ArticleThe European respiratory journal2020
Myositis-specific antibodies identify a distinct interstitial pneumonia with autoimmune features phenotype.
Article in The European respiratory journal, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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The trial behind it
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Who cites it
25 citing papers in PubMed, 54 citations in OpenAlex.
- Idiopathic inflammatory myopathy-related interstitial lung disease patients present to pulmonology: a tertiary center experience.Annals of the American Thoracic Society · 2026Article
- Defining interstitial lung disease related to idiopathic inflammatory myopathies: a systematic review protocol of the Myositis Clinical Trial Consortium (MCTC).Systematic reviews · 2026Article
- Signal or noise? Apply myositis autoantibody line-blot immunoassays in real-world settings: implications for diagnostic accuracy.Clinical rheumatology · 2026Observational
- Article
- Lung transplantation outcomes of patients with interstitial pneumonia with autoimmune features: a single-centre retrospective cohort study.Rheumatology (Oxford, England) · 2025Article
- Serum KL-6 and SP-D: Markers of Lung Function in Autoimmune-Related Interstitial Lung Diseases.International journal of molecular sciences · 2025Article
- Recognition of Idiopathic Inflammatory Myopathies Underlying Interstitial Lung Diseases.Diagnostics (Basel, Switzerland) · 2025Review
- Idiopathic Inflammatory Myopathies-Associated Interstitial Lung Disease in Adults.Tuberculosis and respiratory diseases · 2025Article
- Efficacy and safety of tacrolimus in the treatment of interstitial pneumonia with autoimmune features: a retrospective study.Frontiers in pharmacology · 2025Article
- Prognostic value of myositis-specific antibodies in patients with idiopathic interstitial pneumonia.BMC pulmonary medicine · 2024Article
- CXCL10 predicts autoimmune features and a favorable clinical course in patients with IIP: post hoc analysis of a prospective and multicenter cohort study.Respiratory research · 2024Article
- Interstitial Pneumonia with Autoimmune Features: Aiming to Define, Refine, and Treat.Revista colombiana de reumatologia · 2024Article
- The Pattern and Progression of "Usual" Interstitial Pneumonia with Autoimmune Features: Comparison with Patients with Classic Interstitial Pneumonia with Autoimmune Features and Idiopathic Pulmonary Fibrosis.Journal of clinical medicine · 2024Article
- Clinical phenotyping in patients with anti-synthetase antibodies using cluster analysis.Rheumatology advances in practice · 2024Article
- Treatable traits: a comprehensive precision medicine approach in interstitial lung disease.The European respiratory journal · 2023Article
- Recurrent Exacerbations and Evolution into Polymyositis in a Patient with Interstitial Pneumonia with Autoimmune Features: A Case Report and Literature Review.Medicina (Kaunas, Lithuania) · 2023Review
- Validation of Proposed Criteria for Progressive Pulmonary Fibrosis.American journal of respiratory and critical care medicine · 2023Article
- Fine-tuning characterization of patients with interstitial pneumonia and an underlying autoimmune disease in real-world practice: We get closer with Nailfold videocapillaroscopy.Frontiers in medicine · 2023Article
- Interstitial Pneumonia with Autoimmune Features: What the Rheumatologist Needs to Know.Current rheumatology reports · 2022Review
- Myositis-associated interstitial lung disease: a comprehensive approach to diagnosis and management.Clinical and experimental rheumatology · 2022Review
Corrections and comments
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Authors and funding
16 authors at 5 institutions in 1 country.
Funding
Abstract
Interstitial pneumonia with autoimmune features (IPAF) characterises individuals with interstitial lung disease (ILD) and features of connective tissue disease (CTD) who fail to satisfy CTD criteria. Inclusion of myositis-specific antibodies (MSAs) in the IPAF criteria has generated controversy, as these patients also meet proposed criteria for an antisynthetase syndrome. Whether MSAs and myositis-associated antibodies (MAA) identify phenotypically distinct IPAF subgroups remains unclear.A multicentre, retrospective investigation was conducted to assess clinical features and outcomes in patients meeting IPAF criteria stratified by the presence of MSAs and MAAs. IPAF subgroups were compared to cohorts of patients with idiopathic inflammatory myopathy-ILD (IIM-ILD), idiopathic pulmonary fibrosis and non-IIM CTD-ILDs. The primary end-point assessed was 3-year transplant-free survival.269 patients met IPAF criteria, including 35 (13%) with MSAs and 65 (24.2%) with MAAs. Survival was highest among patients with IPAF-MSA and closely approximated those with IIM-ILD. Survival did not differ between IPAF-MAA and IPAF without MSA/MAA cohorts. Usual interstitial pneumonia (UIP) morphology was associated with differential outcome risk, with IPAF patients with non-UIP morphology approximating survival observed in non-IIM CTD-ILDs. MSAs, but not MAAs identified a unique IPAF phenotype characterised by clinical features and outcomes similar to IIM-ILD. UIP morphology was a strong predictor of outcome in others meeting IPAF criteria.Because IPAF is a research classification without clear treatment approach, these findings suggest that MSAs should be removed from the IPAF criteria and such patients should be managed as an IIM-ILD.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.