{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T02:09:52Z","timestamp":1791684592885,"version":"4.3.4"},"reference-count":42,"publisher":"MDPI AG","issue":"20","license":[{"start":{"date-parts":[[2024,10,12]],"date-time":"2024-10-12T00:00:00Z","timestamp":1728691200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Interstitial lung disease (ILD) is a common extra-muscular manifestation of idiopathic inflammatory myopathies (IIMs), often associated with a poorer prognosis and increased mortality risk. Methods: This retrospective study aimed to characterize lung involvement and treatment response in an IIM cohort at a Portuguese tertiary center, followed between June 2016 and March 2024. We analyzed data from high-resolution computed tomography (HRCT) scans and pulmonary function tests (PFTs) to assess associations with autoantibody profiles and treatment regimens. Results: A total of 198 patients were included, with 69 (34.8%) exhibiting ILD. Antisynthetase syndrome (ASyS) and dermatomyositis were the most common diagnoses among IIM-ILD patients, with ASyS being significantly more frequent in this group than in non-ILD patients (p &lt; 0.001). Anti-Jo1 and anti-MDA-5 antibodies were more frequent in ILD patients (p &lt; 0.001 and p = 0.021), while anti-Mi2 antibodies were less common (p = 0.002). Non-specific interstitial pneumonia (NSIP) was the most common radiological pattern (69.5%). IIM-ILD patients presented with significantly lower forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DLCO) compared to non-ILD patients (p &lt; 0.001 for all values). Longitudinal analysis showed improved DLCO (p = 0.022) and stable or improved FVC (p = 0.097), especially with intravenous immunoglobulin (IVIg) and azathioprine (AZA). Combination therapies including IVIg with mycophenolate mofetil (MMF) or rituximab (RTX) also improved DLCO and FVC. Most ILD patients (89.6%) had stable HRCT patterns over time. Conclusions: Our findings highlight the potential for stabilizing or even improving lung function in IIM-ILD with appropriate immunosuppressive therapy, particularly with regimens incorporating IVIg and AZA, and combination therapies.<\/jats:p>","DOI":"10.3390\/jcm13206085","type":"journal-article","created":{"date-parts":[[2024,10,14]],"date-time":"2024-10-14T12:44:31Z","timestamp":1728909871000},"page":"6085","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Myositis-Associated Interstitial Lung Disease: The Experience of a Tertiary Center"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-4541-2386","authenticated-orcid":false,"given":"Bianca Paulo","family":"Correia","sequence":"first","affiliation":[{"name":"Rheumatology Department, Unidade Local de Sa\u00fade Santa Maria (ULSSM), 1649-028 Lisbon, Portugal"},{"name":"Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Acad\u00e9mico de Medicina de Lisboa, 1649-028 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raquel","family":"Campanilho-Marques","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Unidade Local de Sa\u00fade Santa Maria (ULSSM), 1649-028 Lisbon, Portugal"},{"name":"Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Acad\u00e9mico de Medicina de Lisboa, 1649-028 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2186-2833","authenticated-orcid":false,"given":"Eduardo","family":"Dourado","sequence":"additional","affiliation":[{"name":"Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Acad\u00e9mico de Medicina de Lisboa, 1649-028 Lisbon, Portugal"},{"name":"Rheumatology Department, Unidade Local de Sa\u00fade Regi\u00e3o de Aveiro (ULSRA), 3810-501 Aveiro, Portugal"},{"name":"Aveiro Rheumatology Research Centre, Centro Acad\u00e9mico Cl\u00ednico Egas Moniz Health Alliance, 3810-501 Aveiro, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariana","family":"Silva","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Unidade Local de Sa\u00fade Santa Maria (ULSSM), 1649-028 Lisbon, Portugal"},{"name":"Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Acad\u00e9mico de Medicina de Lisboa, 1649-028 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Augusto","family":"Silva","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Unidade Local de Sa\u00fade Santa Maria (ULSSM), 1649-028 Lisbon, Portugal"},{"name":"Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Acad\u00e9mico de Medicina de Lisboa, 1649-028 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Filipa","family":"Costa","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Unidade Local de Sa\u00fade Santa Maria (ULSSM), 1649-028 Lisbon, Portugal"},{"name":"Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Acad\u00e9mico de Medicina de Lisboa, 1649-028 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matilde","family":"Bandeira","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Unidade Local de Sa\u00fade Santa Maria (ULSSM), 1649-028 Lisbon, Portugal"},{"name":"Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Acad\u00e9mico de Medicina de Lisboa, 1649-028 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Teresa","family":"Melo","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Unidade Local de Sa\u00fade S\u00e3o Jos\u00e9 (ULSSJ), 1169-050 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6807-559X","authenticated-orcid":false,"given":"Sofia C.","family":"Barreira","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Unidade Local de Sa\u00fade Santa Maria (ULSSM), 1649-028 Lisbon, Portugal"},{"name":"Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Acad\u00e9mico de Medicina de Lisboa, 1649-028 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo\u00e3o E.","family":"Fonseca","sequence":"additional","affiliation":[{"name":"Rheumatology Department, Unidade Local de Sa\u00fade Santa Maria (ULSSM), 1649-028 Lisbon, Portugal"},{"name":"Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Acad\u00e9mico de Medicina de Lisboa, 1649-028 Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,10,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1038\/nrrheum.2018.41","article-title":"Classification of Myositis","volume":"14","author":"Lundberg","year":"2018","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1117071","DOI":"10.3389\/fmed.2023.1117071","article-title":"Myositis Interstitial Lung Disease and Autoantibodies","volume":"10","author":"Chaudhry","year":"2023","journal-title":"Front. Med."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1590\/S1806-37132011000100015","article-title":"Myositis-Related Interstitial Lung Disease and Antisynthetase Syndrome","volume":"37","author":"Solomon","year":"2011","journal-title":"J. Bras. Pneumol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1007\/s40674-018-0110-6","article-title":"Myositis-Related Interstitial Lung Diseases: Diagnostic Features, Treatment, and Complications","volume":"5","author":"Shappley","year":"2019","journal-title":"Curr. Treat. Opt. Rheumatol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1038\/s41584-023-01054-9","article-title":"Anti-MDA5 Antibody-Positive Dermatomyositis: Pathogenesis and Clinical Progress","volume":"20","author":"Lu","year":"2024","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Kannappan, R., Kumar, R., Cichelli, K., and Brent, L.H. (2024). A Review of Myositis-Associated Interstitial Lung Disease. J. Clin. Med., 13.","DOI":"10.3390\/jcm13144055"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1177\/2397198320902667","article-title":"Antisynthetase Syndrome: A Distinct Disease Spectrum","volume":"5","author":"Huang","year":"2020","journal-title":"J. Scleroderma Relat. Disord."},{"key":"ref_8","first-page":"190","article-title":"Clinical Characterisation of a Multicentre Nationwide Cohort of Patients with Antisynthetase Syndrome","volume":"1","author":"Martins","year":"2022","journal-title":"ARP Rheumatol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1068402","DOI":"10.3389\/fmed.2023.1068402","article-title":"Interstitial Lung Disease Associated with Inflammatory Myositis: Autoantibodies, Clinical Phenotypes, and Progressive Fibrosis","volume":"10","author":"Ceribelli","year":"2023","journal-title":"Front. Med."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1476","DOI":"10.1016\/j.chest.2023.01.031","article-title":"Diagnosis and Management of Myositis-Associated Lung Disease","volume":"163","author":"Hallowell","year":"2023","journal-title":"Chest"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"3480","DOI":"10.1007\/s00330-021-08411-w","article-title":"Idiopathic Inflammatory Myopathies: CT Characteristics of Interstitial Lung Disease and Their Association(s) with Myositis-Specific Autoantibodies","volume":"32","author":"Laporte","year":"2022","journal-title":"Eur. Radiol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/j.semarthrit.2020.11.009","article-title":"Prevalence of Interstitial Lung Disease in Polymyositis and Dermatomyositis: A Meta-Analysis from 2000 to 2020","volume":"51","author":"Sun","year":"2021","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1016\/j.ccm.2019.05.004","article-title":"Interstitial Lung Disease in Polymyositis and Dermatomyositis","volume":"40","author":"Long","year":"2019","journal-title":"Clin. Chest Med."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1016\/j.rdc.2014.12.006","article-title":"Pulmonary Complications of Inflammatory Myopathy","volume":"41","author":"Miller","year":"2015","journal-title":"Rheum. Dis. Clin. N. Am."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/j.rmed.2016.12.014","article-title":"Interstitial Lung Disease and Myositis-Specific and Associated Autoantibodies: Clinical Manifestations, Survival and the Performance of the New ATS\/ERS Criteria for Interstitial Pneumonia with Autoimmune Features (IPAF)","volume":"123","author":"Rivero","year":"2017","journal-title":"Respir. Med."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1118","DOI":"10.1016\/j.chest.2016.04.007","article-title":"Management of Myositis-Related Interstitial Lung Disease","volume":"150","author":"Morisset","year":"2016","journal-title":"Chest"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1186\/s13075-017-1506-7","article-title":"The Predictive Prognostic Factors for Polymyositis\/Dermatomyositis-Associated Interstitial Lung Disease","volume":"20","author":"Sugiyama","year":"2018","journal-title":"Arthritis Res. Ther."},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Egashira, R. (2021). High-Resolution CT Findings of Myositis-Related Interstitial Lung Disease. Medicina, 57.","DOI":"10.3390\/medicina57070692"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"326","DOI":"10.3389\/fmed.2019.00326","article-title":"Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment","volume":"6","author":"Hervier","year":"2020","journal-title":"Front. Med."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1380","DOI":"10.1016\/j.rmed.2011.05.006","article-title":"HRCT Features of Interstitial Lung Disease in Dermatomyositis with Anti-CADM-140 Antibody","volume":"105","author":"Tanizawa","year":"2011","journal-title":"Respir. Med."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"373","DOI":"10.55563\/clinexprheumatol\/brvl1v","article-title":"Myositis-Associated Interstitial Lung Disease: A Comprehensive Approach to Diagnosis and Management","volume":"40","author":"Hallowell","year":"2022","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1127","DOI":"10.2147\/JMDH.S404017","article-title":"Myositis Multidisciplinary Clinic in a Tertiary Referral Center","volume":"16","author":"Melo","year":"2023","journal-title":"J. Multidiscip. Healthc."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"948","DOI":"10.1183\/09031936.05.00035205","article-title":"Interpretative Strategies for Lung Function Tests","volume":"26","author":"Pellegrino","year":"2005","journal-title":"Eur. Respir. J."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1183\/16000617.00002015","article-title":"Idiopathic Inflammatory Myopathies and the Lung","volume":"24","author":"Lega","year":"2015","journal-title":"Eur. Respir. Rev."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"108","DOI":"10.2174\/157339710791330740","article-title":"Interstitial Lung Disease in Idiopathic Inflammatory Myopathy","volume":"6","author":"Saketkoo","year":"2010","journal-title":"Curr. Rheumatol. Rev."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"e003121","DOI":"10.1136\/rmdopen-2023-003121","article-title":"Interstitial Lung Disease with and without Progressive Fibrosing Phenotype in Patients with Idiopathic Inflammatory Myopathies: Data from a Large Multicentric Cohort","volume":"9","author":"Zanatta","year":"2023","journal-title":"RMD Open"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"937561","DOI":"10.3389\/fmed.2022.937561","article-title":"Treating Autoimmune-Related Interstitial Lung Disease with B Cell Depletion","volume":"9","author":"Liossis","year":"2022","journal-title":"Front. Med."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1378\/chest.11-0492","article-title":"IV Immunoglobulin Might Be Considered as a First-Line Treatment of Severe Interstitial Lung Disease Associated with Polymyositis","volume":"140","author":"Diot","year":"2011","journal-title":"Chest"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1378\/chest.10-0360","article-title":"Polymyositis Associated with Severe Interstitial Lung Disease: Remission After Three Doses of IV Immunoglobulin","volume":"139","author":"Bakewell","year":"2011","journal-title":"Chest"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1002\/rai2.12112","article-title":"Combination Therapy with Methylprednisolone, Rituximab, and Tofacitinib in Antimelanoma Differentiation-Associated 5 Gene Dermatomyositis with Rapidly Progressive Interstitial Lung Disease","volume":"4","author":"Manghani","year":"2024","journal-title":"Rheumatol. Autoimmun."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"e14999","DOI":"10.1111\/1756-185X.14999","article-title":"Aggressive Multi-Combination Therapy for Anti-MDA5 Antibody-Positive Dermatomyositis-Rapidly Progressive Interstitial Lung Disease","volume":"27","author":"Hata","year":"2024","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Yen, T.-H., Tseng, C.-W., Wang, K.-L., and Fu, P.-K. (2021). Combination Therapy with Rituximab, Tofacitinib and Pirfenidone in a Patient with Rapid Progressive Interstitial Lung Disease (RP-ILD) Due to MDA5 Antibody-Associated Dermatomyositis: A Case Report. Medicina, 57.","DOI":"10.3390\/medicina57121358"},{"key":"ref_33","doi-asserted-by":"crossref","unstructured":"Treppo, E., Infantino, M., Benucci, M., Ravagnani, V., Palterer, B., Fabris, M., Tomietto, P., Manfredi, M., Giudizi, M.G., and Ligobbi, F. (2020). Efficacy and Safety of High-Dose Immunoglobulin-Based Regimen in Statin-Associated Autoimmune Myopathy: A Multi-Center and Multi-Disciplinary Retrospective Study. J. Clin. Med., 9.","DOI":"10.3390\/jcm9113454"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"2020-8-8","DOI":"10.7573\/dic.2020-8-8","article-title":"Efficacy and Safety of Mycophenolate Mofetil in the Treatment of Rheumatic Disease-Related Interstitial Lung Disease: A Narrative Review","volume":"10","author":"Cassone","year":"2021","journal-title":"Drugs Context"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1186\/s13075-024-03310-z","article-title":"Effectiveness and Safety of Mycophenolate Mofetil and Rituximab Combination Therapy for Immune Idiopathic Myopathies","volume":"26","author":"Campochiaro","year":"2024","journal-title":"Arthritis Res. Ther."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"2202071","DOI":"10.1183\/13993003.02071-2022","article-title":"Rituximab and Mycophenolate Mofetil Combination in Patients with Interstitial Lung Disease (EVER-ILD): A Double-Blind, Randomised, Placebo-Controlled Trial","volume":"61","author":"Mankikian","year":"2023","journal-title":"Eur. Respir. J."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"2020-8-7","DOI":"10.7573\/dic.2020-8-7","article-title":"Efficacy and Safety of Rituximab in the Treatment of Connective Tissue Disease-Related Interstitial Lung Disease","volume":"10","author":"Vacchi","year":"2021","journal-title":"Drugs Context"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"110342","DOI":"10.1016\/j.intimp.2023.110342","article-title":"Infectious Risk When Prescribing Rituximab in Patients with Hypogammaglobulinemia Acquired in the Setting of Autoimmune Diseases","volume":"120","author":"Boumaza","year":"2023","journal-title":"Int. Immunopharmacol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"671503","DOI":"10.3389\/fimmu.2021.671503","article-title":"Rituximab Associated Hypogammaglobulinemia in Autoimmune Disease","volume":"12","author":"Tieu","year":"2021","journal-title":"Front. Immunol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"2101499","DOI":"10.1183\/13993003.01499-2021","article-title":"ERS\/ATS Technical Standard on Interpretive Strategies for Routine Lung Function Tests","volume":"60","author":"Stanojevic","year":"2022","journal-title":"Eur. Respir. J."},{"key":"ref_41","doi-asserted-by":"crossref","unstructured":"American Thoracic Society (1991). Lung Function Testing: Selection of Reference Values and Interpretative Strategies. Am. Rev. Respir. Dis., 144, 1202\u20131218.","DOI":"10.1164\/ajrccm\/144.5.1202"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1183\/09031936.03.00026703","article-title":"Interstitial Lung Disease in Amyopathic Dermatomyositis, Dermatomyositis and Polymyositis","volume":"22","author":"Cottin","year":"2003","journal-title":"Eur. Respir. J."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/20\/6085\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T16:12:14Z","timestamp":1760112734000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/20\/6085"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,12]]},"references-count":42,"journal-issue":{"issue":"20","published-online":{"date-parts":[[2024,10]]}},"alternative-id":["jcm13206085"],"URL":"https:\/\/doi.org\/10.3390\/jcm13206085","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,10,12]]}}}