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IPF Research

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:

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.
     
PHASE III CLINICAL TRIALS
  • bosentan (Tracleer®) – Manufactured by Actelion Pharmaceuticals, Ltd.: A Multicenter, Double-Blind, Randomized, Placebo-Controlled Phase III clinical trial titled “BUILD-3” - Bosentan Use in Interstitial Lung Disease - evaluating the safety and efficacy of oral bosentan in 390 patients suffering from idiopathic pulmonary fibrosis (IPF) in the US, Canada and 16 other countries around the world.. Enrollment in this event-driven study is ongoing and study results are expected in late 2009. Primary Outcomes: Time to occurrence of disease worsening or death up to end of study. Secondary Outcomes: Proportion of patients who experienced either disease worsening or death at 1 year. For inclusion & exclusion criteria and participating sites please visit www.build-3.com or call Actelion Medical Affairs at 866-228-3546.

  • 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.clinicatrials.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.
     
PHASE II CLINICAL TRIALS
  • Gleevec (imatinib mesylate) - Manufactured by Novartis: A phase II, randomized, double blind, placebo-controlled study of the clinical effects of Gleevec (imatinib mesylate) administered orally to patients with idiopathic pulmonary fibrosis (IPF). The primary endpoint is progression of IPF as defined as a greater than 10% decline in the forced vital capacity or death. Study subjects with IPF will be treated initially with Gleevec (imatinib mesylate) 600 mg orally once per day versus placebo for a period of up to 2 years. Sponsor: Novartis, Inc. (www.novartis.com) Patients will be randomized into the trial using an IVR (Interactive Voice Randomization) system. One Hundred patients will be enrolled into the trial in total (50 active drug and 50 placebo). The study population consists of male and female outpatients with IPF ages 20 to 79. Pregnant or breast feeding subjects are not permitted in the trial. For more information on this clinical trial, please contact Chodie Gabor, RN at Tulane University egabor@tulane.edu or 504-585-6966) or Kathy Mieras, RN at the Mayo Clinic ( mieras.kathleen@mayo.edu or 507-284-9187)

  • 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.clinicatrials.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.

  • Viagra (Sildenafil) - Manufactured by Pfizer, Inc.: A Phase II clinical trial of vasodilator therapy (sildenafil) in IPF patients. The objective of this clinical trial is to assess the possible therapeutic benefit of a vasodilator, sildenafil, on exercise tolerance and dyspnea in IPF patients and to assess changes from baseline in pre- and post-exercise markers of oxidant stress in sildenafil and placebo control groups. It is open to all patients, not just veterans. The investigators are seeking patients with IPF diagnosed clinically. This clinical trial began enrolling patients on July 1, 2006. For additional information or to participate, please contact Robert M. Jackson, MD at 305-575-3548 or by email at rjackson2@med.miami.edu. The trial is funded by a grant from the Veterans Administration Research Service.
     
PHASE I CLINICAL TRIALS
  • GC1008 - Manufactured by Genzyme Corp/Cambridge Antibody Technologies A Phase I clinical trial investigating the safety of GC1008, a fully human monoclonal antibody that neutralizes transforming growth factor beta (TGFb). The trial is currently enrolling patients at several medical centers in the United States. Further information about this clinical trial, including a list of enrolling centers and inclusion and exclusion criteria, may be found at http://www.clinicaltrials.gov/ct/show/NCT00125385

    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.clinicatrials.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. 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.

 

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