Coalition for Pulmonary Fibrosis (CPF)

Researchers are developing and testing a variety of new ways to treat IPF. These approaches target the various steps in the disease process. Therapies under investigation include:

Antifibrotic or antifibrogenic agents (such as interferon and certain blood-pressure–lowering medications) to suppress the scarring process
Antioxidants (such as N-acetylcysteine) to prevent damage to lung tissue
Endothelin antagonists (such as Bosentan)
Monoclonal antibodies to inhibit “bad” cytokines (protein growth factors, such as TGF-beta, TNF-Alpha, or CTGF, that activate inflammation)

Clinical Trials

Clinical trials are being conducted to better understand how pulmonary fibrosis develops and to advance the treatment of IPF. In a clinical trial, healthcare providers use a new treatment for patients to determine if it is safe and effective. These studies investigate the effectiveness of vaccines, new drugs, or new ways of using known treatments. A new drug, for example, might be tested to see if patients live longer than they would without the drug. Not all patients in a clinical trial will receive the new treatment-often these studies compare patients given the new treatment with patients given a placebo (sugar pill) or standard treatment. For more information on clinical trials, please consult your physician.

Please note that you do not necessarily need to live in the same city as the principal investigator (the person managing the clinical trial) in order to participate. Active clinical trials include:

As with any course of care, please consult your physician to determine what current treatment options, including participation in clinical trials, may be appropriate for you.

ACTIVE PHASE IV CLINICAL TRIALS

Viagra (sildenafil) - Manufactured by Pfizer, Inc.: A Phase IV, randomized, placebo-controlled safety and efficacy study of Sildenafil For The Treatment of Pulmonary Arterial Hypertension Secondary to Idiopathic Pulmonary Fibrosis: A Pilot Study is to determine if sildenafil can improve morbidity and mortality in the peri-LT setting in both IPF cohorts with either resting or exercise PAH. Please visit www.clinicaltrials.gov and search "IPF" for a complete description of this trial, including inclusion/exclusion criteria. The study is sponsored by UCLA (Los Angeles, Calif.). Please call 310-825-5635 for more information.

ACTIVE PHASE III CLINICAL TRIALS

bosentan (Manufactured by Actelion, Ltd.) – A phase III, prospective, multicenter, randomized, double-blind, parallel group, placebo-controlled clinical trial to evaluate the effects of bosentan on morbidity and mortality in patients with IPF. The study is named Bosentan Use in Interstitial Lung Disease (BUILD-3). The primary objective is to demonstrate that bosentan delays disease worsening or death in patients with IPF. The study has expanded enrollment from 390 to 600, and Actelion expects to be fully enrolled by the end of 2008. For complete information including inclusion//exclusion criteria, and a listing of participating centers, please visit www.clinicaltrials.gov and search “BUILD3” in the search box, or contact Actelion’s medical affairs department at (650) 624-6900, to learn more.
bosentan (Manufactured by Actelion, Ltd.) – An Open-Label Extension Study in Patients with IPF Who Completed Actelion’s BUILD-3 Phase III Clinical Trial. This clinical trial is only recruiting patients with IPF who have previously completed Actelion’s BUILD-3 Phase III Clinical Trial. The primary goal of the extension study is to asses the long term safety and tolerability of bosentan in patients with IPF. Patients who have completed BUILD-3 should contact their participating site for more information, or contact Actelion’s medical affairs department at (650) 624-6900, to learn more.
Thalomid (Thalidomide) - Manufactured by Celgene: A Phase III, double-blind, placebo-controlled safety and efficacy study investigating the Treatment of Chronic Cough in Idiopathic Pulmonary Fibrosis with Thalidomide. Please visit www.clinicaltrials.gov and search "IPF" for a complete description of this trial, including inclusion/exclusion criteria. The study is sponsored by Johns Hopkins Medical Center (Baltimore,MD). Please call 410-550-4764 for more information.
Pirfenidone (Manufactured by InterMune, Inc.) - An Open-Label Extension Study of the Long Term Safety of Pirfenidone in Patients with IPF. This clinical trial is only recruiting patients with IPF who have previously completed the CAPACITY Clinical Trials (PIPF-004 or PIPF-006). Eligible patients must complete the Final Follow-Up Visit for the CAPACITY studies and meet all of the eligibility criteria noted in the protocol. This clinical trial will escalate the patient’s dose of Pirfenidone to measure adverse events and changes in forced vital capacity (FVC). Patients who have completed PIPF-004 or PIPF-006 should contact their participating site for more information, or contact InterMune’s medical affairs department at (415) 466-2200, to learn more.

ACTIVE PHASE II CLINICAL TRIALS

CNTO 888 (Manufactured by Centocor, Inc.): A Phase 2, Multicenter, Multinational, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group, Dose-Ranging Study Evaluating the Efficacy and Safety of CNTO 888 Administered Intravenously. The primary objective is to determine the efficacy (as measured by pulmonary function) and safety of CNTO 888 in patients with IPF. The secondary outcome measures are to assess the effect of CNTO 888 on measures of disease progression, patient reported outcomes, functional capacity and health-related quality of life, and to assess the pharmacokinetics and pharmacodynamics of CNTO 888 in IPF. The study began recruiting patients in October, 2008, with a goal of recruiting 120 patients. U.S. Sites are located in Phoenix AZ, Wichita KS, Colchester VT, and Philadelphia PA. Patients will be in the study for about 74 weeks. For a complete description of inclusion/exclusion criteria, please visit www.clinicaltrials.gov and search for “CNTO 888”. Please consult your pulmonologist, or contact info1@veritasmedicane.com to learn more.
Interferon alpha (Amarillo Biosciences): A Phase II, randomized, double-blind, placebo-controlled trial to determine whether interferon-alpha, delivered in low doses via orally dissolving lozenges given 3 times per day for 4 weeks, can reduce the frequency and severity of chronic cough in patients with COPD or IPF. Cough frequency will be assessed via 24-hour digital audio recordings made prior to entry, and at weeks 2 and 4 of treatment. Patients will also complete questionnaires regarding cough frequency, duration and intensity, QOL, dyspnea, and antitussive medication usage weekly during treatment. The study is seeking 80 patients however it is only recruiting patients at Texas Tech University (Lubbock, TX). For a complete description of inclusion/exclusion criteria, please visit www.clinicaltrials.gov and search for “Interferon-Alpha”. Please contact study coordinator Betty Lonis, RN at betty.lonis@ttuhsc.edu or 806-743-2227, ext. 249 to learn more.
Viagra (sildenafil) - Manufactured by Pfizer, Inc.: A Phase II, double-blind, placebo-controlled safety and efficacy study is to determine if sildenafil, will improve exercise performance and quality of life in patients with IPF. The study is called STEP-IPF (Sildenafil Trial of Exercise Performance in Idiopathic Pulmonary Fibrosis). Interested patients will have screening procedures, which include breathing tests, x-rays, heart tests, six-minute walk test, and blood tests. Patients who are enrolled will have several follow-up study visits for 6 months to see the doctor. Breathing tests, six-minute walk tests, questionnaires, and blood tests will also be done at these follow-up visits. Please visit www.clinicaltrials.gov and search "IPF" for a complete description of this trial, including inclusion/exclusion criteria, as well as a complete list of participating medical centers.
QAX576 (Manufactured by Novartis, Inc.) - An Open-Label, Multi-Center Study, With a Single Intravenous Dose of QAX576 to Determine IL-13 Production in Patients with IPF. The purpose of this study is to investigate how QAX576 affects levels of interleukin 13 (IL-13) in patients with IPF. The primary outcomes will be to investigate the possibility that some IPF patients experience increased IL-13 production. Blood samples to be collected pre-dose and weekly after dosing. A second primary outcome is to investigate the hypothesis that QAX576 will neutralize IL-13 in patients with IPF. The study seeks to enroll up to 50 patients. Click here for complete clinical trial information, including inclusion/exclusion criteria, and a listing of participating centers, or contact Novartis at 862-778-8300.

ACTIVE PHASE I CLINICAL TRIALS

interferon gamma - Manufactured by InterMune, Inc.: Phase I, Non-Randomized, Open Label safety and efficacy Pilot Study of Aerosol Interferon-Gamma for Treatment of Idiopathic Pulmonary Fibrosis. The goal of the study is to determine if giving interferon-gamma as a nebulized mist directly into the lungs can affect the immune system in a way that decreases fibrosis. Please visit www.clinicaltrials.gov and search "IPF" for a complete description of this trial, including inclusion/exclusion criteria. The study is sponsored by New York University Medical Center (New York, NY). Please call 212-263-7951 for more information.

Investigational Research
The National Institutes of Health (NIH) offers an informative web page at www.clinicaltrials.gov for those interested in identifying IPF-related research at medical centers across the United States. It represents the most up-to-date research information for patients including trial sites, patient inclusion/exclusion criteria, and contact information for each trial. For further information please visit www.clinicaltrials.gov, and enter 'pulmonary fibrosis’ or ‘IPF' in the "Search Clinical Trials" area of the home page

Additional Investigational Research Links:

Please note that this is not a comprehensive list. Check back for information on new research programs or contact the departments of pulmonary and critical care medicine at the medical schools of local universities for current IPF studies.