo Hospital of Pavia, Pavia, Pavia, Italy

Meeting: ACR Convergence 2024

Keywords: ,

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: Anti-synthetase syndrome (ASSD) is a systemic autoimmune rheumatic disorder with significant heterogeneity. ILD is the most common cause of mortality and an important prognostic factor associated with poor survival. We aimed to describe the baseline ILD characteristics in patients with ASSD and clinical outcomes of ILD progression and death using the Classification of ASSD (CLASS) project database.

Methods: We utilized the CLASS database, with data entered by local physicians from 92 centers across 30 countries. The database includes comprehensive clinical, serological, radiographic, and physiological data. First, we compared baseline characteristics between ‘active progressive ILD’ or ‘non-progressive ILD’ (includes stable ILD and improved/resolved ILD) per local physician’s discretion at the last follow-up. Second, we performed association analysis using logistic regression between baseline predictor variables and the following outcomes: all-cause mortality, death by ILD progression, home O2 use for ILD, and hospitalization or intensive care unit (ICU) admission for ILD-related respiratory failure.

Results: 915 patients with ASSD-ILD who had follow-up data were included in the analysis (Table 1).  Patients with active progressive ILD were older at ILD onset and, more frequently, smokers. The nonspecific interstitial pneumonia (NSIP) pattern was the most common HRCT ILD pattern in both groups. Patients with active progressive ILD had higher rates of fibrotic HRCT features (honeycombing, traction bronchiectasis) and a usual interstitial pneumonia (UIP) pattern, and baseline FVC and DLCO were significantly lower. The non-progressive ILD group had a higher proportion of anti-Jo1 antibodies. Follow-up data showed that patients with active progressive ILD had a significantly greater number of deaths, the majority due to ILD progression. The active progressive ILD group also had more respiratory failure and hospitalization or ICU admissions.  Older age at ILD onset, NYHA class III/IV at baseline, and positive anti-PL7 and PL-12 antibodies were associated with a worse prognosis (Table 2).

Conclusion: Patients with active progressive ILD had older age at disease onset, were more frequently smokers, and had more fibrotic baseline HRCT features and lower FVC and DLCO at baseline. Age at ILD diagnosis, baseline NYHA class III-IV, and anti-PL7/PL-12 antibodies were associated with a worse prognosis. Early recognition of patients with poor prognostic factors may allow the implementation of more aggressive pharmacological intervention to improve mortality and other meaningful clinical outcomes.

Supporting image 1

Supporting image 2

Disclosures: F. Bozan: None; S. Bae: None; D. Rivero Gallegos: None; G. Zanframundo: None; S. Faghihi-Kashani: None; I. Bauer Ventura: None; E. Dourado: Bial, 6; D. Lim: None; 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: None; A. Villar: 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; 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; L. Cavagna: None.

To cite this abstract in AMA style:

Bozan F, Bae S, Rivero Gallegos D, Zanframundo G, Faghihi-Kashani S, Bauer Ventura I, Dourado E, Lim D, 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, Villar A, Werth V, Aggarwal R, Cavagna L. Baseline Interstitial Lung Disease (ILD) Characteristics and Outcomes in Patients with Anti-synthetase Syndrome Related ILD (ASSD-ILD): Analysis from the “Classification Criteria for Anti-synthetase Syndrome (CLASS)” Project Database [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/baseline-interstitial-lung-disease-ild-characteristics-and-outcomes-in-patients-with-anti-synthetase-syndrome-related-ild-assd-ild-analysis-from-the-classification-criteria-for-anti-synt/. Accessed .

« Back to ACR Convergence 2024

ACR Meeting Abstracts - https://acrabstracts.org/abstract/baseline-interstitial-lung-disease-ild-characteristics-and-outcomes-in-patients-with-anti-synthetase-syndrome-related-ild-assd-ild-analysis-from-the-classification-criteria-for-anti-synt/