ArticleRespiratory research2023
Functional respiratory impairment and related factors in patients with interstitial pneumonia with autoimmune features (IPAF): Multicenter study from NEREA registry.
Article in Respiratory research, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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10 citing papers in PubMed, 14 citations in OpenAlex.
- Prognostic impact of autoimmune features in interstitial lung disease: a single-center retrospective real-world cohort study.Clinical rheumatology · 2026Article
- Quantitative CT analysis in interstitial pneumonia with autoimmune features: diagnostic and prognostic insights from a retrospective cohort study.Insights into imaging · 2026Article
- Uncovering mirnomes of patients with interstitial lung diseases and an underlying autoimmune process.Respiratory research · 2025Article
- Lung transplantation outcomes of patients with interstitial pneumonia with autoimmune features: a single-centre retrospective cohort study.Rheumatology (Oxford, England) · 2025Article
- Pulmonary Complications in Connective Tissue Disease-Associated Interstitial Lung Disease.The clinical respiratory journal · 2025Review
- Article
- 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 and Prognostic Significance of p-ANCA Positivity in Idiopathic Pulmonary Fibrosis: A Retrospective Observational Study.Diagnostics (Basel, Switzerland) · 2023Article
- High Ferritin Predicts Mortality in Patients With Interstitial Pneumonia and an Underlying Autoimmune Disease.Open respiratory archivesArticle
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14 authors at 10 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe objective of the present study is to describe the characteristics of interstitial pneumonia with autoimmune features (IPAF) patients, to assess the incidence rate of functional respiratory impairment over time and to evaluate the influence of therapeutic alternatives on the prognosis of these patients.
methodsA longitudinal observational multicenter study was performed (NEREA registry). It was carried out by a multidisciplinary team in seven Hospitals of Madrid. Patients were included from IPAF diagnosis. MAIN OUTCOME: poor prognosis as functional respiratory impairment (relative decline in FVC % defined as ≥ 5% every 6 months). Covariates: therapy, sociodemographic, clinical, radiological patterns, laboratory and functional tests. STATISTICS: Survival techniques were used to estimate IR per 100 patients-semester with their 95% confidence interval [CI]. The influence of covariates in prognosis were analyzed through cox multivariate regression models (hazard ratio (HR) and [CI]).
results79 IPAF were included, with a mean and a maximum follow-up of 3.17 and 12 years respectively. Along the study, 77.2% received treatment (52 glucocorticoids, 25 mycophenolate, 21 azathioprine, 15 rituximab and 11 antifibrotics). IR was 23.9 [19.9-28.8], and 50% of IPAF developed functional respiratory impairment after 16 months from its diagnosis. Multivariate analysis: usual interstitial pneumonia (UIP) had poorer prognosis compared to non-specific interstitial pneumonia (NSIP) (p = 0.001). In NSIP, positive ANA, increased the risk of poor prognosis. In UIP, glucocorticoids (HR: 0.53 [0.34-0.83]), age (HR: 1.04 [1.01-1.07]), and Ro-antibodies (HR: 0.36 [0.19-0.65]) influenced the prognosis.
conclusionsIPAF have functional impairment during the first years of disease. Factors predicting deterioration differ between radiographic patterns. Our real-life study suggests the potential benefit of particular therapies in IPAF.
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