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Validation of the Risk Stratification Score in Idiopathic Pulmonary Fibrosis

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ClinicalTrials.gov Identifier: NCT02632123
Recruitment Status : Recruiting
First Posted : December 16, 2015
Last Update Posted : November 17, 2021
Sponsor:
Information provided by (Responsible Party):
Lawson Health Research Institute

Brief Summary:

Idiopathic pulmonary fibrosis (IPF) is characterized by a poor prognosis, with a progressive decline in lung function and a considerable variability in the disease's natural history. Besides lung transplantation (LTx), the only available treatments are anti-fibrosing drugs, which have shown to slower the disease course. Therefore, predicting the prognosis is of pivotal importance to avoid treatment delays, which may be fatal for patients with a high risk of progression. Previous studies showed that a multi-dimensional approach is practical and effective to create a reliable prognostic score for IPF. In the RIsk Stratification scorE (RISE), physiological parameters, an objective measure of patient-reported dyspnea and exercise capacity are combined to capture different domains of the complex pathophysiology of IPF.

This is an observational, multi-centre, prospective cohort study. A development cohort and a validation cohort will be included. Patients newly diagnosed with IPF based on the ATS/ERS criteria and multi-disciplinary discussion will be included in the study. A panel of chest radiologists and lung pathologists will further assess eligibility. At the first visit (time of diagnosis), and every 4-months, MRCDS, pulmonary function tests (FEV1, FVC and DLCO), and 6MWD will be recorded and patients will be prospectively followed for 3 years. Comorbidities will be considered. The radiographic extent of fibrosis on HRCT will be recalculated at a 2-year interval. RISE, Gender-Age-Physiology, CPI and Mortality Risk Scoring System will be calculated at 4-month intervals. Longitudinal changes of each variable considered will be assessed. The primary endpoint is 3-year LTx-free survival from the time of diagnosis. Secondary endpoints include several, clinically-relevant information to ensure reproducibility of results across a wide range of disease severity and in concomitance of associated pulmonary hypertension, emphysema.

The present study aims at validating RISE as a simple, straightforward, inexpensive and reproducible tool to guide clinical decision making in IPF and potentially as an endpoint for future clinical trials.


Condition or disease
Idiopathic Pulmonary Fibrosis

Show Show detailed description

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Study Type : Observational
Estimated Enrollment : 260 participants
Observational Model: Cohort
Time Perspective: Prospective
Official Title: Validation of the Risk Stratification Score in Idiopathic Pulmonary Fibrosis
Actual Study Start Date : December 2015
Estimated Primary Completion Date : December 2025
Estimated Study Completion Date : December 2025


Group/Cohort
Development cohort
Patients newly diagnosed with idiopathic pulmonary fibrosis
Validation cohort
Patients newly diagnosed with idiopathic pulmonary fibrosis



Primary Outcome Measures :
  1. Survival [ Time Frame: 3 years ]
    Primary outcome is lung-transplant-free 3-year survival since the time of diagnosis


Secondary Outcome Measures :
  1. Subgroup analysis [ Time Frame: 3 years ]
    Comparative analysis of RISE as predictor of LTx-free survival in subgroups stratified by age; gender; baseline lung function; baseline 6MWD; baseline extent of fibrosis on HRCT; time between onset of symptoms and diagnosis; presence of APH; presence of concomitant emphysema; presence of comorbidities (COPD, CAD, LHD, SA); participating site

  2. Clinical progression [ Time Frame: 3 years ]
    Clinical progression as a predictor of mortality, defined as either: absolute decline of FVC >10% pred; decline of 6-min walk distance >50 m; hospitalization for respiratory causes; LTx assessment; or death

  3. Incidence of acute exacerbations [ Time Frame: 3 years ]
    Incidence of acute exacerbations (AEs) of IPF and its predictors, if any.

  4. Incidence of hospitalizations [ Time Frame: 3 years ]
    Incidence of hospitalizations due to respiratory causes and its predictors, if any.

  5. Results stratification by HRT pattern [ Time Frame: 3 years ]
    LTx-free survival, incidence of AEs, incidence of hospitalizations, and time to progression of patients with HRCT pattern definite/probable for UIP vs. patients with indeterminate/inconsistent pattern. In this subanalysis, 2 groups of patients matched for age, gender, BMI, and extent of fibrosis at the time of diagnosis will be considered.

  6. Results stratification by autoimmune markers [ Time Frame: 3 years ]
    LTx-free survival, incidence of AEs, incidence of hospitalizations, and time to progression of patients with autoimmune markers (although not meeting criteria for interstitial pneumonia with autoimmune features vs. those without. In this subanalysis, 2 groups of patients matched for age, gender, BMI and extent of fibrosis at the time of diagnosis will be considered

  7. Results stratification by concomitant emphysema [ Time Frame: 3 years ]
    LTx-free survival, incidence of AEs, incidence of hospitalizations and time to progression of patients with concomitant emphysema vs. those without. In this subanalysis, 2 groups of patients matched for age, gender, BMI and extent of fibrosis at the time of diagnosis will be considered.

  8. Results stratification by anti-fibrotic treatment [ Time Frame: 3 years ]
    LTx-free survival, incidence of AEs, incidence of hospitalizations and time to progression of patients treated with pirfenidone vs. patients treated with nintedanib. In this subanalysis, 2 groups of patients matched for age, gender, BMI and extent of fibrosis at the time of diagnosis will be considered.

  9. Results stratification by anti-fibrotic treatment switch [ Time Frame: 3 years ]
    LTx-free survival, incidence of AEs, incidence of hospitalizations and time to progression of patients whose therapy was switched (from pirfenidone to nintedanib and viceversa). In this subanalysis, 2 groups of patients matched for age, gender, BMI and extent of fibrosis at the time of diagnosis will be considered.

  10. 6MWD m vs. % pred as predictors of mortality [ Time Frame: 3 years ]
    6-min walk distance in meters vs. percent predicted as predictors of mortality.

  11. Results on patient listed for lung transplant [ Time Frame: 3 years ]
    Survival, incidence of AEs and time to progression on the LTx list of listed patients.

  12. Time to progression in patients with initially non-clinically significant disease. [ Time Frame: 3 year ]
    Time to clinical progression in patients who initially had non-clinically significant disease

  13. Long-term follow-up [ Time Frame: 5 years ]
    Long-term follow-up (beyond 3 years) of patients who are still alive without a LTx, until required recruitment is completed. This will include LTx-free survival, incidence of AEs, incidence of hospitalizations and time to progression.



Information from the National Library of Medicine

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Ages Eligible for Study:   18 Years and older   (Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Patients newly diagnosed with idiopathic pulmonary fibrosis.
Criteria

Inclusion Criteria:

  • A new diagnosis of IPF based on the American Thoracic Society/European Respiratory Society criteria (Am J Respir Crit Care Med 2018;198:e44-e68) and confirmed by a panel of expert chest radiologists and lung pathologists in the context of multi-disciplinary discussion.

Exclusion Criteria:

  • Interstitial lung disease other than IPF
  • Not a new diagnosis of IPF

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT02632123


Contacts
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Contact: Marco Mura, MD, PhD +1-5196676744 marco.mura@lhsc.on.ca

Locations
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Canada, Ontario
London Health Science Centre Recruiting
London, Ontario, Canada, N6A 5W9
Contact: Marco Mura, MD, PhD    +15196676744    marco.mura@lhsc.on.ca   
Contact: Maria Modod, RRT    +15196858500 ext 73451    maria.modod@lhsc.on.ca   
Principal Investigator: Marco Mura, MD, PhD         
Italy
Policlinico Tor Vergata Recruiting
Rome, Lazio, Italy, 00133
Contact: Gian Marco Manzetti, MD       gianmarcomanzetti@yahoo.it   
Principal Investigator: Paola Rogliani, MD         
Sponsors and Collaborators
Lawson Health Research Institute
Publications:

Publications automatically indexed to this study by ClinicalTrials.gov Identifier (NCT Number):
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Responsible Party: Lawson Health Research Institute
ClinicalTrials.gov Identifier: NCT02632123    
Other Study ID Numbers: 10010803
First Posted: December 16, 2015    Key Record Dates
Last Update Posted: November 17, 2021
Last Verified: November 2021
Keywords provided by Lawson Health Research Institute:
idiopathic pulmonary fibrosis
outcome
prognosis
survival
staging
Additional relevant MeSH terms:
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Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis
Fibrosis
Pathologic Processes
Lung Diseases
Respiratory Tract Diseases