Patients with idiopathic pulmonary fibrosis have poor clinical outcomes with COVID-19 disease: a propensity matched multicentre research network analysis

IntroductionOutcomes of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in patients with pre-existing idiopathic pulmonary fibrosis (IPF) remain understudied, and it is unknown if IPF is an independent predictor of worse disease course. Herein, we report the clinical outcomes in a large cohort of 251 patients with COVID-19 in the setting of known IPF. Outcomes were compared with a propensity matched cohort of patients with COVID-19 without IPF.MethodsAnalysis of a federated multicentre research network TriNetX was performed including patients more than 16 years of age diagnosed with SARS-CoV-2 infection. Outcomes in patients diagnosed as positive for SARS-CoV-2 infection with concurrent IPF were compared with a propensity matched cohort of patients without IPF.ResultsA total of 311 060 patients with SARS-CoV-2 infection on the research network were identified, 251 patients (0.08%) carried a diagnosis of IPF. Mean age of patients with IPF was 68.30±12.20 years, with male predominance (n=143, 56.97%). Comorbidities including chronic lower respiratory diseases, diabetes mellitus, ischaemic heart disease and chronic kidney disease were more common in patients with IPF when compared with the non-IPF cohort. After propensity matching, higher rates of composite primary outcome (death or mechanical ventilation) at 30 and 60 days, as well as need for hospitalisation, critical care, and acute kidney injury were observed in the IPF cohort.ConclusionPoor outcomes of COVID-19 disease were observed in patients with IPF after robust matching of confounders. Our data confirm that patients with IPF constitute a high-risk cohort for poor outcomes related to COVID-19 disease.

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Changes in Neutrophil–Lymphocyte or Platelet–Lymphocyte Ratios and Their Associations with Clinical Outcomes in Idiopathic Pulmonary Fibrosis

Journal of Clinical Medicine ◽

10.3390/jcm10071427 ◽

2021 ◽

Vol 10 (7) ◽

pp. 1427

Author(s):

Steven D. Nathan ◽

Jayesh Mehta ◽

John Stauffer ◽

Elizabeth Morgenthien ◽

Ming Yang ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Clinical Outcomes ◽

Predictive Biomarkers ◽

Phase Iii ◽

Response To Treatment ◽

Reference Ranges ◽

Neutrophil Lymphocyte Ratio ◽

Lymphocyte Ratio ◽

The Relationship

Identification of prognostic and predictive biomarkers in idiopathic pulmonary fibrosis (IPF) could aid assessment of disease severity and prediction of progression and response to treatment. This analysis examined reference ranges for neutrophil–lymphocyte ratio (NLR) and platelet–lymphocyte ratio (PLR) in IPF, and the relationship between NLR or PLR changes and clinical outcomes over 12 months. This post hoc analysis included patients with IPF from the Phase III, double-blind trials of pirfenidone, ASCEND (NCT01366209) and CAPACITY (NCT00287716 and NCT00287729). The relationship between change from baseline to Month 12 in NLR or PLR (divided into quartiles (Q1–Q4)) and outcomes (mortality, respiratory hospitalization, declines in lung function, exercise capacity and quality of life) was assessed. Estimated reference ranges at baseline for all patients analyzed (n = 1334) were 1.1–6.4 for NLR and 56.8–250.5 for PLR. Significant trends were observed across NLR and PLR quartiles for all outcomes in placebo-treated patients, with patients manifesting the greatest NLR or PLR changes experiencing the worst outcomes. These results suggest that the greatest NLR or PLR changes over 12 months were associated with worse clinical outcomes. Further research is needed to determine the utility of NLR and PLR as prognostic biomarkers in IPF.

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Blood mitochondrial DNA as a biomarker of clinical outcomes in idiopathic pulmonary fibrosis

European Respiratory Journal ◽

10.1183/13993003.01769-2020 ◽

2020 ◽

Vol 56 (5) ◽

pp. 2001769

Author(s):

Hee-young Yoon ◽

Kwanghun Choi ◽

Miae Kim ◽

Hak-Su Kim ◽

Jin Woo Song

Keyword(s):

Mitochondrial Dna ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Clinical Outcomes

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Longitudinal clinical outcomes in a real-world population of patients with idiopathic pulmonary fibrosis: the PROOF registry

Respiratory Research ◽

10.1186/s12931-019-1182-z ◽

2019 ◽

Vol 20 (1) ◽

Cited By ~ 3

Author(s):

Wim A. Wuyts ◽

Caroline Dahlqvist ◽

Hans Slabbynck ◽

Marc Schlesser ◽

Natacha Gusbin ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Pulmonary Function ◽

Clinical Outcomes ◽

Real World ◽

Lung Transplant ◽

World Population ◽

Duration Of Treatment

Abstract Background The PROOF registry is an observational study initiated in October 2013 with the aim to monitor disease progression in a real-world population of patients with idiopathic pulmonary fibrosis (IPF). Here, we present longitudinal clinical outcomes from the PROOF registry. Methods Patients with IPF were enrolled across eight centers in Belgium and Luxembourg. For all patients, clinical outcomes data were collected, including mortality, lung transplant, acute exacerbations, and pulmonary hypertension. For patients treated with pirfenidone at any time during follow-up (2013–2017), for any duration of treatment (the pirfenidone-treated population): pirfenidone treatment patterns were collected; changes in pulmonary function (forced vital capacity [FVC] and carbon monoxide diffusing capacity [DLco]) were reviewed up to 24 months post-inclusion; and time-to-event analyses from the time of registry inclusion were performed. Results The PROOF registry enrolled a total of 277 patients. During follow-up, 23.1% of patients died, 5.1% received a lung transplant, 5.4% experienced an acute exacerbation, and 6.1% had comorbid pulmonary hypertension. In the pirfenidone-treated population (N = 233, 84.1%), 12.9% of patients had a temporary dose discontinuation and 31.8% had a temporary dose reduction; 4.3% of patients permanently discontinued pirfenidone due to an adverse drug reaction. Mean percent predicted FVC was 81.2% (standard deviation [SD] 19.0) at Month 0 and 78.3% (SD 25.0) at Month 24, and mean percent predicted DLco was 47.0% (SD 13.2) and 45.0% (SD 16.5), respectively. Rates of ≥ 10% absolute decline in percent predicted FVC and ≥ 15% absolute decline in percent predicted DLco over 24 months were 31.0% and 23.2%, respectively. Mean times from registry inclusion to categorical absolute decline in percent predicted FVC and percent predicted DLco were 20.1 (standard error [SE] 0.6) months and 23.4 (SE 0.5) months, respectively; mean time from registry inclusion to death was 31.0 (SE 0.9) months. Conclusions The PROOF registry is a source of European data characterizing longitudinal clinical outcomes of patients with IPF. Over 12 months of follow-up, pulmonary function remained largely stable in patients with IPF who received pirfenidone for any duration of treatment. Pulmonary function remained similar at 24 months of follow-up, although patient numbers were lower. Trial registration PROOF is registered with the relevant authorities in Belgium and Luxembourg, with registration to Comité National d’Éthique et de Recherche (CNER) N201309/03–12 September 2013 and a notification to Comité National de Protection des Données (CNDP) for Luxembourg.

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Changes in neutrophil-lymphocyte and platelet-lymphocyte ratios and clinical outcomes in idiopathic pulmonary fibrosis

10.1183/13993003.congress-2018.pa4805 ◽

2018 ◽

Cited By ~ 1

Author(s):

Sangeeta Bhorade ◽

Elizabeth Morgenthien ◽

Jessica Castle ◽

Susan L. Limb ◽

Jayesh Mehta

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Clinical Outcomes

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Clinical Outcomes of Patients with Combined Idiopathic Pulmonary Fibrosis and Emphysema in the IPF-PRO Registry

Lung ◽

10.1007/s00408-021-00506-x ◽

2022 ◽

Author(s):

Hyun J. Kim ◽

Laurie D. Snyder ◽

Megan L. Neely ◽

Anne S. Hellkamp ◽

David L. Hotchkin ◽

...

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Clinical Outcomes ◽

Proportional Hazards ◽

Cox Proportional Hazards ◽

Cox Proportional Hazards Models ◽

Baseline Variable ◽

Former Smokers ◽

The Impact ◽

Baseline Covariates

Abstract Purpose To assess the impact of concomitant emphysema on outcomes in patients with idiopathic pulmonary fibrosis (IPF). Methods The IPF-PRO Registry is a US registry of patients with IPF. The presence of combined pulmonary fibrosis and emphysema (CPFE) at enrollment was determined by investigators’ review of an HRCT scan. Associations between emphysema and clinical outcomes were analyzed using Cox proportional hazards models. Results Of 934 patients, 119 (12.7%) had CPFE. Compared with patients with IPF alone, patients with CPFE were older (median 72 vs 70 years); higher proportions were current/former smokers (88.2% vs 63.7%), used oxygen with activity (49.6% vs 31.9%) or at rest (30.8% vs 18.4%), had congestive heart failure (13.6% vs 4.8%) and had prior respiratory hospitalization (25.0% vs 16.7%); they had higher FVC (median 71.8 vs 69.4% predicted) and lower DLco (median 35.3 vs 43.6% predicted). In patients with CPFE and IPF alone, respectively, at 1 year, rates of death or lung transplant were 17.5% (95% CI: 11.7, 25.8) and 11.2% (9.2, 13.6) and rates of hospitalization were 21.6% (14.6, 29.6) and 20.6% (17.9, 23.5). There were no significant associations between emphysema and any outcome after adjustment for baseline variables. No baseline variable predicted outcomes better in IPF alone than in CPFE. Conclusion Approximately 13% of patients in the IPF-PRO Registry had CPFE. Physiologic characteristics and comorbidities of patients with CPFE differed from those of patients with IPF alone, but the presence of emphysema did not drive outcomes after adjustment for baseline covariates. Trial registration ClinicalTrials.gov, NCT01915511; registered August 5, 2013.

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