The Effect of Pirfenidone on Cough in Patients With Idiopathic Pulmonary Fibrosis (Cough-IPF)

Observational Study of the Effect of Pirfenidone on Cough in Patients With Idiopathic Pulmonary Fibrosis

In this study we evaluate the effect of Pirfenidone on cough and quality of life in patients with idiopathic pulmonary fibrosis (IPF) that are treated with Pirfenidone in daily practice. The hypothesis is that Pirfenidone will decrease cough and increase quality of life.

Study Overview

• Status: Completed
• Conditions: Idiopathic Pulmonary Fibrosis
• Intervention / Treatment: Other: Cough monitor

Detailed Description

Rationale: Idiopathic Pulmonary Fibrosis (IPF) is a progressive fibrotic lung disease of unknown cause with a median survival of 3-5 years. No curative treatment exists, though in 2011 Pirfenidone was approved for the treatment of IPF as it appeared to slow down the decline in lung function. In patients with IPF, the most common symptoms are cough and breathlessness. Cough is not only a major distressing and disabling symptom but also an independent predictor of disease progression and death in IPF. Recent preliminary data suggest a possible effect of Pirfenidone on cough.

Objective: In this study we want to objectively measure the effect of Pirfenidone on cough in patients with IPF that are treated with Pirfenidone in daily practice .

Study design: This is a prospective, observational, international multicenter study.

Intervention: Objective 24-hour cough frequency will be recorded using the Leicester Cough Monitor (LCM), a validated ambulatory cough monitoring system, prior to starting with Pirfenidone treatment. The cough recording will be repeated at 4 weeks and at 12 weeks during treatment with Pirfenidone. At the days of cough recording, patients will be asked to fill in questionnaires related to cough and to quality of life. Patient will be treated according to normal clinical practice at their Physician's discretion.

Main study parameters/endpoints: The primary endpoint is change in cough frequency measured by the Leicester cough monitor at week 12 compared to baseline. Secondary endpoints look at the relationships between cough, change in cough, quality of life and clinical parameters.

• Study Type: Observational
• Enrollment (Actual): 43

Contacts and Locations

Study Locations

• France
  • Lyon, France
    • University Lyon 1, Louis Pradel hospital, Lyon. FranceService de pneumologie, hôpital Louis Pradel
• Italy
  • Catania, Italy
    • Regional Centre for Rare Lung Disease University of Catania.
• Netherlands
  • Rotterdam, Netherlands, 3015 CE
    • Erasmus MC Rotterdam, Dep. of Pulmonology

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 40 years to 85 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

Patients with IPF about to start on Pirfenidone according to regular practice in the participating University Hospitals

Description

Inclusion Criteria:

• Diagnosis of IPF according to American Thoracic Society (ATS) / European Respiratory Society (ERS) criteria (5), definite and probable patients will be eligible
• Written informed consent
• Daily cough related to IPF (exclusion of other causes) present > 8 weeks
• cough score on visual analogue scale of ≥ 40 mm.
• Carbon monoxide transfer capacity corrected for hemoglobin (TLCOc) ≥ 30% and Forced Vital Capacity (FVC) ≥ 50%
• Pirfenidone therapy about to be initiated
• if a history positive for Gastro Esophageal Reflux (GER), using proton pump inhibitor (PPI) > 4 weeks

Exclusion Criteria:

• Opiates, antitussive medication, antihistamines, steroids > equivalent of 10 mg prednisone or N-acetylcysteine (NAC) within two weeks before study
• Change of steroid < 10 mg, inhalation steroids within 2 weeks of the study - History of bronchial hyper responsiveness or asthma or relevant airway obstruction (FEV1/FVC < 0.7)
• within 6 weeks of the start signs of respiratory tract infection, change of sputum production and fever.

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Prospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Cough IPF; Male and female with idiopathic pulmonary fibrosis and cough and about to start on Pirfenidone according to regular practice will be asked to wear a cough monitor 24 hours before starting Pirfenidone and twice 24 hours while using Pirfenidone. Patients will also be asked to fill in questionnaires about quality of life and cough.	Other: Cough monitor; questionnaires about cough and quality of life; Other Names: cough monitoring; Leicester Cough Monitor

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Change in cough frequency measured by cough recorder at week 12 compared to baseline	12 weeks

Secondary Outcome Measures

Outcome Measure	Time Frame
Impact of cough on quality of life	12 weeks
Change in cough frequency measured by cough recorder at 4 weeks compared to baseline	4 weeks

Other Outcome Measures

Outcome Measure	Time Frame
Change in Leicester Cough Questionnaire at week 12 compared to baseline	12 weeks
Change in Visual Analogue Score at week 12 compared to baseline	12 weeks
Change in cough frequency in relation to FVC	12 weeks
Clinical characteristics predictive of cough response	12 weeks
Impact of cough on anxiety and depression	12 weeks
Change in cough frequency in relation to TLCOc	12 weeks

Collaborators and Investigators

• Sponsor: Erasmus Medical Center
• Collaborators: King's College Hospital NHS Trust; Royal Brompton & Harefield NHS Foundation Trust; University of Catania; University of Lyon
• Investigators: Study Chair: M. S. Wijsenbeek, Dr., Erasmus Medical Centre Rotterdam, the Netherlands; Principal Investigator: C. Vancheri, Prof., University of Catania, Italy; Principal Investigator: V. Cottin, Prof., Louis Pradel hospital, Lyon, France; Principal Investigator: S Birring, Dr., Department of Respiratory Medicine,King's College Hospital.Denmark Hill, London; Principal Investigator: A Russell, Royal Brompton & Harefield NHS Foundation Trust; Principal Investigator: E Renzoni, Dr., Royal Brompton & Harefield NHS Foundation Trust

Study record dates

Study Major Dates

• Study Start: December 1, 2013
• Primary Completion (Actual): December 1, 2016
• Study Completion (Actual): December 1, 2016

Study Registration Dates

• First Submitted: December 1, 2013
• First Submitted That Met QC Criteria: December 8, 2013
• First Posted (Estimate): December 11, 2013

Study Record Updates

• Last Update Posted (Estimate): December 23, 2016
• Last Update Submitted That Met QC Criteria: December 21, 2016
• Last Verified: December 1, 2016

More Information

Terms related to this study

• Keywords: quality of life; idiopathic pulmonary fibrosis; cough
• Additional Relevant MeSH Terms: Pathologic Processes; Respiratory Tract Diseases; Lung Diseases; Fibrosis; Pulmonary Fibrosis; Idiopathic Pulmonary Fibrosis
• Other Study ID Numbers: NL44729.078.13