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A Study to Characterize the Disease Behavior of Idiopathic Pulmonary Fibrosis (IPF) and Interstitial Lung Disease (ILD) During the Peri-Diagnostic Period

Not Applicable
Completed
Conditions
Idiopathic Pulmonary Fibrosis
Interstitial Lung Disease
Interventions
Other: Spirometry
Other: Accelerometry
Other: 6MWT
Registration Number
NCT03261037
Lead Sponsor
Hoffmann-La Roche
Brief Summary

This international clinical study will enroll participants with a suspected diagnosis of IPF/ILD. This study will characterize the disease behavior of IPF and ILD in the peri-diagnostic period. This objective will be achieved using a multidimensional approach assessing changes in pulmonary function, measured by daily handheld spirometry and site spirometry as well as assessing physical functional capacity at home (accelerometry) and at site (6-minute walk tests \[6MWT\]). Daily handheld spirometry or physical functional capacity assessments are not routinely performed in this participant population. By following participants' lung function before and after diagnosis using home spirometry, levels of physical activity, as well as self-assessment data from the participants (patient reported outcomes; PRO), the study would provide potentially more rapid information on disease behavior and eventually progression compared to usual clinic measurements that occur only every 3-6 months. By receiving data from daily handheld spirometry measurements, treating physicians may have an improved chance of detecting earlier and outside of hospital visits a decline in lung function that could potentially lead to improvements in both diagnosis and treatment for participants with IPF/ILD.

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
178
Inclusion Criteria
  • Able to comply with the study protocol, in the Investigator's judgment - for example, the ability to use the provided spirometer and tablet and the ability to fill in the required patient reported outcomes questionnaires
  • Suspicion of IPF/ILD: radiological evidence of IPF/ILD in symptomatic participants (unexplained dyspnea on exertion and/or cough)
Read More
Exclusion Criteria
  • Participation in any investigational study within 28 days prior to inclusion
  • History of clinically significant cardiac disease that could explain the patient's symptomatology in the opinion of the Investigator
  • Known history of any connective tissue disease, including, but not limited to, rheumatoid arthritis, scleroderma, systemic lupus erythematosus, or mixed connective tissue disease
Read More

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Participants With Suspicion of IPF/ILDSpirometryA participant will be eligible for inclusion if the Investigator has a suspicion that the participant may have IPF/ILD based on symptoms and radiological evidence.
Participants With Suspicion of IPF/ILDAccelerometryA participant will be eligible for inclusion if the Investigator has a suspicion that the participant may have IPF/ILD based on symptoms and radiological evidence.
Participants With Suspicion of IPF/ILD6MWTA participant will be eligible for inclusion if the Investigator has a suspicion that the participant may have IPF/ILD based on symptoms and radiological evidence.
Primary Outcome Measures
NameTimeMethod
Time-Adjusted Semi-Annual Forced Vital Capacity (FVC) Decline in Participants With IPF During the Peri-Diagnostic Period, Measured in Milliliters (mL) by Daily Home SpirometryFrom Day 1 into the study to end of the study (up to 18 months) and adjusted for 6 months. There was no fixed visit schedule for the whole study and reported results comprise all data points from enrollment to EOS

Minimum site visits were: baseline, diagnosis, and EOS for each participant. From inclusion into the study, participants were followed for up to12 months. The EOS was defined as: If no diagnosis was made 12 months after inclusion, the participant left the study; Participants diagnosed with non-ILD left the study on the date of diagnosis; Participants diagnosed with IPF or non-IPF ILD were followed up to the start of drug treatment (within 6 months) or up to 6 months if no drug treatment was prescribed. Therefore, the total length of the study was variable for each participant. Measurements were taken daily by home spirometry. The semi-annual mean FVC decline was calculated by use of the estimated semi-annual FVC decline of each individual participant. The individual FVC decline was estimated by applying a linear regression model to all acceptable FVC measurements flagged as 'Good blow' collected by the individual participant during the entire peri-diagnostic period (up to 18 months).

Secondary Outcome Measures
NameTimeMethod
Change From Baseline in the FVC Decline in IPF and Non-IPF ILD Participants During the Peri-Diagnostic Period, Measured in mL by Site SpirometryFrom Baseline to end of the study (up to 18 months)

The peri-diagnostic period covered the pre-diagnostic period (from inclusion into the study (enrollment visit), participants were followed for a maximum of 12 months in the pre-diagnostic assessment period) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

Change From Baseline in the Percent Predicted FVC in IPF and Non-IPF ILD Participants During the Peri-Diagnostic Period, Measured by Site SpirometryFrom Baseline to end of the study (up to 18 months)

The peri-diagnostic period covered the pre-diagnostic period (from inclusion into the study (enrollment visit), participants were followed for a maximum of 12 months in the pre-diagnostic assessment period) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

Change From Baseline in Distance Walked (Meters) on the 6MWT in IPF and Non-IPF ILD Participants During the Peri-Diagnostic Period, Assessed at SiteFrom Baseline to end of the study (up to 18 months)

The 6MWT measures the distance a patient is able to walk quickly on a flat, hard surface in a period of 6 minutes. The peri-diagnostic period covered the pre-diagnostic period (from inclusion into the study (enrollment visit), participants were followed for a maximum of 12 months in the pre-diagnostic assessment period) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

Fatigue Assessment Scale (FAS) Score During the Peri-Diagnostic PeriodFrom inclusion into the study to end of the study (up to 18 months)

The FAS is a fatigue questionnaire consisting of 10 items; five questions reflect physical fatigue and five questions analyze mental fatigue. A five-point scale (one for "never" to five for "always") is used for participant responses. The scale score is calculated by summing all items. Therefore, the FAS scores can range from 10 to 50. The peri-diagnostic period covered the pre-diagnostic period (from inclusion into the study (enrollment visit), participants were followed for a maximum of 12 months in the pre-diagnostic assessment period) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

Pearson's Correlation Coefficient of FVC (mL) Between Home and Site Spirometry in IPF and Non-IPF ILD Participants During the Peri-Diagnostic PeriodFrom inclusion into the study to end of the study (up to 18 months)

The correlation of FVC (mL) between home and site spirometry was analyzed by taking into account the individually estimated linear regression models of each participant for home-based FVC measurements and site FVC measurements. This analysis was performed for participants diagnosed with IPF or non-IPF ILD in the peri-diagnosis period. The peri-diagnostic period covered the pre-diagnostic period (from inclusion into the study (enrollment visit), participants were followed for a maximum of 12 months in the pre-diagnostic assessment period) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

Percentage of Participants Requiring Respiratory-Related HospitalizationsFrom inclusion into the study to end of the study (up to 18 months)

The number of participants requiring the hospitalization were counted during pre- and post-diagnosis period.

Time-Adjusted Semi-Annual FVC Decline in Participants With Non-IPF ILD During the Peri-Diagnostic Period, Measured in mL by Daily Home SpirometryFrom Day 1 into the study to end of the study (up to 18 months) and adjusted for 6 months. There was no fixed visit schedule for the whole study and reported results comprise all data points from enrollment to EOS

Minimum site visits were: baseline, diagnosis, and EOS for each participant. From inclusion into the study, participants were followed for up to12 months. The EOS was defined as: If no diagnosis was made 12 months after inclusion, the participant left the study; Participants diagnosed with non-ILD left the study on the date of diagnosis; Participants diagnosed with IPF or non-IPF ILD were followed up to the start of drug treatment (within 6 months) or up to 6 months if no drug treatment was prescribed. Therefore, the total length of the study was variable for each participant. Measurements were taken daily by home spirometry. The semi-annual mean FVC decline was calculated by use of the estimated semi-annual FVC decline of each individual participant. The individual FVC decline was estimated by applying a linear regression model to all acceptable FVC measurements flagged as 'Good blow' collected by the individual participant during the entire peri-diagnostic period (up to 18 months).

Decline in Physical Function Capacity (Steps/Day) in IPF and Non-IPF ILD Participants During the Peri-Diagnostic Period, Measured by Daily Home AccelerometryFrom inclusion into the study to end of the study (up to 18 months)

Patients were asked to wear an accelerometer during the course of the study which provided information on their physical activity at home, with daily measurements used to investigate the development of physical function capacity. The peri-diagnostic period covered the pre-diagnostic period (from inclusion into the study (enrollment visit), participants were followed for a maximum of 12 months in the pre-diagnostic assessment period) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

Calorie Expenditure in IPF and Non-IPF ILD Participants During the Peri-Diagnostic Period, Measured by Daily Home AccelerometryFrom inclusion into the study to end of the study (up to 18 months)
Change in the King's Brief Interstitial Lung Disease (K-BILD) Questionnaire Total Score by Visit During the Peri-Diagnostic PeriodFrom inclusion into the study to end of the study (up to 18 months)

The K-BILD questionnaire was specifically developed to analyze the health status of participants with ILD. The questionnaire consists of 15 items (assessed by the patients on a scale ranging from 1 to 7, where 1 and 7 represent worst and best health status). Items are compiled into 3 domains: breathlessness and activities (range: 0-21), psychological (range: 0-34) , and chest symptoms (range: 0-8). To score the K-BILD, the Likert response scale weightings for individual items are combined and scores are transformed to a range of 0-100 by using logit values (higher scores indicate better health status). The peri-diagnostic period covered the pre-diagnostic period (from enrollment visit participants were followed for up to 12 months) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

Modified Medical Research Council (mMRC) Dyspnea Scale Scores During the Peri-Diagnostic PeriodFrom inclusion into the study to end of the study (up to 18 months)

The mMRC dyspnea scale was used to grade the effects of breathlessness on daily activities over time. The following grading categories are used for this scale: Grade 0 - I only get breathless with strenuous exercise; Grade 1 - I get short of breath when hurrying on the level or walking up a slight hill; Grade 2 - I walk slower than people of the same age on the level because of breathlessness or have to stop for breath when walking on my own pace; Grade 3 - I stop for breath after walking about 100 yards or after a few minutes on level ground; Grade 4 - I am too breathless to leave the house. The peri-diagnostic period covered the pre-diagnostic period (from inclusion into the study (enrollment visit), participants were followed for a maximum of 12 months in the pre-diagnostic assessment period) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

The EuroQol 5-Dimension 5-Level Questionnaire (EQ-5D-5L) 5-Item Health State Profile Scores During the Peri-Diagnostic PeriodFrom inclusion into the study to end of the study (up to 18 months)

The EQ-5D-5L questionnaire is a self-reported health status questionnaire that assesses mobility, self-care, usual activities, pain/discomfort, and anxiety/depression. Each item is rated by the participant on a five-point scale indicating the followings: Level 1 - no problem; Level 2 - slight problems; Level 3 - moderate problems; Level 4 - severe problems; Level 5 - extreme problems. The peri-diagnostic period covered the pre-diagnostic period (from inclusion into the study (enrollment visit), participants were followed for a maximum of 12 months in the pre-diagnostic assessment period) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

Percentage of Participants With Events Related to the Study AssessmentsFrom inclusion into the study to end of the study (up to 18 months)

The number of participants with events related to the study assessments were counted during pre- and post-diagnosis period. Investigator-reported acute exacerbations, deaths, and events related to study assessments were only collected in the clinical database. As the study had no protocol mandated IMP or treatment requirements, any untoward occurrences such as AEs and SAEs were not collected.

The EuroQol Visual Analogue Scale (EQ-VAS) Health State Profile Scores During the Peri-Diagnostic Period During the Peri-Diagnostic PeriodFrom inclusion into the study to end of the study (up to 18 months)

The EQ-VAS questionnaire is a self-reported questionnaire that measures health state. The VAS is a 100 mm scale from worst (0 mm) to best (100 mm) health the participant can imagine. The peri-diagnostic period covered the pre-diagnostic period (from inclusion into the study (enrollment visit), participants were followed for a maximum of 12 months in the pre-diagnostic assessment period) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

Pearson's Correlation Coefficient of Physical Functional Capacity Assessed at Home in Terms of the Decline in the Number of Footsteps and on Site in Terms of the Decline in Distance of the 6MWTFrom inclusion into the study to end of the study (up to 18 months)

The measure represents the correlation of distance walked (meter) assessed by physical function capacity at home and the 6MWT at the site in the peri-diagnosis period. The peri-diagnostic period covered the pre-diagnostic period (from inclusion into the study (enrollment visit), participants were followed for a maximum of 12 months in the pre-diagnostic assessment period) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

Percentage of Participants Who Died Due to Respiratory-Related DiseasesFrom inclusion into the study to end of the study (up to 18 months)

The number of participants, who died due to respiratory-related diseases, were counted during pre- and post-diagnosis period.

The EuroQoL 5-dimension 5-level Questionnaire Index Utility Scores During the Peri-Diagnostic PeriodFrom inclusion into the study to end of the study (up to 18 months)

The EQ-5D-5L questionnaire is a self-reported health status questionnaire that assesses mobility, self-care, usual activities, pain/discomfort, and anxiety/depression. A unique health state is defined for each participant by combining the levels from each of the 5 dimensions. Each state is referred to in terms of a 5 digit code, e.g. state 11111 indicates no problems on any of the 5 dimensions, while state 12345 indicates no problems with mobility, slight problems with washing or dressing, moderate problems with usual activities, severe pain or discomfort and extreme anxiety or depression. From these 5 answers an index value is derived between 0 and 1, with a higher value corresponding with a better Quality of Life.

Cough, Urge to Cough, and Fatigue Visual Analogue Scales (VAS) Score During the Peri-Diagnostic PeriodFrom inclusion into the study to end of the study (up to 18 months)

The VAS are 100 mm scales on which participants indicate the severity of their cough, the urge to cough and their fatigue. In the VAS, participants had to record their health state on a scale ranging from 0 (best imaginable health state) to 100 (worst imaginable health state). A mean of this health state was recorded for participant analyzed in this outcome measure. The peri-diagnostic period covered the pre-diagnostic period (from inclusion into the study (enrollment visit), participants were followed for a maximum of 12 months in the pre-diagnostic assessment period) and the post-diagnostic period (from diagnosis to the start of drug treatment within 6 months or a maximum of 6 months if no drug treatment was prescribed).

Percentage of Participants With Non-Elective HospitalizationsFrom inclusion into the study to end of the study (up to 18 months)

The number of participants requiring the hospitalization were counted during pre- and post-diagnosis period. Investigator-reported acute exacerbations, deaths, and events related to study assessments were only collected in the clinical database. As the study had no protocol mandated IMP or treatment requirements, any untoward occurrences such as AEs and SAEs were not collected.

Percentage of Participants With Investigator-Reported Acute ExacerbationsFrom inclusion into the study to end of the study (up to 18 months)

Acute exacerbation was defined as per Collard et al. 2016. Investigator-reported acute exacerbations, deaths, and events related to study assessments were only collected in the clinical database. As the study had no protocol mandated IMP or treatment requirements, any untoward occurrences such as AEs and SAEs were not collected.

Percentage of Participants Who Died Due to Any CauseFrom inclusion into the study to end of the study (up to 18 months)

The number of participants, who died due to any cause, were counted during pre- and post-diagnosis period.

Trial Locations

Locations (37)

A.O.U. S. Luigi Gonzaga; Interstiziopatie Malattie Rare Polmone

🇮🇹

Orbassano (to), Piemonte, Italy

Hopital Cote De Nacre; Pneumologie

🇫🇷

Caen, France

Hopital de Pontchaillou; Service de Pneumologie

🇫🇷

Rennes, France

Hopital Calmette; Pneumologie

🇫🇷

Lille, France

A.O.U. Sassari; Dip. Medicina Clinica e Sperimentale

🇮🇹

Sassari, Sardegna, Italy

Ch De Granville; Pneumologie

🇫🇷

Granville, France

Clinique de pneumologie et du sommeil de Lanaudiere

🇨🇦

Saint-Charles-Borromee, Quebec, Canada

Kingston Health Sciences Centre; research Kingston General Health Research Institute

🇨🇦

Kingston, Ontario, Canada

Erasmus Medisch Centrum

🇳🇱

Rotterdam, Netherlands

Ospedale Policlinico S. Matteo; S.C. Pneumologia

🇮🇹

Pavia, Lombardia, Italy

Azienda Ospedaliero-Universitaria Careggi; SOD Pneumologia e Fisiopatologia Toracico Polmonare

🇮🇹

Firenze, Toscana, Italy

A.O. Universitaria Policlinico Di Modena; DIP. Malattie Dell'apparato Respiratorio

🇮🇹

Modena, Emilia-Romagna, Italy

SBEI HPE "The First St.Petersburg State Medical University n.a. acad. I.P.Pavlova"of MoH of RF

🇷🇺

Sankt-peterburg, Leningrad, Russian Federation

Az. Osp. Univ. P. Giaccone; UOC di Pneumologia

🇮🇹

Palermo, Sicilia, Italy

Isala

🇳🇱

Zwolle, Netherlands

SBIH at Vladimir Region "Regional Clinical Hospital"

🇷🇺

Vladimir, Russian Federation

Budget Institution of Healthcare of Voronezh Region "Voronezh Regional Clinical Hospital #1"

🇷🇺

Voronezh, Russian Federation

Hopital Tenon;Pneumologie

🇫🇷

Paris, France

Hopital Arnaud De Villeneuve; Pneumologie A

🇫🇷

Montpellier, France

Ch Bretagne Atlantique; Pneumologie-Oncologie

🇫🇷

Vannes, France

Municipal Healthcare Institution City Clinical Hospital #3 named after M.A. Podgorbunskogo

🇷🇺

Kemerovo, Russian Federation

Dr. Syed Anees Medicine Profession Corporation

🇨🇦

Windsor, Ontario, Canada

Centre Pneumologie Et Allergie

🇫🇷

Perpignan, France

Hopital Louis Pradel; Pneumologie

🇫🇷

Bron, France

Azienda Ospedaliera dei Colli - P. Monaldi; Dip. Medicina Clinica e Chirurgia

🇮🇹

Napoli, Campania, Italy

P.O. Clinicizz. 'SS. Annunziata'; U.O.C. Pneumologia

🇮🇹

Chieti, Abruzzo, Italy

ASST Rhodense; U.O.C. Pneumologia

🇮🇹

Garbagnate Milanese (MI), Lombardia, Italy

Admiraal de Ruijter Ziekenhuis

🇳🇱

Goes, Netherlands

SBI at Rostov Region "Regional Clinical Hospital #2"

🇷🇺

Rostov-na-donu, Rostov, Russian Federation

Pulmonologii NII FMBA of Russia

🇷🇺

Moscow, Moskovskaja Oblast, Russian Federation

SBIH "Chelyabinsk Regional Clinical Hospital"

🇷🇺

Chelyabinsk, Sverdlovsk, Russian Federation

Cork University Hospital

🇮🇪

Cork, Ireland

Policlinico A. Gemelli e C.I.C.; Polo Scienze Cardiovascolari e Toraciche - UOC Pneumologia

🇮🇹

Roma, Lazio, Italy

St James's Hospital

🇮🇪

Dublin, Ireland

St. Clare'S Mercy Hospital

🇨🇦

St. John's, Newfoundland and Labrador, Canada

Az. Osp.Univ.San Martino; Pneumologia

🇮🇹

Genova, Liguria, Italy

Pres. Osp. Unico ZT9 - Ospedale Generale Provinciale; U.O.C. Pneumologia

🇮🇹

Macerata, Marche, Italy

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