IPFnet PANTHER study revised, Click Here for urgent information.

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

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:


ACTIVE PHASE III CLINICAL TRIALS

• BIBF 1120 - Manufactured by Boehringer Ingelheim: A Phase III, double-blind trial in patients with PF to investigate the effect of Oral BIBF 1120, 150 mg Twice Daily, on Annual Forced Vital Capacity Decline. A primary outcome measure will be the annual rate of decline in in FVC over 32 weeks. For further information on this study, please visit www.clinicaltrials.gov.
  
  
• Cyclosporine Inhalation Solution (CIS) - Manufactured by APT Pharmaceuticals -  (For lung transplant recipients only):  A Phase III multi-center, randomized, controlled study to demonstrate the efficacy and safety of cyclosporine inhalation solution (CIS) in Improving Bronchiolitis Obliterans Syndrome-Free Survival Following Lung Transplantation.  The study seeks to demonstrate the efficacy and safety of CIS in improving survival, and preventing BOS when given prophylactically to lung transplant recipients in addition to their standard immunosuppressive regimen.  The study is no longer recruiting patients. Click here for complete clinical trial information.
  
  
• Pirfenidone - Manufactured by Intermune: A Phase III, double-blind, placebo-controlled trial to evaluate the treatment effect of pirfenidone on change in a lung function measure (percent predicted forced vital capacity, or %FVC) and to evaluate the safety of treatment with pirfenidone compared with placebo. For a complete description of the study, including participating sites in the United States and internationally, please visit www.Ascendtrial.com or www.clinicaltrials.gov
  
  
• 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.
  
  

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.  Patients will be in the study for about 74 weeks.  For a complete description of the study, including participating sites in the United States and Internationally, please visit www.clinicaltrials.gov and search for "CNTO 888".  Please consult your pulmonologist, or contact info1@veritasmedicine.com to learn more.
  
  
• FG-3019  -- Manufactured by FibroGen, Inc.  phase 2 study of FG-3019 (therapeutic antibody against connective tissue growth factor) for patients with IPF.  The study is a Phase 2a, Open-Label, Single Arm Study to Evaluate the Safety, Tolerability, and Efficacy of FG-3019 in Subjects With Idiopathic Pulmonary Fibrosis.  Further information on the study including inclusion/exclusion criteria is available at www.clinicaltrials.gov and directly at http://www.clinicaltrials.gov/ct2/show/NCT01262001?term=FG-3019&rank=6. Please refer to this study by its ClinicalTrials.gov identifier: NCT01262001.  The study is sponsored by FibroGen.  Please call Loredie Lugos, RN, BSN at 415-978-1353 or email at llugos@fibrogen.com for more information. 
  
  
• 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. TEMPORARILY ON HOLD
  
  
• STX-100 - (Stromedix, Inc.): STX-100 is a monoclonal antibody being developed for the treatment of idiopathic pulmonary fibrosis (IPF). This multi-center, randomized, double-blind, placebo-controlled study will evaluate the safety, tolerability, pharmacokinetics, and pharmacodynamics of STX-100 in patients with IPF. The study began recruitment in Q4 '11 and will enroll patients at sites across the United States. A complete description of the study is available at ClinicalTrials.gov
  (http://clinicaltrial.gov/ct2/show/NCT01371305). For more information please contact Emily Graham at Emily.Graham@covance.com (Phone: 734-369-3873) or Kris Hermanson at Kris.Hermanson@covance.com (Phone: 608-310-4086).
  
  

ACTIVE PHASE I CLINICAL TRIALS

• GS 6624 (formerly AB0024) - Developed by Gilead Sciences, Inc. Phase 1 drug candidate (humanized monoclonal antibody targeting the human LOXL2 protein) for patients with IPF.  An ongoing Phase 1 study is designed to evaluate the safety, tolerability, pharmacokinetics and pharmacodynamics of GS 6624 in adult patients with IPF. Further information on the study including inclusion/exclusion criteria is available at www.clinicaltrials.gov and directly at http://www.clinicaltrials.gov/ct2/show/NCT01242189?term=AB0024&rank=1. Please refer to this study by its ClinicalTrials.gov identifier: NCT01242189.
   
• IW001 - Manufactured by Immuneworks. A phase I study of the safety and tolerability of 3 doses of IW001 per day in patients with IPF over a 24 week treatment period. To explore the biologic effects of IW001 on T-cell and B-cell reactivity. This study will also explore relationships between Collagen V reactivity and clinical measures of lung function in patients with IPF. This study is now recruiting. For further information about the trial and the specific sites, please go to: http://immuneworks.com/ipf-clinical-trials, or trials http://www.clinicaltrials.gov/ct2/show/NCT01199887?term=pulmonary+fibrosis+immuneworks&rank=1.
   
• PRM-151 - Manufactured by Promedior, Inc. A phase 1b study of the safety, tolerability, pharmacokinetics, and pharmacodynamics of IV PRM-151 in patients with idiopathic pulmonary fibrosis has initiated in the US and the Netherlands. This study is now recruiting patients. PRM-151 is being developed for potential therapeutic uses to prevent, treat, and reduce fibrosis in a number of disease settings. For further information about this trial including inclusion/exclusion criteria or if you have potential interest in participating, please go to http://www.clinicaltrials.gov/ct2/show/NCT01254409?term=PRM-151&rank=2, www.promedior.com, or e-mail clinicaltrials@promedior.com.

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.

Familial Pulmonary Fibrosis Study - National Jewish Health
The purpose of this study is to investigate inherited genetic factors that play a role in the development of familial pulmonary fibrosis and to identify a group of genes that predispose individuals to develop pulmonary fibrosis.  This investigation seeks to identify a group of genetic loci that play a role in the development of familial interstitial pneumonia (FIP or FPF), where 2 or more cases of IIP are seen within a family.  To be eligible for this study, patients must have at least 2 members of the family diagnosed with Idiopathic Interstitial Pneumonia (IIP).  The patient may be included in this number and does not have to live near the centers affiliated with the study.   If you wish to join this study, please go to http://www.clinicaltrials.gov/ct2/show/study/NCT01088217?term=pulmonary fibrosis&rank=3 for further information or contact Janet Talbert at National Jewish Medical Center (1-800-423-8891 X 1022).  This study is ongoing.

Peripheral Blood Biomarkers in Idiopathic Interstitial Pneumonias - Duke University Medical Center
The current diagnostic tools for IIP are inadequate. In addition to inaccurate diagnosis, they are very costly, and often result in delayed diagnosis and treatment. The challenge(s) we intend to address in this proposal is to improve the accurate and early diagnosis of idiopathic interstitial lung pneumonia (IIP), and to improve the ability to differentiate the subtypes of idiopathic interstitial pneumonias (IIPs) by developing peripheral blood biomarkers.  The Broad Challenge Area addressed in this proposal is Biomarker Discovery and Validation, and the Specific Challenge Topic is 03-HL-101 (Identify and validate clinically relevant, quantifiable biomarkers of diagnostic and therapeutic responses for blood, vascular, cardiac, and respiratory tract dysfunction).  For further information please go to   http://www.clinicaltrials.gov/ct2/show/NCT01151527?term=pulmonary fibrosis&intr=pulmonary fibrosis blood biomarkers&rank=1 or contact Raven Kidd at Duke University raven.kidd@duke.edu
This study is ongoing.

IPFnet
The IPFnet is a national clinical research trial program of vital interest to the PF community.  If you or someone in your family would like to participate in an IPFnet trial, please contact the appropriate center for you using the contact names and numbers listed on www.ipfnet.org or www.coalitionforpfor.org.

The network is currently recruiting patients for two important studies:

• The PANTHER-IPF: Prednisone, Azathioprine, and N-Acetylcysteine: : PFnet PANTHER study revised, Click Here for urgent information.
  
  
• The ACE-IPF: Anticoagulant Effectiveness in Idiopathic Pulmonary Fibrosis trial is investigating whether treatment with warfarin improves clinical outcome in subjects with IPF.  The study is a 2-arm study with a 1 to 1 randomization to Warfarin or placebo.  Patients may participate whether they are near a study center or elsewhere in the country. This trial is available to all subjects with IPF between 35-80 years of age, regardless of time of diagnosis who meet study specific enrollment criteria.