The Idiopathic Pulmonary Fibrosis Clinical Research Network (IPFnet)

Collard, Harold R.; Colby, Thomas V.; Schwarz, Marvin I.; Zisman, David A.; Chapman, Jennifer; Olman, Mitchell A.; Lubell, S.; Morrison, L. D.; Steele, Mark P.; Haram, T.; Roman, Jesse; Perez, Rafael L.; Pérez, T. Alonso; Ryu, Jay H.; Utz, James P.; Limper, Andrew H.; Daniels, Craig; Meiras, K.; Walsh, Simon L.F.; Brown, Kevin K.; Bair, Christopher; Kervitsky, Dolly; Lasky, Joseph A.; Ditta, S.; deAndrade, Joao; Thannickal, Victor J.; Stewart, M. K.; Calahan, E.; López, Pedro; King, Talmadge E.; Jeffrey, Robert R.; Noth, Imre; Hogarth, D. Kyle; Sandbo, Nathan; Strek, Mary E.; White, Steve R.; Brown, C. Ann; Garic, Irena; Maleckar, Spring; Martínez, Fernando J.; Flaherty, Kevin R.; Moore, Bethany B.; Toews, Galen B.; Dahlgren, D.; Raghu, Ganesh; Hayes, Jennifer; Snyder, Mariah; Loyd, James E.; Lancaster, Lisa; Lawson, William; Greer, Rebecca; Mason, Wendi R.; Kaner, Robert J.; Monroy, Virginia Sánchez; Wang, M.; Lynch, David A.; Anstrom, Kevin J.; Becker, Ricardo; Eisenstein, Eric L.; MacIntyre, Neil R.; Rochon, Justine; Sundy, John S.; Davidson‐Ray, Linda; Dignacco, Patricia; Edwards, Rex; Anderson, RA; Beci, R.; Calvert, Sara B.; Cain, Kelli L.; Gentry-Bumpass, Tedryl; Hill, David B.; Ingham, M.; Kagan, Elena; Kaur, Jasleen; Matti, C.; McClelland, Jodie A.; Meredith, Amanda; Nguyen, Trọng Hung; Pesarchick, Jean; Roberts, Rhonda; Tate, Wendy R.; Thomas, Tracy A.; Walker, Jeremy; Whelan, Daniel B.; Winsor, Jane; Yang, Qinghong; Yow, Eric; Tian, Xinlun; Kiley, James P.; Hunninghake, Gary M.; Culver, Daniel A.; Wehrmann, Ron; Kidd, Raven; Kallay, Michael C.; Lyda, Elizabeth; Sahn, Steven A.; O'Banner, N.; Stokes, Felicia; Ettinger, Neil A.; Merli, Stefania; Andrade, Joao de; Belperio, John A.; Lynch, Joseph P.; Golden, Jeffrey A.; Wolters, Paul J.; Eller, Alexandra; Rahimova, G.; Sardin, Latriese V.; Glassberg, Marilyn K.; Simonet, Emmanuelle S.; Baumann, Kathryn; Chan, K.W.; Chughtai, Aamer; Gross, Benjamin D.; Han, Mei Lan K.; Hyzy, Robert C.; Kazerooni, Ella A.; Myers, Jeffrey L.; White, Eric S.; Rossman, Milton D.; Kreider, Maryl; Le, Ke-Jia; Fitzgerald, John E.; Glazer, Craig S.; Scholand, Mary Beth; Brewster, Luke P.; Johnson, ArnoldL.; Berry-Bell, P.; Snydsman, A.; Kinser, Kathleen J.; Richardson, Ronald R.; Bandong, K.; Antin‐Ozerkis, Danielle; Holm, C.; Estrom, J.; Hofmann, Patrick; Hwang, Kung‐Chang; Ramey, Sharon Landesman; Sharlow, Amber; Weinmann, Gail G.; Reynolds, Herbert Y.; Schmetter, Barry; Zibrak, Joseph D.; Demersky, A.; Vey, M.; Rosas, Iván O.; Debrosse, P.; Padilla, María; Berhanu, Gemechu; Criner, Gerard J.; Swift, Irene; Satti, Aditi; Cordova, Francis; Patel, Nina; West, Kelly M.; Jones, Georgina; Huang, Kun

doi:10.1378/chest.14-2889

Cited by 39 publications

(24 citation statements)

References 10 publications

(2 reference statements)

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“…As such, non-elective respiratory hospitalization appears to be the optimal clinical intermediate marker for long-term mortality in IPF. This evidence has been incorporated in CleanUP-IPF, including the use of an adjudication group based on IPFnet experience [49]. The CleanUP-IPF event adjudication process is designed to be both efficient and accurate, incorporating the [30].…”

Section: Primary Endpoints and Endpoint Adjudicationmentioning

confidence: 99%

Design and rationale of a multi-center, pragmatic, open-label randomized trial of antimicrobial therapy – the study of clinical efficacy of antimicrobial therapy strategy using pragmatic design in Idiopathic Pulmonary Fibrosis (CleanUP-IPF) clinical trial

et al. 2020

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Compelling data have linked disease progression in patients with idiopathic pulmonary fibrosis (IPF) with lung dysbiosis and the resulting dysregulated local and systemic immune response. Moreover, prior therapeutic trials have suggested improved outcomes in these patients treated with either sulfamethoxazole/ trimethoprim or doxycycline. These trials have been limited by methodological concerns. This trial addresses the primary hypothesis that long-term treatment with antimicrobial therapy increases the time-to-event endpoint of respiratory hospitalization or all-cause mortality compared to usual care treatment in patients with IPF. We invoke numerous innovative features to achieve this goal, including: 1) utilizing a pragmatic randomized trial design; 2) collecting targeted biological samples to allow future exploration of 'personalized' therapy; and 3) developing a strong partnership between the NHLBI, a broad range of investigators, industry, and philanthropic organizations. The trial will randomize approximately 500 individuals in a 1: 1 ratio to either antimicrobial therapy or usual care. The site principal investigator will declare their preferred initial antimicrobial treatment strategy (trimethoprim 160 mg/ sulfamethoxazole 800 mg twice a day plus folic acid 5 mg daily or doxycycline 100 mg once daily if body weight is < 50 kg or 100 mg twice daily if ≥50 kg) for the participant prior to randomization. Participants randomized to antimicrobial therapy will receive a voucher to help cover the additional

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Section: Primary Endpoints and Endpoint Adjudicationmentioning

confidence: 99%

Design and rationale of a multi-center, pragmatic, open-label randomized trial of antimicrobial therapy – the study of clinical efficacy of antimicrobial therapy strategy using pragmatic design in Idiopathic Pulmonary Fibrosis (CleanUP-IPF) clinical trial

et al. 2020

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“…Previous studies showed various availability of HRCT for suspected patients with AE‐IPF: 50% (9/18) in a Sildenafil study and 86% (12/14) in a Japanese pirfenidone study . In addition, even in clinical trials, insufficient evaluation for suspected AE‐IPF was carried out in a significant minority of patients; 35% (31/88) of reported AE‐IPF cases were diagnosed as unclassifiable acute worsening because of insufficient data by central analysis . Given the crucial prognosis of AE‐IPF, sufficient and precise diagnostic evaluation is essential.…”

Section: Acute and Subacute Iipsmentioning

confidence: 99%

Acute and subacute idiopathic interstitial pneumonias

Taniguchi

Kondoh

2016

Respirology

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Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE‐IPF) and AE‐NSIP. Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug‐induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs. Despite the severe lack of randomized controlled trials for the treatment of acute and subacute IIPs, the mainstream treatment remains corticosteroid therapy. Other potential therapies reported in the literature include corticosteroids and immunosuppression, antibiotics, anticoagulants, neutrophil elastase inhibitor, autoantibody‐targeted treatment, antifibrotics and hemoperfusion therapy. With regard to mechanical ventilation, patients in recent studies with acute and subacute IIPs have shown better survival than those in previous studies. Therefore, a careful value‐laden decision about the indications for endotracheal intubation should be made for each patient. Noninvasive ventilation may be beneficial to reduce ventilator associated pneumonia.

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“…Chronic hypersensitivity pneumonitis can often be misdiagnosed as IPF [69]. Currently there are no approved therapies for hypersensitivity pneumonitis.…”

Section: Hypersensitivity Pneumonitismentioning

confidence: 99%

Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence

Richeldi

Varone

Bergna³

et al. 2018

Eur Respir Rev

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A proportion of patients with interstitial lung diseases (ILDs) are at risk of developing a progressive-fibrosing phenotype, which is associated with a deterioration in lung function and early mortality. In addition to idiopathic pulmonary fibrosis (IPF), fibrosing ILDs that may present a progressive phenotype include idiopathic nonspecific interstitial pneumonia, connective tissue disease-associated ILDs, hypersensitivity pneumonitis, unclassifiable idiopathic interstitial pneumonia, ILDs related to other occupational exposures and sarcoidosis. Corticosteroids and/or immunosuppressive therapies are sometimes prescribed to patients with these diseases. However, this treatment regimen may not be effective, adequate on its own or well tolerated, suggesting that there is a pressing need for efficacious and better tolerated therapies. Currently, the only approved treatments to slow disease progression in patients with IPF are nintedanib and pirfenidone. Similarities in pathobiological mechanisms leading to fibrosis between IPF and other ILDs that may present a progressive-fibrosing phenotype provide a rationale to suggest that nintedanib and pirfenidone may be therapeutic options for patients with the latter diseases.This review provides an overview of the therapeutic options currently available for patients with fibrosing ILDs, including fibrosing ILDs that may present a progressive phenotype, and explores the status of the randomised controlled trials that are underway to determine the efficacy and safety of nintedanib and pirfenidone.

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The Idiopathic Pulmonary Fibrosis Clinical Research Network (IPFnet)

Abstract: ClinicalTrials.gov; No.: NCT00517933, NCT00650091, NCT00957242; URL: www.clinicaltrials.gov.

Cited by 39 publications

References 10 publications

Design and rationale of a multi-center, pragmatic, open-label randomized trial of antimicrobial therapy – the study of clinical efficacy of antimicrobial therapy strategy using pragmatic design in Idiopathic Pulmonary Fibrosis (CleanUP-IPF) clinical trial

Design and rationale of a multi-center, pragmatic, open-label randomized trial of antimicrobial therapy – the study of clinical efficacy of antimicrobial therapy strategy using pragmatic design in Idiopathic Pulmonary Fibrosis (CleanUP-IPF) clinical trial

Acute and subacute idiopathic interstitial pneumonias

Pharmacological management of progressive-fibrosing interstitial lung diseases: a review of the current evidence

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