Abstracts tagged "interstitial lung disease"

Abstract Number: 2139 • ACR Convergence 2025
Real-Life Treatment Strategies for Refractory Still’s Disease: Results from a Worldwide Survey, the METAPHOR Project
Greta Rogani¹, Francesco Baldo², Claudia Bracaglia³, Dirk Foell⁴, Marco Gattorno⁵, Marija Jelusic⁶, Jordi Anton⁷, Paul Brogan⁸, Scott Canna⁹, Randy Cron¹⁰, Fabrizio De Benedetti¹¹, Alexei Grom¹², Merav Heshin Bekenstein¹³, AnnaCarin Horne¹⁴, Raju Khubchandani¹⁵, Mao Mizuta¹⁶, Seza Özen¹⁷, Pierre Quartier Dit Maire¹⁸, Angelo Ravelli¹⁹, Masaki Shimizu²⁰, Grant Schulert¹², Christiaan Scott²¹, Rashmi Sinha²², Nicolino Ruperto²³, Joost Swart²⁴, Bruno Fautrel²⁵, Sebastiaan Vastert¹ and Francesca Minoia²⁶, ¹University Medical Center Utrecht, Utrecht, Utrecht, Netherlands, ²ASST-Pini-CTO, Milano, Milan, Italy, ³IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Rome, Italy, ⁴University Hospital Muenster, Muenster, Germany, ⁵IRCCS G. Gaslini, Genova, Genoa, Italy, ⁶University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, ⁷Hospital Sant Joan de Düu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, ⁸UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁹Children's Hospital of Philadelphia, Philadelphia, PA, ¹⁰University of Alabama at Birmingham, Birmingham, AL, ¹¹Bambino Gesu Children's Hospital, Rome, Rome, Italy, ¹²Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ¹³Tel Aviv Medical Center Israel, Binyamina, Tel Aviv, Israel, ¹⁴Karolinska University Hospital, Sollentuna, Sweden, ¹⁵SRCC Childrens Hospital Mumbai, Mumbai, India, ¹⁶Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan, Kobe, Japan, ¹⁷Hacettepe University Medical Faculty, Ankara, Turkey, ¹⁸Necker hospital, Paris Cedex ¹⁵, France, ¹⁹IRCCS Istituto Giannina Gaslini, Genoa, Italy, Genoa, Genoa, Italy, ²⁰Tokyo Medical and Dental University, Bunkyo-ku, Kanazawa, Japan, ²¹Childrens Hospital of Eastern Ontario (CHEO), Ottawa, ON, Canada, ²²Systemic JIA Foundation, Cincinnati, OH, ²³Université Milano Bicocca and Fondazione IRCSS S. Gerardo dei Tintori, Monza, Monza and Brianza, Italy, ²⁴Wilhelmina Children's Hospital / UMC Utrecht, Utrecht, Utrecht, Netherlands, ²⁵Sorbonne Université - APHP, Department of Rheumatology, Hôpital Pitié-Salpêtrière, Inserm UMRS ¹¹³⁶-⁵, PARIS, France, Paris, France, ²⁶Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Milan, Italy
Background/Purpose: The outcome of Still’s disease (SD) has significantly improved due to new therapeutic options [37923864], early biologic initiation [24623686] and treat-to-target strategies [39317417]. Nonetheless,…
Abstract Number: 1876 • ACR Convergence 2025
Targeting the NK cell checkpoint NKG2A promotes lung fibrosis resolution by enhancing immune clearance of senescent myofibroblasts
Wolfgang Merkt¹, Lea Rodon², Franca Sophie Deicher³, Maren Claus⁴, Rachel Lister⁵, Hongwei Han⁵, Yan Zhou⁵, Zhengwang Sun⁵, Arik Horne⁶, Ayla Nadja Stuetz⁷, Michael Kreuter⁸, nicolas kahn⁹, Marc Schneider⁹, Simon Haas¹⁰, Norbert Blank¹¹, Hanns-Martin Lorenz¹², Carsten Watzl⁴, Daniel Hübschmann¹³ and David Lagares⁵, ¹University Hospital Düsseldorf, Düsseldorf, Germany, ²University Hospital Heidelberg, Heidelberg, Hungary, ³Uniklinik Düsseldorf, Düsseldorf, Germany, ⁴IfADo, Leibniz Institute Dortmund, Dortmund, Germany, ⁵Massachusetts General Hospital and Harvard Medical School, Boston, ⁶Charite, Berlin, Berlin, ⁷Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Nordrhein-Westfalen, Germany, ⁸Universitätsmedizin Mainz, Mainz, ⁹Thoraxklinik, Heidelberg University, Heidelberg, Germany, ¹⁰Charite, Berlin, Berlin, Germany, ¹¹University Hospital Heidelberg, Heidelberg, Germany, ¹²Universitétsklinikum Heidelberg, Heidelberg, Germany, ¹³Heidelberg University, Berlin, Germany
Background/Purpose: A key event driving pulmonary fibrosis in interstitial lung disease (ILD) is the accumulation of pathologic senescent myofibroblasts, thought to be promoted by insufficient…
Abstract Number: 1658 • ACR Convergence 2025
Cardiovascular Disease Risk and Risk Calculator Performance in Rheumatoid Arthritis-Associated Interstitial Lung Disease
Omair Khan¹, Halie Frideres², Punyasha Roul³, Grant Cannon⁴, Gary Kunkel⁵, Brian Sauer⁶, Joshua Baker⁷, Ted Mikuls¹, Bryant England¹ and Tate Johnson¹, ¹University of Nebraska Medical Center, Omaha, NE, ²UNMC Department of Rheumatology, Omaha, NE, ³UNMC, Omaha, NE, ⁴University of Utah and Salt Lake City VA, Salt Lake City, UT, ⁵University of Utah and George E Wahlen VAMC, Salt Lake City, UT, ⁶Salt Lake City VA/University of Utah, Salt Lake City, UT, ⁷University of Pennsylvania, Philadelphia, PA
Background/Purpose: Existing cardiovascular disease (CVD) risk stratification strategies underperform in RA. Lung disease is an established CVD risk factor, and RA-interstitial lung disease (RA-ILD) clinically…
Abstract Number: 1343 • ACR Convergence 2025
Six-Minute Walk Distance as a Functional Measure in Rheumatoid Arthritis-associated Interstitial Lung Disease Reflecting Both Lung and Joint Involvement
Sung Hae Chang¹, You-Jung Ha², Sung Won Lee³, Misti Paudel⁴, Gregory McDermott⁵, Qianru Zhang⁶, Min Uk Kim⁷, Jeong Seok Lee⁸, Chan Ho Park⁹, Ji-Won Kim¹⁰, Jang Woo Ha¹¹, SANGWAN CHUNG¹², Eun Ha Kang¹³, Yeon-Ah Lee¹², Yong-Beom Park¹⁴, Jung-Yoon Choe¹⁵, Eun Young Lee¹⁶ and Jeffrey Sparks¹⁷, ¹Soonchunhyang University College of Medicine, Cheonan, MA, South Korea, ²Seoul National University Bundang Hospital, Seongnam, South Korea, ³Soon Chun Hyang University Hospital, Seoul, South Korea, ⁴Brigham and Women's Hospital, Division of Rheumatology, Inflammation, and Immunity, Boston, MA, ⁵Brigham and Women's Hospital, Brookline, MA, ⁶Harvard Medical School, Boston, MA, ⁷SMG-SNU Boramae Medical Center, Seoul, Republic of Korea, ⁸Korea Advanced Institute of Science and Technology, KAIST, DaeJeon, Republic of Korea, ⁹Soonchunhyang University College of Medicine, Cheonan, Republic of Korea, ¹⁰Daegu Catholic University School of Medicine, Nam-gu, Daegu, Republic of Korea, ¹¹Division of Rheumatology, Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine, Seodaemun-gu, Seoul, Republic of Korea, ¹²Kyung Hee University Medical Center, seoul, Republic of Korea, ¹³Seoul National University Bundang Hospital, Seongnam, Republic of Korea, ¹⁴Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea, ¹⁵Division of Rheumatology, Department of Internal Medicine, Daegu Catholic University School of Medicine, Daegu, Republic of Korea, ¹⁶Seoul National University College of Medicine, Seoul, South Korea, ¹⁷Brigham and Women's Hospital, Boston, MA
Background/Purpose: The six-minute walk distance (6MWD) measures functional capacity in cardiopulmonary diseases, predicts mortality in interstitial lung disease (ILD), and is used as an endpoint…
Abstract Number: 1162 • ACR Convergence 2025
Antineutrophil cytoplasmic antibody-associated isolated interstitial lung disease
Javier Narváez¹, Pol Maymó-Paituvi², Vanesa Vicens Zygmunt³, Guadalupe Bermudo⁴, Martí Aguilar-Coll⁵, Montserrat Roig Kim², Laia De Daniel Bisbe², Joan Miquel Nolla² and Maria Molina-Molina⁴, ¹Hospital Universitario de Bellvitge, Barcelona, Spain, ²Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Spain, ³Interstitial Lung Disease Unit. Department of Pneumology. Hospital Universitario de Bellvitge, Barcelona, Spain, ⁴Interstitial Lung Disease Unit. Department of Pneumology. Hospital Universitario de Bellvitge., Barcelona, Spain, ⁵Department of Rheumatology. Hospital Universitario de Bellvitge, Barcelona, Spain
Background/Purpose: There is increasing evidence of the association between interstitial lung disease (ILD) and ANCA positivity without other manifestations of systemic vasculitis. The prognosis of…
Abstract Number: 0782 • ACR Convergence 2025
Expansion of T-bet⁺ Age-Associated B Cells Is Associated with Clinical Complications in Still’s Disease
Krisztian Csomos¹, Boglarka Ujhazi¹, Mariana Correia Marques², Emily Rosenbaum¹ and Michael Ombrello³, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), NIH, Bethesda, MD, ²Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ³National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), North Bethesda, MD
Background/Purpose: Still’s disease is a rare and severe autoinflammatory disorder characterized by daily spiking fevers, arthritis, an evanescent rash, and prominent systemic features, including lymphadenopathy,…
Abstract Number: 0022 • ACR Convergence 2025
Genome-wide association study identifies novel genetic risk factors for rheumatoid arthritis-associated interstitial lung disease
Austin Wheeler¹, Thomas Riley², Riku Takei³, Joshua Baker², Yangyuna Yang¹, Punyasha Roul⁴, Katherine Wysham⁵, Grant Cannon⁶, Gary Kunkel⁷, Gail Kerr⁸, Dana Ascherman⁹, Paul Monach¹⁰, Andreas Reimold¹¹, Jill Poole¹, Ted Mikuls¹, Tony Merriman¹² and Bryant England¹, ¹University of Nebraska Medical Center, Omaha, NE, ²University of Pennsylvania, Philadelphia, PA, ³University of Alabama at Birmingham, Birmingham, AL, ⁴UNMC, Omaha, NE, ⁵VA PUGET SOUND/UNIVERSITY OF WASHINGTON, Seattle, WA, ⁶University of Utah and Salt Lake City VA, Salt Lake City, UT, ⁷University of Utah and George E Wahlen VAMC, Salt Lake City, UT, ⁸Washington DC VAMC/Georgetown and Howard Universities, Washington, DC, ⁹University of Pittsburgh, Pittsburgh, PA, ¹⁰VA Boston Healthcare System, Boston, MA, ¹¹Dallas VA Medical Center, Dallas, TX, ¹²University of Alabama at Birmingham, Homewood, AL
Background/Purpose: Interstitial lung disease (ILD) is clinically present in ~10% of individuals with RA. There is recognized overlap between RA-ILD and idiopathic pulmonary fibrosis (IPF)…
Abstract Number: 2485 • ACR Convergence 2025
Prevalence of progressive pulmonary fibrosis in systemic sclerosis-associated interstitial lung disease
Javier Narváez¹, Irene Carrión-Barberà², Carlos Valera Ribera³, Alfredo Guillen del Castillo⁴, Laura Tio⁵, Laura Triginer⁶, Lidia Montala-Valencia⁷, Carmen Pilar Simeon⁸, Anna Pros², JUAN JOSE ALEGRE SANCHO⁹ and Joan Miquel Nolla¹⁰, ¹Hospital Universitario de Bellvitge, Barcelona, Spain, ²Department of Rheumatology. Hospital del Mar, Barcelona, Spain, ³Hospital Universitario Doctor Peset, València, Spain, ⁴Departmend of Internal Medicine. Hospital Universitario Vall d´Hebron, Barcelona, Spain, ⁵Hospital del Mar Research Institute, Barcelona, Spain, ⁶Vall d´Hebron Institut de Recerca (VHIR), Barcelona, Spain, ⁷Department of Rheumatology. Hospital Universitario de Bellvitge, Barcelona, Spain, ⁸Department of Internal Medicine, Hospital Universitario Vall d’Hebron, Barcelona, Spain, ⁹Department of rheumatology. Hospital Universitario Doctor Peset, Valencia, Spain, ¹⁰Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Spain
Background/Purpose: To estimate the prevalence of the progressive pulmonary fibrosis (PPF) phenotype among patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) and identify risk factors…
Abstract Number: 2134 • ACR Convergence 2025
Krebs von den Lungen-6 (KL-6) as a Potential Diagnostic Biomarker of Lung Disease in Pediatric Systemic Juvenile Idiopathic Arthritis: Preliminary Findings from a Multisite US Cohort
Eileen Rife¹, Lexi Auld², Guihua Zhai³, Esraa Eloseily⁴, Grant Schulert⁵ and Yukiko Kimura⁶, ¹University of Alabama at Birmingham, birmingham, AL, ²Cincinnati Children's Hospital Medical Center, Division of Rheumatology, Cincinnati, OH, ³UAB, Birmingham, ⁴UT Southwestern Children's Medical Center, Dallas, TX, ⁵Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁶Hackensack Meridian School of Medicine, New York, NY
Background/Purpose: Children with Systemic Juvenile Idiopathic Arthritis (SJIA) who develop lung disease (LD) are at significantly increased risk of serious complications and even death. Early…
Abstract Number: 1872 • ACR Convergence 2025
High-throughput Screening Uncovers Distinct Molecular Signatures Linked with HRCT Patterns in SARD-ILD
Philip Stauffer¹, Vyacheslav Palchevskiy², Laura Much³, Phuong Diep⁴, Magdalena Abel⁴, Natasha Moe⁴, Thomas Gaisl⁵, Carolin Steinack⁵, Shahrzad Lari², Elena Pachera⁶, Øyvind Molberg⁷, Oliver Distler⁸, S. Samuel Weigt², John A. Belperio² and Anna-Maria Hoffmann-Vold⁴, ¹University of Zurich Hospital, Schlieren, Switzerland, ²UCLA, Los Angeles, ³Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁴Oslo University Hospital, Oslo, Norway, ⁵USZ, Zürich, Switzerland, ⁶University Hospital Zurich, Zurich, Switzerland, ⁷Department of Rheumatology, Oslo University Hospital, Oslo, Norway, ⁸Department of Rheumatology, University Hospital Zurich, University of Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: High-Resolution Computed Tomography (HRCT) is crucial for diagnosing and managing interstitial lung disease associated with systemic autoimmune rheumatic diseases (SARD-ILD), as it reveals characteristic…
Abstract Number: 1589 • ACR Convergence 2025
Nintedanib in Systemic Sclerosis-Associated Interstitial Lung disease: Real-World Cohort Study on Tolerability and Discontinuation
Punchalee Kaenmuang¹, Nina Goldman², Arthihai Srirangan¹, Fiammetta Danzo¹, Wing-Ho Yip¹, Ali Taki³, Shereen Boreland¹, Benedetta Vitri¹, Carmel JW. Stock¹, Christopher Denton⁴, Athol U. Wells¹, Voon H. Ong⁵, Piersante Sestini⁶ and Elisabetta A. Renzoni¹, ¹Interstitial Lung Disease Unit, Royal Brompton Hospital, Guy’s and St Thomas’ National Health Service Foundation Trust, London, England, United Kingdom, ²Division of Medicine, Centre for Rheumatology, University College London, London, England, United Kingdom, ³Royal Free Hospital, London, England, United Kingdom, ⁴University College London, UK, London, United Kingdom, ⁵Division of Medicine, Centre for Rheumatology, University College London, London, United Kingdom, ⁶Department of Medical and Surgical Sciences and Neurosciences, University of Siena, Siena, Toscana, Italy
Background/Purpose: Nintedanib slows the progression of systemic sclerosis-associated interstitial lung disease (SSc-ILD). However, potential high discontinuation rates due to adverse side-effects raise concerns about its…
Abstract Number: 1335 • ACR Convergence 2025
Chest x-Ray and pulmonary function tests are not sensitive for screening intertitial lung disease in reumatoid arthritis
Jesús Loarce¹, Alonso Sebastián González Montes², SONIA JIMENEZ BARRIOS³, Mauro Ferre-Sanfrancisco⁴, Verónica García García⁵ and Javier Bachiller⁶, ¹Ramón y Cajal University Hospital, Madrid, Spain, ²Ramón y Cajal university hospital, Madrid, Madrid, Spain, ³Hospital Universitario Ramón y Cajal, Madrid, Spain, ⁴Department of Rheumatology, Hospital Universitario Ramon y Cajal, Madrid, Spain, Madrid, Madrid, Spain, ⁵Ramón y Cajal University Hospital, MADRID, Spain, ⁶Hospital Ramon y Cajal, Madrid, Madrid, Spain
Background/Purpose: Interstitial lung disease (ILD) is a frequent and severe complication of rheumatoid arthritis (RA). Although early diagnosis and screening of ILD in RA patients…
Abstract Number: 1161 • ACR Convergence 2025
Comparative efficacy, safety and tolerability of antifibrotic therapies across systemic autoimmune diseases
Javier Narváez¹, Orfa Barrios², Pol Maymó-Paituvi³, JUAN JOSE ALEGRE SANCHO⁴, Ivan Castellví⁵, Vanesa Vicens Zygmunt⁶, Guadalupe Bermudo⁷, Laia De Daniel Bisbe³, Martí Aguilar-Coll⁸, Montserrat Roig Kim³, Joan Miquel Nolla³ and Maria Molina-Molina⁷, ¹Hospital Universitario de Bellvitge, Barcelona, Spain, ²Department of Rheumatology. Hospital Nacional Arzobispo Loayza, Lima, Peru, ³Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Spain, ⁴Department of rheumatology. Hospital Universitario Doctor Peset, Valencia, Spain, ⁵Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, barcelona, Spain, ⁶Interstitial Lung Disease Unit. Department of Pneumology. Hospital Universitario de Bellvitge, Barcelona, Spain, ⁷Interstitial Lung Disease Unit. Department of Pneumology. Hospital Universitario de Bellvitge., Barcelona, Spain, ⁸Department of Rheumatology. Hospital Universitario de Bellvitge, Barcelona, Spain
Background/Purpose: To evaluate the efficacy, safety, and tolerability of the antifibrotic agents nintedanib and pirfenidone in the treatment of interstitial lung disease (ILD) associated with…
Abstract Number: 0773 • ACR Convergence 2025
Clinical Significance of Anti-MDA5 Epitope Antibodies as Prognostic Indicators for Interstitial Lung Disease With or Without Dermatomyositis
Tsuneo Sasai¹, Ran Nakashima², Motohiro Nonaka³, Norimichi Nomura⁴, Atsubumi Ogawa¹, Yasuhiro Nohda¹, Mirei Shirakashi², Ryosuke Hiwa¹, Hideaki Tsuji¹, Hajime Yoshifuji², Shogo Matsuda⁵, Masao Katsushima⁶, Michinori Ishitoku⁷, Yusuke Yoshida⁷, Yasuyuki Todoroki⁸, Satoshi Kubo⁹, Tomohiro Handa¹⁰, Hiromi Tomioka¹¹, Ryo Tachikawa¹², Keisuke Tomii¹², Kiminobu Tanizawa¹³, Toru Arai¹⁴, Takuya Kotani¹⁵, Motomu Hashimoto¹⁶, Shintaro Hirata⁷, Yoshiya Tanaka¹⁷, Tsuneyo Mimori¹⁸ and Akio Morinobu¹⁹, ¹Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan, ²Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Kyoto, Japan, ³Department of Human Health Sciences, Graduate School of Medicine, Kyoto University, Kyoto, Japan, ⁴Department of Cell Biology, Graduate School of Medicine, Kyoto University, Kyoto, Japan, ⁵Department of Internal Medicine IV, Division of Rheumatology, Osaka Medical and Pharmaceutical University, Osaka, Japan, ⁶Department of Clinical Immunology, Osaka Metropolitan Graduate School of Medicine, Osaka, Japan, ⁷Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital, Hiroshima, Japan, ⁸Department of Molecular Targeted Therapies, University of Occupational and Environmental Health, Kitakyushu, Japan, ⁹Department of Molecular Targeted Therapies (DMTT), University of Occupational and Environmental Health, Japan, Kitakyushu, Japan, ¹⁰Department of Advanced Medicine for Respiratory Failure and Graduate School of Medicine, Kyoto University, Kyoto, Japan, ¹¹Department of Respiratory Medicine, Kobe City Medical Center West Hospital, Kobe, Japan, ¹²Department of Respiratory Medicine, Kobe City Medical Center General Hospital, Kobe, Japan, ¹³Respiratory Medicine, National Hospital Organization Kyoto Medical Center, Kyoto, Japan, ¹⁴Clinical Research Center, NHO Kinki-Chuo Chest Medical Center, Sakai, Japan, ¹⁵Division of Rheumatology, Department of Internal Medicine IV, Osaka Medical and Pharmaceutical University, Osaka, Japan, ¹⁶Osaka Metropolitan University, Osaka, Japan, ¹⁷University of Occupational and Environmental Health, Japan, Kitakyushu, Japan, ¹⁸Takeda Clinic for Rheumatic Diseases, Kyoto, Japan, ¹⁹Kyoto University, Kyoto, Kyoto, Japan
Background/Purpose: Melanoma differentiation-associated gene 5 (MDA5) is a cytoplasmic RNA sensor and activates the innate immune response. Autoantibodies against MDA5 are associated with rapidly progressive…
Abstract Number: 0340 • ACR Convergence 2024
Progressive Pulmonary Fibrosis Predicts Mortality in Myositis-Associated Interstitial Lung Disease
Yu-Wan Liao¹, Ming-Cheng Liu¹, Pin-Kuei Fu¹ and Wen-Nan Huang², and Integrated Care Center of Interstitial Lung Disease, ¹Taichung Veterans General Hospital, Taichung, Taiwan (Republic of China), ²Taichung Veterans General Hospital, Taichung, Taiwan, Taichung, Taiwan (Republic of China)
Background/Purpose: Myositis-associated interstitial lung disease (MA-ILD) is a complex condition with a variable prognosis and diverse clinical manifestations. The 2022 ATS/ERS/JRS/ALAT criteria for PPF provide…

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