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Pilot Study of Pirfenidone in Pulmonary Fibrosis With Anti-myeloperoxydase Antibodies
Study Purpose
The purpose of this study is to determine wether pirfenidone is safe and effective in the
treatment of pulmonary fibrosis with anti-myeloperoxydase (MPO) antibodies or pulmonary
fibrosis with anti-MPO associated vasculitis.
Recruitment Criteria
Accepts Healthy Volunteers
Healthy volunteers are participants who do not have a disease or condition, or related conditions or symptoms
No
Study Type
An interventional clinical study is where participants are assigned to receive one or more interventions (or no intervention) so that researchers can evaluate the effects of the interventions on biomedical or health-related outcomes.
An observational clinical study is where participants identified as belonging to study groups are assessed for biomedical or health outcomes.
Searching Both is inclusive of interventional and observational studies.
Interventional
Eligible Ages
18 Years and Over
Gender
All
More Inclusion & Exclusion Criteria
Inclusion Criteria:
- Age > 18 years.
- Presence of anti-MPO antibody (ELISA) at inclusion or during pulmonary fibrosis
follow-up and/or diagnosis of anti-MPO associated vasculitis according to the 2012
Revised International Chapel Hill Consensus Conference definitions.
- Definite or possible Usual Interstitial Pneumonia or Non Specific Interstitial
Pneumonia based on high-resolution computed tomography.
- Presence of pulmonary fibrosis, defined as a range of 50 to 90% of the %FVC and a
range of 30 to 90% of the %DLCO.
- Pulmonary fibrosis refractory (according to the investigator's judgment) to a
conventional regimen used for anti-MPO associated vasculitis when a treatment against
vasculitis has been used.
- Have the ability to understand the requirements of the study, provide written informed
consent (including consent for the use and disclosure of research-related health
information) and comply with the study protocol procedures (including required study
visits)
- Have affiliation with a mode of social security (profit our being entitled).
Exclusion Criteria:
- Other type of systemic vasculitis;
- Active vasculitis defined by Birmingham Vasculitis Activity Score >3 (BVAS) ;
- Contraindication to Pirfenidone;
- Unable to perform pulmonary function test (PFT);
- Pregnancy or lactation.
Women of childbearing capacity are required to have a negative
serum pregnancy test before treatment and must agree to maintain highly effective
contraception by practicing abstinence or by using an effective method of birth
control from the date of consent through the end of the study : implants of
levonorgestrel; injectable progesterone; any intrauterine device (IUD) with a
documented failure rate of less than 1% per year; oral contraceptives (either combined
or progesterone only); double barrier method (condom, cervical cap or diaphragm with
spermicidal agent); transdermal contraceptive patch; male partner who is sterile prior
to the female subject's entry into the study and is the sole sexual partner for the
female subject;
- Any of the following liver function test criteria above specified limits: total
bilirubin above 1,5 times the upper limit of normal (ULN), excluding patients with
Gilbert's syndrome; aspartate (AST)/Glutamate Oxaloacétique Transaminase (SGOT) or
alanine aminotransferase (ALT)/Glutamate Pyruvate Transaminase (SGPT), (AST/SGOT or
ALT/SGPT) >3 × ULN; alkaline phosphatase >2.5 × ULN;
- Creatinine clearance (CrCl<30) mL/min, calculated using the Cockcroft-Gault formula at
screening.
- Current treatment with Nintedanib or past treatment with Nintedanib in the last 12
months;
- Current treatment with Fluvoxamine or past treatment with Fluvoxamine in the last 28
days before screening.
- Prior use of Pirfenidone or known hypersensitivity to any of the components of study
treatment;
- Expected to receive a lung transplant within 1 year from randomization or, on a lung
transplant waiting list at randomization;
- Associated connective tissue disease (such as systemic sclerosis).
;
- Electrocardiogram (ECG), with a heart-rate-corrected QT interval (corrected using
Fridericia's formula, QTcF) ≥ 500 ms at Screening, or a family or personal history of
long QT syndrome;
- Treatment with Cyclophosphamide in the last 3 months;
- Current smoking or past smoking in the last 3 months.
Trial Details
Trial ID:
This trial id was obtained from ClinicalTrials.gov, a service of the U.S. National Institutes of Health, providing information on publicly and privately supported clinical studies of human participants with locations in all 50 States and in 196 countries.
Phase 1: Studies that emphasize safety and how the drug is metabolized and excreted in humans.
Phase 2: Studies that gather preliminary data on effectiveness (whether the drug works in people who have a certain disease or condition) and additional safety data.
Phase 3: Studies that gather more information about safety and effectiveness by studying different populations and different dosages and by using the drug in combination with other drugs.
Phase 4: Studies occurring after FDA has approved a drug for marketing, efficacy, or optimal use.
Phase 2
Lead Sponsor
The sponsor is the organization or person who oversees the clinical study and is responsible for analyzing the study data.
Assistance Publique - Hôpitaux de Paris
Principal Investigator
The person who is responsible for the scientific and technical direction of the entire clinical study.
Jonathan London, MD
Principal Investigator Affiliation
Cochin Hospital
Agency Class
Category of organization(s) involved as sponsor (and collaborator) supporting the trial.
Other, Industry
Overall Status
Recruiting
Countries
France
Conditions
The disease, disorder, syndrome, illness, or injury that is being studied.
Pulmonary Fibrosis
Additional Details
Pulmonary fibrosis can be associated with Anti-Neutrophil Cytoplasmic Antibody (ANCA)
directed against MPO or with anti-MPO associated vasculitis, leading to increased disability
and poor prognosis. The pathophysiology of this association remains unclear. Conventional
therapies used for the treatment of vasculitis manifestations are often disappointing for the
treatment of pulmonary fibrosis. The main cause of death in patients with anti-MPO ANCA
associated vasculitis and associated pulmonary fibrosis is the progression of pulmonary
fibrosis. No treatment has demonstrated efficacy to stabilize or improve pulmonary fibrosis
associated with anti-MPO associated vasculitis.
Previous studies showed that Pirfenidone improves survival and pulmonary function in patients
with idiopathic pulmonary fibrosis (IPF) and that Pirfenidone treatment is safe and well
tolerated in IPF. Patients with anti-MPO associated vasculitis (or anti-MPO antibodies
without vasculitis) and associated pulmonary fibrosis might benefit from the use of
Pirfenidone. However, the efficacy and safety of pirfenidone in patients with anti-MPO
associated vasculitis and associated pulmonary fibrosis has not been evaluated. This study
was designed to assess the efficacy and safety of pirfenidone in patients with pulmonary
fibrosis and anti-MPO ANCA associated vasculitis or anti-MPO antibodies without vasculitis.
Arms & Interventions
Arms
Experimental: Pirfenidone
All patients will receive Pirfenidone
Interventions
Drug: - Pirfenidone
Pirfenidone at a dose of 2403 mg/day for 50 weeks, after a 2 weeks period of titration (801 mg/day for one week then 1602 mg/day for one week).
Contact a Trial Team
If you are interested in learning more about this trial, find the trial site nearest to your location and contact the site coordinator via email or phone. We also strongly recommend that you consult with your healthcare provider about the trials that may interest you and refer to our terms of service below.