ArticleBMC pulmonary medicine2024
Design and baseline characteristics of the ILD-PRO registry in patients with progressive pulmonary fibrosis.
Article in BMC pulmonary medicine, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT01915511 (Idiopathic Pulmonary Fibrosis Prospective Outcomes), which is not on this map. Cited by 5 papers.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Idiopathic Pulmonary Fibrosis Prospective Outcomes (IPF-PRO) and Interstitial Lung Disease Prospective Outcomes (IPF-PRO/ILD-PRO) Registry
Who cites it
5 citing papers in PubMed.
- Nerandomilast in progressive pulmonary fibrosis: data from the whole follow-up period of the FIBRONEER-ILD trial.The European respiratory journal · 2026Trial
- Observational
- Progressive pulmonary fibrosis: the importance of identification and intervention.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- Quality of life in patients with hypersensitivity pneumonitis and with idiopathic pulmonary fibrosis in real-life clinical practice.Journal of thoracic disease · 2025Article
- Extent of lung fibrosis is of greater prognostic importance than HRCT pattern in patients with progressive pulmonary fibrosis: data from the ILD-PRO registry.Respiratory research · 2025Article
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Authors and funding
11 authors.
Funding
Abstract
backgroundTo assess the characteristics of patients enrolled in the ILD-PRO Registry.
methodsThe ILD-PRO Registry is a multicentre US registry of patients with progressive pulmonary fibrosis. This registry is enrolling patients with an interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis who have reticular abnormality and traction bronchiectasis on HRCT, and who meet criteria for ILD progression within the prior 24 months. Patient characteristics were analysed based on the number of patients with available data.
resultsOf the first 491 patients enrolled, the majority were white (75.4%) and female (60.6%); 47.4% had a history of smoking. Reported ILDs were autoimmune disease-associated ILDs (47.2%), hypersensitivity pneumonitis (17.5%), idiopathic non-specific interstitial pneumonia (9.1%), interstitial pneumonia with autoimmune features (8.9%), unclassifiable ILD (7.6%), other ILDs (9.7%). At enrolment, median (Q1, Q3) FVC % predicted was 62.2 (49.4, 72.4) and DLco % predicted was 39.2 (30.2, 49.2). Median (Q1, Q3) total score on the St. George's Respiratory Questionnaire was 50.8 (35.9, 64.7). The most common comorbidities were gastroesophageal reflux disease (61.1%) and sleep apnoea (29.6%). Overall, 64.5% of patients were receiving immunosuppressive or cytotoxic therapy, 61.1% proton-pump inhibitors, 53.2% oral steroids, 19.8% nintedanib and 3.6% pirfenidone.
conclusionsPatients enrolled into the ILD-PRO Registry have a variety of ILD diagnoses, marked impairment in lung function and health-related quality of life, and high medication use. Longitudinal data from this registry will further our knowledge of the course of progressive pulmonary fibrosis.
trial registrationClinicalTrials.gov, NCT01915511; registered August 5, 2013.
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