Clinical Trial on Idiopathic Pulmonary Fibrosis - Clinical Trials Registry

Study Overview

Status

Completed

Conditions

Idiopathic Pulmonary Fibrosis

Detailed Description

Idiopathic pulmonary fibrosis (IPF) is a chronic disease of unknown etiology, characterized by scar tissue (fibrosis) within the lungs. IPF is one of the most common forms of interstitial lung disease and is associated with substantial morbidity and mortality (average survival of approximately three years from the time of diagnosis)).

The epidemiology and natural history of IPF is still not completely understood. Incidence and prevalence of IPF are difficult to determine because uniform diagnostic criteria have only recently been defined. Recent data among subjects aged 50 years or older suggests an age- and sex-adjusted incidence rate ranging from 8.8 cases to 17.4 cases per 100,000 person-years in the USA. In the same population, age- and sex- adjusted prevalence ranged from 27.9 cases to 63 cases per 100,000 persons.

Familial form of IPF accounts for 0.5 to 2% of all cases of IPF. Several environmental or occupational exposures seem to be prevalent in the medical histories of patients diagnosed with the disease.

The onset of IPF symptoms is slow, but symptoms become progressively worse over time. Breathlessness upon exertion and chronic dry cough are the major symptoms. Gastro-esophageal acid reflux is present in almost 90% of patients with IPF but often occurs without symptoms.

Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of patients with IPF.

Quality of life of patients with IPF is impacted by the disease.

Establishing an accurate diagnosis for the patient who presents with interstitial lung disease is an essential component of management. The recent ATS/ERS/JRS/ALAT recommendations on the diagnosis and management of IPF were developed from a systematic review of the published literature. High-resolution computed tomography (HRCT) scanning has a central role in the IPF diagnostic pathway with formal designation of criteria for an HRCT pattern of Usual Interstitial Pneumonia (UIP). In the correct clinical context, a UIP pattern on HRCT is indicative of a definite diagnosis of IPF without the need for a surgical lung biopsy.

IPF presents significant associations with other cardiopulmonary disorders including coronary artery disease, pulmonary embolism, sleep apnea, respiratory infections and lung cancer. In addition, patients with IPF are at risk for 'acute exacerbations'. Acute exacerbation is defined as an acute, clinically significant respiratory deterioration characterized by evidence of new widespread alveolar abnormality. Diagnostic criteria are: previous or concurrent diagnosis of IPF; acute worsening or development of dyspnea typically < 1 mo duration; computed tomography with new bilateral ground-glass opacity and/or consolidation superimposed on a background pattern consistent with usual interstitial pneumonia pattern; deterioration not fully explained by cardiac failure or fluid overload. These acute exacerbations are responsible for 50% of deaths in IPF and may affect previously stable patients.

The clinical course of IPF is highly variable and as a result, therapeutic strategies should be highly individualized, based upon the specific patients' medical history and co-morbidities.

Pirfenidone and Nintedanib are drugs approved for the treatment of adult patients with mild to moderate idiopathic pulmonary fibrosis in the European Union. In order to complement the data collected in previous studies, we implement a registry to describe the epidemiology and natural history of IPF, as well as the quality of life of IPF patients, and use of health care resources, in Belgium and Luxembourg. This registry will also enable the collection of clinical data of patients treated with Pirfenidone or Nintedanib and other therapies used in the treatment of IPF. The registry will be implemented in collaboration with pulmonologists and expert centers in order to enable the inclusion and follow up of recently diagnosed IPF patients.

Study Type

Observational

Enrollment (Actual)

575

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

Belgium
- - Antwerp, Belgium, 2000
    - Ziekenhuisnetwerk Antwerpen vzw
  - Brussels, Belgium, 1200
    - Cliniques universitaires Saint-Luc
  - Brussels, Belgium, 1070
    - Cliniques Universitaires de Bruxelles - Hôpital Erasme
  - Ghent, Belgium, 9000
    - Universitair Ziekenhuis Gent
  - Leuven, Belgium, 3000
    - University Hospitals Leuven
  - Liège, Belgium, 4000
    - Centre Hospitalier Universitaire Sart Tilman
  - Liège, Belgium, 4000
    - CHR de la Citadelle
  - Yvoir, Belgium, 5530
    - CHU UCl Namur asbl - Site Godinne
Luxembourg
- - Luxembourg, Luxembourg, 1210
    - Centre Hospitalier de Luxembourg (CHL)/ Luxembourg Institute of Health (LIH)

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

19 years and older (ADULT, OLDER_ADULT)

Accepts Healthy Volunteers

No

Genders Eligible for Study

All

Sampling Method

Probability Sample

Study Population

Adults diagnosed with an IPF diagnosis "definite" or "probable" (according to 2011 ATS/ERS guidelines definitions) which is confirmed by a multidisciplinary team (= minimum of pulmonologist, radiologist, pathologist, all with expertise in IPF).

Description

Inclusion Criteria:

Patients >18 years of age
IPF diagnosis "definite" or "probable" (2011 ATS/ERS guidelines definitions) confirmed by a multidisciplinary team (= minimum of pulmonologist, radiologist, pathologist, all with expertise in IPF).
Patients who agreed to participate in the registry and have completed and signed the Informed Consent Form

Exclusion Criteria:

Patients incapable of giving informed consent
Patients participating in a clinical trial at the time of inclusion in the registry and whose protocol does not allow participation in another trial

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
The first occurrence of a decrease of ≥ 10% in percent predicted forced vital capacity (FVC) since baseline. Time Frame: overall duration of study (5 years)	FVC data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The first occurrence of a decrease of ≥ 15% in percent predicted diffusing capacity of the lung for carbon monoxide (DLCO) since baseline Time Frame: overall duration of study (5 years)	DLCO data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
Number of participants alive Time Frame: overall duration of study (5 years)	Data on death will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The evolution of percent predicted forced vital capacity (FVC) Time Frame: overall duration of study (5 years)	FVC data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The evolution of percent predicted diffusing capacity of the lung for carbon monoxide (DLCO) Time Frame: overall duration of study (5 years)	DLCO data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The number of acute exacerbations of IPF Time Frame: overall duration of study (5 years)	Acute exacerbations of IPF data will be retrieved from the medical file of the participant.	overall duration of study (5 years)

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
The number of patients per IPF treatment drug Time Frame: overall duration of study (5 years)	IPF treatment drug data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The mean initial dose per IPF treatment drug Time Frame: overall duration of study (5 years)	IPF treatment drug data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The mean number of dose changes per IPF treatment drug Time Frame: overall duration of study (5 years)	IPF treatment drug data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The number of participants who have discontinued the IPF treatment per IPF treatment drug Time Frame: overall duration of study (5 years)	IPF treatment drug data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The mean duration of treatment in days per IPF treatment drug Time Frame: overall duration of study (5 years)	IPF treatment drug data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The mean duration of dose reduction in days per IPF treatment drug Time Frame: overall duration of study (5 years)	IPF treatment drug data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The mean duration of drug interruption in days per IPF treatment drug Time Frame: overall duration of study (5 years)	IPF treatment drug data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The number of participants with lung transplantation Time Frame: overall duration of study (5 years)	Lung transplantation data will be retrieved from the medical file of the participant.	overall duration of study (5 years)
The (S)ADRs per IPF treatment reported which are possibly or probably related to the IPF treatment drug. Time Frame: overall duration of study (5 years)	(S)ADR data will be retrieved from the medical file of the patient.	overall duration of study (5 years)
The utilisation of health care resources by participants Time Frame: overall duration of study (5 years)	The utilisation of health care resources will be retrieved from the medical file of the patient.	overall duration of study (5 years)
The impact of IPF and IPF treatment on the severity of cough measured with the Cough-Visual Analogue Scale. Time Frame: overall duration of study (5 years)	Cough severity in the last 2 weeks was assessed at inclusion and at every follow-up visit using the Cough-VAS, a linear visual scale with 0 mm representing 'no cough' and 100 mm.	overall duration of study (5 years)
The impact of IPF and IPF treatment on quality of life measured with the SGRQ. Time Frame: overall duration of study (5 years)	The St- George's Respiratory questionnaire (SGRQ) is a 50-item questionnaire consisting of two parts. The first part evaluates the symptoms (i.e. cough, sputum production, wheezing and breathlessness) and the second part evaluates the activities (i.e. activities causing breathlessness or limited by breathlessness) and the impacts (e.g. on employment, daily life, etc.). Each question is scored as positive (i.e. =1) or negative (i.e. =0) and the total score is expressed as a percentage where 0% represents the best possible health status and 100% the worst possible health status. A total score can be obtained as well as a score for each of the three components. Data are collected at inclusion and minimal twice a year during follow-up.	overall duration of study (5 years)
The impact of IPF and IPF treatment on quality of life measured with the EQ-5D-5L questionnaire. Time Frame: overall duration of study (5 years)	The EQ-5D-5L consists of the EQ-5D descriptive system and the EQ visual analogue scale (EQ VAS). The EQ-5D descriptive system comprises five dimensions: mobility, self-care, usual activities, pain/discomfort and anxiety/depression. Each dimension has 5 levels, going from no problems to extreme problems. Only 1 option can be chosen by the patient. This decision results in a 1-digit number that expresses the level selected for that dimension: no problems (=1), slight problems(=2), moderate problems(=3), severe problems(=4) and extreme problems(=5). The digits for the five dimensions can be combined into a 5-digit number that describes the patient's health state.The EQ VAS records the patient's self-rated health on a vertical visual analogue scale, where 0 mm represents 'worst health imaginable' and 100 mm representing 'best health imaginable'. Data are collected at Inclusion and at every follow-up visit.	overall duration of study (5 years)
The impact of IPF and IPF treatment on quality of life measured with with the K-BILD questionnaire Time Frame: overall duration of study (5 years)	Patients will receive the K-BILD questionnaire measuring their health-related quality of life. The K-BILD is a 15-item self-completed questionnaire measuring the health-related quality of life (HRQL) of patients with interstitial lung disease. It consists of three domains: breathlessness and activities, psychological and chest symptoms. Each question has a seven-point response scale resulting in a total score ranging between 0 and 100 with a higher score reflecting a higher HRQL. Data are collected at inclusion and at every follow-up visit.	overall duration of study (5 years)

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Belgian Thoracic Society

Collaborators

Boehringer Ingelheim

Roche Farma, S.A

Investigators

Study Director: Wim Wuyts, Phd, MD, Universitaire Ziekenhuizen KU Leuven

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (ACTUAL)

January 9, 2018

Primary Completion (ACTUAL)

November 1, 2021

Study Completion (ACTUAL)

January 9, 2022

Study Registration Dates

First Submitted

July 26, 2018

First Submitted That Met QC Criteria

November 5, 2018

First Posted (ACTUAL)

November 7, 2018

Study Record Updates

Last Update Posted (ACTUAL)

February 15, 2022

Last Update Submitted That Met QC Criteria

February 14, 2022

Last Verified

February 1, 2022

More Information

Terms related to this study

Keywords

IPF

Additional Relevant MeSH Terms

Other Study ID Numbers

BVPSBP01

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

NO

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

PROOF-Registry New and Extended Belgium -Luxembourg (PROOF-NEXT)

A Prospective Observational Registry to Describe the Disease Course and Outcomes of Idiopathic Pulmonary Fibrosis Patients in a Real-world Clinical Setting. PROOF-Registry New and Extended Belgium -Luxembourg