Validation of proposed criteria for progressive pulmonary fibrosis

التفاصيل البيبلوغرافية
العنوان: Validation of proposed criteria for progressive pulmonary fibrosis
المؤلفون: Janelle Vu Pugashetti, Ayodeji Adegunsoye, Zhe Wu, Cathryn T. Lee, Anand Srikrishnan, Sahand Ghodrati, Vivian Vo, Elisabetta A. Renzoni, Athol U. Wells, Christine Kim Garcia, Felix Chua, Chad A. Newton, Philip L. Molyneaux, Justin M. Oldham
بيانات النشر: American Thoracic Society, 2022.
سنة النشر: 2022
مصطلحات موضوعية: Pulmonary and Respiratory Medicine, Critical Care and Intensive Care Medicine
الوصف: RATIONALE: Criteria for progressive pulmonary fibrosis (PPF) have been proposed, but their prognostic value beyond categorical decline in forced vital capacity (FVC) remains unclear. OBJECTIVE: To determine whether proposed PPF criteria predict transplant-free survival (TFS) in patients with non-idiopathic pulmonary fibrosis (IPF) forms of interstitial lung disease (ILD). METHODS: A retrospective, multi-center cohort analysis was performed. Patients diagnosed with fibrotic connective tissue disease associated ILD, fibrotic hypersensitivity pneumonitis and non-IPF idiopathic interstitial pneumonia from three US centers and one UK center comprised test and validation cohorts, respectively. Cox proportional hazards regression was used to test the association between five-year TFS and 10% FVC decline, followed by thirteen additional PPF criteria satisfied in the absence of >=10% FVC decline. MAIN RESULTS: One thousand three hundred forty-one patients met inclusion criteria. A >=10% relative FVC decline was the strongest predictor of reduced TFS and showed consistent TFS association across cohorts, ILD subtypes and treatment groups, resulting in a phenotype that closely resembled IPF. Ten additional PPF criteria satisfied in the absence of >=10% relative FVC decline were also associated with reduced TFS in the US test cohort, with six maintaining TFS association in the UK validation cohort. Validated PPF criteria requiring a combination of physiologic, radiologic, and symptomatic worsening performed similarly to their stand-alone components but captured a smaller number of patients. CONCLUSIONS: An FVC decline of >=10% and six additional PPF criteria satisfied in the absence of such decline identify non-IPF ILD patients at increased risk of death or lung transplant.
اللغة: English
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a6d3a566bb0e7e9000a30d6ca2ee4125
http://hdl.handle.net/10044/1/98961
Rights: OPEN
رقم الانضمام: edsair.doi.dedup.....a6d3a566bb0e7e9000a30d6ca2ee4125
قاعدة البيانات: OpenAIRE