Rare Medical News

Spotlight On

Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) is a rare, progressive illness of the respiratory system, characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue

Prevalence

1-5 / 10,000

33,100 - 165,500

US Estimated

1-5 / 10,000

51,350 - 256,750

Europe Estimated

Age of Onset

ageofonset-adult.svg

ICD-10

J84.1

Inheritance

Autosomal dominant

no.svg

Autosomal recessive

no.svg

Mitochondrial/Multigenic

rnn-multigenetic.svg

X-linked dominant

no.svg

X-linked recessive

no.svg

5 Facts you should know

FACT

1

IPF is characterized by the thickening and stiffening of lung tissue, associated with the formation of scar tissue.

FACT

2

Risk factors include cigarette smoking, acid reflux disease (GERD), certain viral infections.

FACT

3

Those in their 60s and 70s are most commonly affected.

FACT

4

Males are affected more often than females.

FACT

5

The average life expectancy following diagnosis is about four years.

Idiopathic Pulmonary Fibrosis (IPF)

IDF, Fibrosing alveolitis, cryptogenic; Familial idiopathic pulmonary fibrosis; Fibrocystic pulmonary dysplasia; Fibrosing alveolitis

What’s your rare IQ?

Which seemingly unrelated symptom is...?

Common Signs & Symptoms.

Bronchiectasis

Permanent enlargement of the airways of the lungs

Clubbing of hands and fingers

Cough

Crackles

Exertional dyspnea

Gastroesophageal reflux

Acid reflux, Acid reflux disease, Heartburn

Ground-glass opacification on pulmonary HRCT

Pulmonary fibrosis

Reticular pattern on pulmonary HRCT

Frequent ear infections

Current treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

pirfenidone(Brand name: <a href="https://www.esbriet.com/" target="_blank" rel="noopener">Esbriet</a>)

Manufactured by InterMune, Inc.
FDA-approved indication: Treatment of idiopathic pulmonary fibrosis.
National Library of Medicine Drug Information Portal

Nintedanib (Brand name: <a href="https://www.ofev.com/" target="_blank" rel="noopener">Ofev</a>)

Manufactured by Boehringer Ingelheim Pharmaceuticals, Inc.
FDA-approved indication: October 2014, nintedanib (Ofev) was approved for the treatment of idiopathic pulmonary fibrosis.
National Library of Medicine Drug Information Portal
Medline Plus Health Information

Top clinical studies

TitleDescriptionPhasesStatusConditionsInterventionsSponsor/CollaboratorsLocationsMore Information
Evaluation of Efficacy and Safety of Pamrevlumab in Patients With Idiopathic Pulmonary Fibrosis.This is a Phase 3 trial to evaluate the efficacy and safety of 30 mg/kg intravenous (IV) infusions of pamrevlumab administered every 3 weeks as compared to placebo in subjects with Idiopathic Pulmonary FibrosisPhase 3RecruitingIdiopathic Pulmonary FibrosisDrug: Pamrevlumab|Drug: PlaceboFibroGenUniversity of Alabama at Birmingham Hospital, Birmingham, Alabama, United States|Banner University Medical Center - Phoenix, Phoenix, Arizona, United States|Norton Thoracic Institute, Phoenix, Arizona, United States|Pulmonary Associates, PA - Research, Phoenix, Arizona, United States|University of Arizona, Tucson, Arizona, United States|J&L Research, Conway, Arkansas, United States|Loma Linda University Health, Loma Linda, California, United States|David Geffen School of Medicine at UCLA, Los Angeles, California, United States|UC Davis Medical Center, Sacramento, California, United States|University of California San Diego, San Diego, California, United States|Stanford University Medical Center, Stanford, California, United States|University of Colorado Denver, Aurora, Colorado, United States|National Jewish Health, Denver, Colorado, United States|Yale University School of Medicine, New Haven, Connecticut, United States|Medstar Georgetown University Hospital, Washington, District of Columbia, United States|St. Francis Medical Center, Clearwater, Florida, United States|University of Florida Pulmonary, Critical Care & Sleep Medicine Division, Gainesville, Florida, United States|University of Florida Health, Jacksonville, Jacksonville, Florida, United States|Mayo Clinic, Jacksonville, Florida, United States|Pulmonary Disease Specialist, PA d/b/a, PDS Research, Kissimmee, Florida, United States|University of Miami, Miami, Florida, United States|University of South Florida, Tampa, Florida, United States|Emory University, Atlanta, Georgia, United States|Piedmont Healthcare, Inc., Austell, Georgia, United States|Northwestern University, Chicago, Illinois, United States|Loyola University of Chicago, Maywood, Illinois, United States|Indiana University School of Medicine, Indianapolis, Indiana, United States|The University of Kansas Medical Center, Kansas City, Kansas, United States|University of Maryland, Baltimore, Baltimore, Maryland, United States|University of Michigan, Ann Arbor, Michigan, United States|Henry Ford Hospital, Detroit, Michigan, United States|University of Minnesota, Minneapolis, Minnesota, United States|The Lung Research Center, LLC, Chesterfield, Missouri, United States|Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire, United States|Albany Medical College, Albany, New York, United States|Pulmonary Health Physicians, PC, Liverpool, New York, United States|Pulmonix, LLC, Greensboro, North Carolina, United States|University of Cincinnati, Cincinnati, Ohio, United States|Ohio State University, Columbus, Ohio, United States|INTEGRIS Baptist Medical Center, Oklahoma City, Oklahoma, United States|Legacy Research Institute, Portland, Oregon, United States|The Oregon Clinic, Portland, Oregon, United States|Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania, United States|Temple University, Philadelphia, Pennsylvania, United States|Rhode Island Hospital, Providence, Rhode Island, United States|Lowcountry Lung and Critical Care, PA, Charleston, South Carolina, United States|Medical University of South Carolina, Charleston, South Carolina, United States|Tennessee Comprehensive Lung and Sleep Center, Hendersonville, Tennessee, United States|Vanderbilt University Medical Center, Nashville, Tennessee, United States|Houston Pulmonary Sleep, Allergy and Asthma Associates, Cypress, Texas, United States|Baylor Scott and White Research Institute, Dallas, Texas, United States|University of Texas Southwestern Medical Center, Dallas, Texas, United States|Baylor College of Medicine, Houston, Texas, United States|University of Texas - Houston, Houston, Texas, United States|UT Health San Antonio, San Antonio, Texas, United States|Renovatio Clinical, The Woodlands, Texas, United States|University of Utah Health, Salt Lake City, Utah, United States|The University of Vermont, Burlington, Vermont, United States|University of Washington, Seattle, Washington, United States|Medical College of Wisconsin, Milwaukee, Wisconsin, United States|Centro de Investigación Clínica Aplicada - Hospital Regional Español Bahía Blanca, Bahia Blanca, Buenos Aires, Argentina|Fundacion Respirar - Centro Médico Dra. De Salvo, Ciudad Autonoma De Buenos Aires (caba), Buenos Aires, Argentina|Centro de Investigacion Metabolica 'CINME', Ciudad Autonoma de Buenos Aires, Buenos Aires, Argentina|Consultorios Medicos. Organización del Buen Ayre. SRL, Ciudad Autonoma de Buenos Aires, Buenos Aires, Argentina|Centro Medico de Enfermedades Respiratorias, Florida, Buenos Aires, Argentina|Centro Platense en Investigaciones Respiratorias, La Plata, Buenos Aires, Argentina|Instituto ave Pulmo Fundacion enfisema, Mar del Plata, Buenos Aires, Argentina|INSARES, Ciudad de Mendoza, Mendoza, Argentina|Respira Salud Clinica Integral, Godoy Cruz, Provincia De Mendoza, Argentina|Investigaciones en Patologias Respiratorias, San Miguel De Tucumán, Tucumán, Argentina|Westmead Hospital, Westmead, New South Wales, Australia|Mater Health Services Adult Hospital, South Brisbane, Queensland, Australia|Princess Alexandra Hospital, Woolloongabba, Queensland, Australia|Royal Adelaide Hospital, Adelaide, South Australia, Australia|Box Hill Hospital, Box Hill, Victoria, Australia|The Alfred Hospital, Melbourne, Victoria, Australia|Centro de Investigacion Clinica V Region CINVEC, Vina del Mar, Region De Valparaiso, Chile|Centro Investigacion del Maule CIM, Talca, Region Del Maule, Chile|Instituto Nacional del Torax, Santiago, Region Metropolitana, Chile|CIMER, Santiago, Region Metropolitana, Chile|M y F estudios clinicos, Santiago, Region Metropolitana, Chile|Second Affiliated Hospital of Anhui Medical University, Hefei, Anhui, China|BeiJing Chao-Yang Hospital,Capital medical university, Beijing, Beijing, China|China-Japan friendship hospital, Beijing, Beijing, China|Peking Union Medical College Hospital, Beijing, Beijing, China|Nanfang Hospital of Southern Medical University, Guangzhou, Guangdong, China|The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong, China|Shenzhen people's hoepital, Shenzhen, Guangdong, China|Henan Provincial People's Hospital, Zhengzhou, Henan, China|Henan Provincial chest hospital, Zhengzhou, Henan, China|Tongji Hospital, Tongji Medical College of Huazhong University of Science and Technology, Wuhan, Hubei, China|Union Hospital,Tongji Medical College of Huazhong University of Science and Technology, Wuhan, Hubei, China|The Second Xiangya Hospital of Central South University, Changsha, Hunan, China|Affiliated Hospital of Inner Mongolia Medical University, Hohhot, Inner Mongolia, China|People's Hospital of Inner Mongolia Autonomous Region, Hohhot, Inner Mongolia, China|Nanjing Drum Tower Hospital, Nanjing, Jiangsu, China|The second hospital of Dalian medical university, Dalian, Liaoning, China|The First Hospital of China Medical University, Shenyang, Liaoning, China|General Hospital of Ningxia Medical University, Yinchuan, Ningxia, China|Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, Shanghai, China|Shanghai Pulmonary Hospital of Tongji University, Shanghai, Shanghai, China|The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shanxi, China|Huaxi Hospital of Sichuan University., ChenDu, Sichuan, China|General Hospital of Tianjin Medical University, Tianjin, Tianjin, China|Hangzhou First People's Hospital, Hangzhou, Zhejiang, China|Nemocnice Jihlava, Jihlava, Czechia|Hôpital Cardio-Vasculaire et Pneumologique Louis Pradel, Bron, France|C.H.R.U. de Montpellier - Hôpital Lapeyronie, Montpellier, France|CHU de Nice Hôpital Pasteur, Nice, France|Hôpital Bichat - Claude Bernard, Paris, France|CHU de Reims Hopital Maison Blanche, Reims, France|CHU de Rennes Hôpital Pontchaillou, Rennes, France|Ruhrlandklinik-Universitaetsmedizin Essen, Essen, Germany|Agaplesion Evangelisches Krankenhaus Mittelhessen, Gießen, Germany|Universitätsklinikum Hamburg-Eppendorf, Hamburg, Germany|Queen Mary Hospital, Central, Hong Kong|Princess Margaret Hospital, Hong Kong, Hong Kong|Tuen Mun Hospital, Hong Kong, Hong Kong|Petz Aldar County Teaching Hospital, Gyor, Hungary|Fejer Megyei Szent Gyorgy kh, Szekesfehervar, Hungary|Gaspare Rodiloco Hospital, Catania, Italy|AOU Policlinico di Modena, Modena, Italy|Monaldi Hospital, Naples, Italy|IRCCS Fondazione San Matteo di Pavia, Pavia, Italy|Soonchunhyang University Bucheon Hospital, Bucheon-si, Gyeonggi-do, Korea, Republic of|The Catholic University of Korea, Bucheon St. Mary's Hospital, Bucheon-si, Gyeonggi-do, Korea, Republic of|Seoul National University Bundang Hospital, Seongnam-si, Gyeonggi-do, Korea, Republic of|Ajou University Hospital, Suwon-si, Gyeonggi-do, Korea, Republic of|Inje University Busan Paik Hospital, Busan, Korea, Republic of|Gachon University Gil Medical Center, Incheon, Korea, Republic of|Korea University Anam Hospital, Seoul, Korea, Republic of|Seoul National University Hospital, Seoul, Korea, Republic of|Asan Medical Center, Seoul, Korea, Republic of|Samsung Medical Centerx, Seoul, Korea, Republic of|Hotel Dieu De France, Beirut, Lebanon|St. Antonius Ziekenhuis BV, Nieuwegein, Netherlands|Canisius-Wilhelmina Ziekenhuis, Nieuwegein, Netherlands|Clinica San Pablo, Lima, Peru|Hospital Guillermo Almenara Irygoyen, Lima, Peru|Clinica Providencia, Lima, Peru|Hospital Nacional Cayetano Heredia / Servicio de Inmunología y Reumatología, Lima, Peru|Centro de Investigacion Ricardo Palma, San Isidro, Peru|Clinica La luz, Santa Beatriz, Peru|Regional Clinical Hospital #3, Chelyabinsk, Russian Federation|Medical Association "NEW HOSPITAL", Ekaterinburg, Russian Federation|Kazan State Medical University based on Republican Clinical Hospital, Kazan, Russian Federation|Clinical Hospital #3, Kemerovo, Russian Federation|Euromedservice, Clinical and Diagnostic Center, Moscow, Russian Federation|MONIKI, Moscow, Russian Federation|City Clinical Hospital #1 n.a. A.N. Kabanov, Omsk, Russian Federation|Republican Hospital named V.A. Baranov, Petrozavodsk, Russian Federation|FSBEI HE First Pavlov Medical University, Saint-Petersburg, Russian Federation|Vvedenskaya hospital, St. Petersburg, Russian Federation|Medical Center "Reavita Med SPb", St. Petersburg, Russian Federation|Hospital Universitario de Bellvitge, Barcelona, Spain|Hospital Clinic de Barcelona, Barcelona, Spain|Hospital Universitaio de La Princesa, Madrid, Spain|Hospital General Universitario Gregorio Maranon, Madrid, Spain|Hospital Universitario Quironsalud Madrid, Madrid, Spain|Hospital Universitario Marques de Valdecilla, Santander, Spain|Taichung Veterans General Hospital, Taichung, Taiwan|Taipei Medical University - Shuang-Ho Hospital, Ministry of Health and Welfare, Taipei, TaiwanMore Information
A Study to Evaluate Long Term Safety and Efficacy of Recombinant Human Pentraxin-2 (rhPTX-2; PRM-151) in Participants With Idiopathic Pulmonary Fibrosis This study will evaulate the long-term safety, efficacy and pharmacokinetics (PK) of recombinant human pentraxin-2 (rhPTX-2; PRM-151) administered by intravenous (IV) infusion to participants with idiopathic pulmonary fibrosis (IPF).Phase 3Not yet recruitingIdiopathic Pulmonary FibrosisDrug: PRM-151Hoffmann-La RocheInst. of Healthcare Assessment, Inc., San Diego, California, United States|UCSF Medical Center, San Francisco, California, United States|Paloma Medical Group, San Juan Capistrano, California, United States|National Jewish Health Medical Center, Denver, Colorado, United States|Yale New Haven Hospital, New Haven, Connecticut, United States|Meris Clinical Research, Brandon, Florida, United States|Florida Lung, Asthma and Sleep Specialists (FLASS) - Celebration, Celebration, Florida, United States|Advanced Pulmonary & Sleep Research Institute of Florida, Daytona Beach, Florida, United States|San Marcus Research Clinic Inc., Miami, Florida, United States|Central Florida Pulmonary Group, PA, Orlando, Florida, United States|Bassetti Medical Research, Inc, Sebring, Florida, United States|Renstar Medical Research, Wesley Chapel, Florida, United States|Integral - Clinical Trial Solutions, Weston, Florida, United States|Florida Pulmonary Research Institute, LLC, Winter Park, Florida, United States|University of Kansas Medical Center, Kansas City, Kansas, United States|University of Louisville Health Sciences Center, Louisville, Kentucky, United States|Massachusetts General Hospital, Boston, Massachusetts, United States|SleepMed of South Carolina, Columbia, South Carolina, United States|Lowcountry Lung and Critical Care, North Charleston, South Carolina, United States|Horizon Clinical Research Group, Cypress, Texas, United States|University of Texas Southwestern Medical Center; Pediatrics, Dallas, Texas, United States|Inova Fairfax Hospital/IHVI; Cardiology, Falls Church, Virginia, United States|University of Washington Medical Center, Seattle, Washington, United States|University of Wisconsin-Madison, Madison, Wisconsin, United States|Medical College of Wisconsin, Milwaukee, Wisconsin, United States|Centro de Alergia y Enfermedades Respiratorias, Buenos Aires, Argentina|Fundacion Respirar (Centro Medico Dra. De Salvo), Ciudad Autonoma Buenos Aires, Argentina|Instituto de Medicina Respiratoria, IMeR; Clinic/Outpatient Facility, Córdoba, Argentina|Centro de estudios respiratorios, Mar del Plata, Argentina|INSARES, Mendoza, Mendoza City, Argentina|Fundación Scherbovsky, Mendoza, Argentina|Centro Respiratorio Infantil, Rosario, Argentina|Investigaciones en Patologias Respiratorias, San Miguel de Tucuman, Argentina|ULB Hôpital Erasme, Brussels, Belgium|Cliniques Universitaires Saint-Luc; Pharmacy, Bruxelles, Belgium|CHU UCL Namur / site Godinne, Yvoir, Belgium|Thomayerova nemocnice; Lekarna - pavilon H, Praha 4, Czechia|Hopital Louis Pradel, Bron, France|Klifeck GmbH, Delitzsch, Germany|Universitatsklinikum Gießen und Marburg GmbH, Gießen, Germany|Thoraxklinik-Heidelberg gGmbH; Apotheke der Thoraxklinik, Heidelberg, Germany|Evangelismos Hospital; Endocrinology, Diabetes & Metabolism, Athens, Greece|National and Kapodistrian University of Athens EKPA Medical School, Athens, Greece|Sotiria Hospital for Diseases of the Chest, Academic Department of Pneumonology, Athens, Greece|University General Hospital of Heraklion, Herakleion, Greece|University Hospital of Ioannina; Pulmonology Department, Ioannina, Greece|University General Hospital of Larisa; Neurology Clinic, Larisa, Greece|Athens Medical Center, Marousi, Greece|"General hospital of Chest Diseases ""Sotiria"", 7th Pulmonary Clinic ", Sotiria Athens, Greece|General Hospital of Thessalonikis G.Papanikolaou; Nephrology, Thessaloniki, Greece|Swiss Clinic - Swiss Premium Egeszsegkozpont, Budapest, Hungary|Trial Pharma Kft, Csorna, Hungary|Debreceni Egyetem, Debrecen, Hungary|Hadassah Medical Center, Jerusalem, Israel|The Chaim Sheba Medical Center, Ramat Gan, Israel|Tel Aviv Sourasky Medical Center; Department of Neurology, Tel Aviv, Israel|"AZIENDA OSPEDALIERA DEI COLLI Monaldi-Cotugno-CTO - Pneumologia ", Napoli, Campania, Italy|Azienda Ospedaliera Dei Colli, Napoli, Campania, Italy|Azienda Ospedaliera Universitaria Federico II, Napoli, Campania, Italy|Università degli Studi di Napoli Federico II; Dipartimento di Scienze Oftalmologiche, Napoli, Campania, Italy|Ospedale Morgagni Pierantoni; Patologia clinica, Forlì, Emilia-Romagna, Italy|Policlinico Tor Vergata - U.O.C. Malattie Apparato Respiratorio, Rome, Lazio, Italy|Fondazione Policlinico Universitario A Gemelli; Servizio di Farmacia, Rome, Lazio, Italy|"Asst Rhodense - Unità operativa di pneumologia Ospedale ""Guido Salvini"" ", Garbagnate Milanese, Lombardia, Italy|Ospedale San Giuseppe; U.O. di Pneumologia, Milano, Lombardia, Italy|Ospedale San Gerardo, Monza, Lombardia, Italy|Fondazione IRCCS Policlinico San Matteo ; Divisione di Ematologia Dipartimento di Onco-Ematologia, Pavia, Lombardia, Italy|Policlinico Riuniti di Foggia - Department of Medical and Surgical Sciences - University of Foggia, Foggia, Puglia, Italy|Ospedale di Ostuni - Pneumologia, Ostuni, Puglia, Italy|A.O.U. Sassari; Dip. Medicina Clinica e Sperimentale, Sassari, Sardegna, Italy|Azienda Ospedaliero - Universitaria Policlinico - Vittorio Emanuele, Catania, Sicilia, Italy|Az. Osp. Univ. P. Giaccone; UOC di Pneumologia, Palermo, Sicilia, Italy|A.O. Univ. Senese Policlinico S. Maria alle Scotte; Dip. Medicina clinica e Scienze Immunologiche, Siena, Toscana, Italy|University of Padova Ca' Foncello Hospital Immunologic & Respiratory Rare Diseases Center, Treviso, Veneto, Italy|Erasmus Medisch Centrum, Rotterdam, Netherlands|Centrum Medycyny Oddechowej Mroz sp. j., Białystok, Poland|Nasz Lekarz Osrodek Badan Klinicznych, Bydgoszcz, Poland|Jagiellonskie Centrum Innowacji, Kraków, Poland|Centrum Badań Klinicznych, Wrocław, Poland|University of Cape Town Lung Institute; Lung Clinical Research, Cape Town, South Africa|Into Research; Life Groenkloof Hospital Medical Centre, Groenkloof, South Africa|Vergelegen Medi-Clinic, Somerset West, South Africa|Hospital Universitari de Bellvitge, L'hospitalet de Llobregat, Barcelona, Spain|CHUS - H. Clinico U. de Santiago, Santiago de Compostela, LA Coruña, Spain|Hospital Universitario Quironsalud Madrid, Pozuelo de Alarcón, Madrid, Spain|Universidad de Navarra - Clinica Universitaria de Navarra (CUN), Pamplona, Navarra, Spain|Centre Hospitalier Universitaire Vaudois (CHUV) ; Département d'Oncologie médicale - BH09 - Bureau 7, Lausanne, Switzerland|Municipal Non-Profit Enterprise City Clinical Hospital #16 of Dnipro City Council, Dnipro, Katerynoslav Governorate, Ukraine|National Academy of Medical Science of Ukraine (NAMS); NRCRM, Kyiv, KIEV Governorate, Ukraine|Yanovskyy Inst of Phth. & Pulm; Clinical-functional, Kyiv, UkraineMore Information
A Study to Evaluate the Efficacy and Safety of Recombinant Human Pentraxin-2 (rhPTX-2; PRM-151) in Participants With Idiopathic Pulmonary Fibrosis This phase III study will evaluate the efficacy, safety and pharmacokinetics (PK) of recombinant human pentraxin-2 (rhPTX-2; PRM-151) compared with placebo in participants with idiopathic pulmonary fibrosis (IPF).
Phase 3RecruitingIdiopathic Pulmonary FibrosisDrug: PRM-151|Drug: PlaceboHoffmann-La RocheUniversity of Alabama at Birmingham, Birmingham, Alabama, United States|Banner University Medical Center Phoenix; Main Pharmacy, Phoenix, Arizona, United States|Lovelace Scientific Resources, Inc., Phoenix, Arizona, United States|VA Long Beach Healthcare System, Long Beach, California, United States|Keck School of Medicine - University of Southern California, Los Angeles, California, United States|University of California Los Angeles Medical Center, Los Angeles, California, United States|University of California Davis, Sacramento, California, United States|Inst. of Healthcare Assessment, Inc., San Diego, California, United States|Sutter Health, San Francisco, California, United States|UCSF Medical Center, San Francisco, California, United States|Paloma Medical Group, San Juan Capistrano, California, United States|William Sansum Diabetes Center, Santa Barbara, California, United States|Standford Hosp, Stanford, California, United States|Cedars-Sinai Medical Center (CSMC) - Women's Guild Lung Institute, West Hollywood, California, United States|University of Colorado - Anschutz Medical Campus (University of Colorado Health Sciences Center), Aurora, Colorado, United States|National Jewish Health Medical Center, Denver, Colorado, United States|Yale New Haven Hospital, New Haven, Connecticut, United States|Christiana Care Pulmonary Associates - Newark, Newark, Delaware, United States|Georgetown University Medical Center, Washington, District of Columbia, United States|Meris Clinical Research, Brandon, Florida, United States|Florida Lung, Asthma and Sleep Specialists (FLASS) - Celebration, Celebration, Florida, United States|Advanced Pulmonary & Sleep Research Institute of Florida, Daytona Beach, Florida, United States|University of Florida Health Pulmonology - Jacksonville, Jacksonville, Florida, United States|San Marcus Research Clinic Inc., Miami, Florida, United States|Renstar Medical Research, Ocala, Florida, United States|Central Florida Pulmonary Group, PA, Orlando, Florida, United States|Sarasota Memorial Health Care System; Cancer Research Program, Sarasota, Florida, United States|Bassetti Medical Research, Inc, Sebring, Florida, United States|University of South Florida, Tampa, Florida, United States|Integral - Clinical Trial Solutions, Weston, Florida, United States|Clinical Site Partners Orlando, Winter Park, Florida, United States|Emory University, Atlanta, Georgia, United States|Piedmont Healthcare Pulmonary and Critical Care Research, Austell, Georgia, United States|Rush University Medical Center, Chicago, Illinois, United States|NorthShore University HealthSystem, Evanston, Illinois, United States|Loyola University Medical Center; Clinical Trials Pharmacy, Maywood, Illinois, United States|Indiana Univ School of Med, Indianapolis, Indiana, United States|University of Iowa Hospitals and Clinics; Investigational Drug Services, Iowa City, Iowa, United States|University of Kansas Medical Center, Kansas City, Kansas, United States|University of Kentucky Albert B. Chandler Hospital - Pavilion H, Lexington, Kentucky, United States|University of Louisville Health Sciences Center, Louisville, Kentucky, United States|Tulane University School of Medicine, New Orleans, Louisiana, United States|Massachusetts General Hospital, Boston, Massachusetts, United States|Brigham & Women'S Hospital, Boston, Massachusetts, United States|Beth Israel Deaconess Med Ctr, Boston, Massachusetts, United States|Covenant Medical Center - Cooper, Saginaw, Michigan, United States|University of Minnesota Medical Center, Minneapolis, Minnesota, United States|Mayo Clinic Rochester, Rochester, Minnesota, United States|The Lung Research Center, Chesterfield, Missouri, United States|Washington University, Saint Louis, Missouri, United States|University of Nebraska Medical Center, Omaha, Nebraska, United States|Dartmouth-Hitchcock Medical Center ; Pulmonary/Clinic 5C, Lebanon, New Hampshire, United States|Newark Beth Israel Medical Center, Newark, New Jersey, United States|Pulmonary & Allergy Associates, Summit, New Jersey, United States|Albany Med College, Albany, New York, United States|Stony Brook Internists PC, Commack, New York, United States|NYU Langone Medical Center; Investigational Drug Pharmacy, New York, New York, United States|Icahn School of Medicine at Mount Sinai, New York, New York, United States|Lenox Hill Hospital, New York, New York, United States|American Health Research Inc., Charlotte, North Carolina, United States|Duke University Medical Center, Durham, North Carolina, United States|Pulmonix Research, Greensboro, North Carolina, United States|PMG Research of Wilmington, Wilmington, North Carolina, United States|University of Cincinnati; Department of Psychiatry and Behavioral Neuroscience, Cincinnati, Ohio, United States|University Hospitals Cleveland Medical Center, Cleveland, Ohio, United States|Cleveland Clinic, Cleveland, Ohio, United States|The Ohio State University Wexner Medical Center, Columbus, Ohio, United States|Mercy St. Vincent Medical Center, Toledo, Ohio, United States|Oregon Health and Science University, Portland, Oregon, United States|Penn State Milton S. Hershey Medical Center, Hershey, Pennsylvania, United States|Jefferson University Physicians (JUP) - Jefferson Pulmonary, Philadelphia, Pennsylvania, United States|Temple University Medical Center, Philadelphia, Pennsylvania, United States|University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States|Medical University of South Carolina Hospital, Charleston, South Carolina, United States|SleepMed of South Carolina, Columbia, South Carolina, United States|Greenville Pharmaceutical Research, Greenville, South Carolina, United States|Lowcountry Lung and Critical Care, North Charleston, South Carolina, United States|Carolina Lung Clinic, PA, Rock Hill, South Carolina, United States|Spartanburg Medical Center, Spartanburg, South Carolina, United States|Vanderbilt University Medical Center, Nashville, Tennessee, United States|Horizon Clinical Research Group, Cypress, Texas, United States|University of Texas Southwestern Medical Center; Pediatrics, Dallas, Texas, United States|University of Texas Medical Branch; Cardiology Department, Galveston, Texas, United States|Baylor College of Medicine; Dept. of Psychiatry, Houston, Texas, United States|DCOL Center for Clinical Research, Longview, Texas, United States|Metroplex Pulmonology & Sleep Center, McKinney, Texas, United States|University of Texas Health Sciences Center in San Antonio, San Antonio, Texas, United States|University Of Utah Hosp & Clin; Investigational Pharmacy, Salt Lake City, Utah, United States|Inova Fairfax Hospital/IHVI; Cardiology, Falls Church, Virginia, United States|Virginia Commonwealth University, Richmond, Virginia, United States|University of Washington Medical Center, Seattle, Washington, United States|University of Wisconsin-Madison, Madison, Wisconsin, United States|Medical College of Wisconsin, Milwaukee, Wisconsin, United States|Fundacion Favaloro, Buenos Aires, Argentina|Centro de Alergia y Enfermedades Respiratorias, Buenos Aires, Argentina|Hospital Britanico de Buenos Aires, Ciudad Autonoma Buenos Aires, Argentina|Fundacion Respirar (Centro Medico Dra. De Salvo), Ciudad Autonoma Buenos Aires, Argentina|Instituto de Medicina Respiratoria, IMeR; Clinic/Outpatient Facility, Córdoba, Argentina|Hospital Universitario Austral, Derqui-Pilar, Argentina|Centro Medico de Enfermedades Respiratorias (CEMER), Florida, Argentina|Centro de estudios respiratorios, Mar del Plata, Argentina|INSARES, Mendoza, Mendoza City, Argentina|Fundación Scherbovsky, Mendoza, Argentina|Centro Respiratorio Infantil, Rosario, Argentina|Investigaciones en Patologias Respiratorias, San Miguel de Tucuman, Argentina|Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia|South Western Sydney Local Health District - Eastern Campus Liverpool Hospital, Liverpool, New South Wales, Australia|Lung Research Queensland, Nundah, Queensland, Australia|Mater Medical Centre - Brisbane, South Brisbane, Queensland, Australia|Princess Alexandra Hospital, Woolloongabba, Queensland, Australia|The Queen Elizabeth Hospital, Adelaide, South Australia, Australia|Austin Hospital; Cancer Clinical Trials Centre, Melbourne, Victoria, Australia|Monash Medical Centre; Medicine, Melbourne, Victoria, Australia|The Alfred Hospital, Prahan, Victoria, Australia|Fiona Stanley Hospital, Murdoch, Western Australia, Australia|St John of God Hospital, Murdoch, Western Australia, Australia|Sir Charles Gairdner Hospital; Department Of Respirtory Medicine, WA Lung Reserch, Nedlands, Western Australia, Australia|Medizinische Universitaet Innsbruck - Universitaetsklinik fuer Innere Medizin III, Innsbruck, Austria|Klinikum Klagenfurt am Wörthersee; 2. Medizinische Abteilung, Klagenfurt, Austria|Landeskrankenhaus Salzburg; Gynäkologie Und Onkologie, Salzburg, Austria|AKH - Medizinische Universität Wien; Department of Oncology, Vienna, Austria|ULB Hôpital Erasme, Brussels, Belgium|Cliniques Universitaires Saint-Luc; Pharmacy, Bruxelles, Belgium|UZ Leuven, Leuven, Belgium|C. H. U. Sart-Tilman; Laboratory, Liège, Belgium|CHU UCL Namur / site Godinne, Yvoir, Belgium|Núcleo de Estudos Clínicos da Bahia Hospital Universitário Professor Edgard Santos, Salvador, BA, Brazil|Hospital das Clínicas da Universidade Federal do Paraná - HCPR, Belo Horizonte, MG, Brazil|Hospital Madre Tereza, Belo Horizonte, MG, Brazil|Santa Casa de Misericordia de Porto Alegre, Porto Alegre, RS, Brazil|Hospital Ernesto Dornelles, Porto Alegre, RS, Brazil|Hospital Sao Lucas da PUCRS*X, Porto Alegre, RS, Brazil|Hospital Dia do Pulmão, Blumenau, SC, Brazil|Faculdade de Medicina do ABC - FMABC, Santo Andre, SP, Brazil|CEMEC - Centro Multidisciplinar de Estudos Clínicos, Sao Bernardo Do Campo, SP, Brazil|*X*Hospital das Clinicas - FMUSP; Hematologia, Sao Paulo, SP, Brazil|University of Alberta Hospital, Edmonton, Alberta, Canada|Synergy Medical Clinic, Sherwood Park, Alberta, Canada|Kelowna Respiratory and Allergy Cllinic, Kelowna, British Columbia, Canada|Vancouver General Hospital; Gordon and Leslie Diamond Health Care, Vancouver, British Columbia, Canada|Clinique de pneumologie de Moncton Respiratory Clinic, Moncton, New Brunswick, Canada|Eastern Health - General Hospital, St. John's, Newfoundland and Labrador, Canada|St. Joseph's Healthcare Hamilton, Hamilton, Ontario, Canada|The Rhema Research Institute, Owen Sound, Ontario, Canada|CHUM Hôpital Notre-Dame, Montreal, Quebec, Canada|McGill University, Montreal Chest Institute; Viral and other Infectious, Montreal, Quebec, Canada|CMU-Beijing Chao-Yang Hospital-Beijing Institute of Respiratory Medicine, Beijing City, China|Peking Union Medical College Hospital; Pharmacy, Beijing City, China|Beijing Friendship Hospital Affiliated of Capital University of Medical Science, Beijing Shi, China|Peking University Third Hospital, Beijing, China|Anhui Provincial Hospital, Hefei, China|Ningbo First Hospital, Ningbo City, China|Zhongshan Hospital Fudan University, Shanghai, China|Huadong Hospital Affiliated to Fudan University, Shanghai, China|Shanghai Pulmonary Hospital, Shanghai, China|China Medical University (CMU) First Affiliated Hospital, Shenyang City, China|Tongji Hospital Tongji Medical College Huazhong University of Science and Technology, Wuhan City, China|Henan University of Traditional Chinese Medicine (HUTCM) The First Affiliated Hospital, Zhengzhou Shi, China|Fakultni nemocnice Brno; Interni hematologicka a onkologicka klinika, Brno, Czechia|Fakultni nemocnice Olomouc, Olomouc, Czechia|Fakultni Nemocnice Plzen - Klinika Pneumologie a Ftizeologie, Plzen, Czechia|Nemocnice Na Bulovce, Prague, Czechia|Fakultni poliklinika VFN; RS centrum, Praha 2, Czechia|Thomayerova nemocnice; Lekarna - pavilon H, Praha 4, Czechia|Krajska nemocnice T. Bati a.s.; Neurologicke oddeleni, Zlin, Czechia|Aarhus Universitetshospital Skejby, Aarthus N, Denmark|Gentofte Hospital, Hellerup, Denmark|Odense Universitetshospital, Odense C, Denmark|Oulun Yliopistollinen Sairaala; Teho-osasto, Oulu, Finland|Turku University Hospital, Turku, Finland|CHU Angers, Angers, France|Hopital Avicenne, Bobigny, France|Centre Hospitalier Regional Universitaire de Brest - Hopital de la Cavale Blanche, Brest, France|Hopital Louis Pradel, Bron, France|Hospital d Instructions des Armees Percy, Clamart, France|CHU Dijon Bourgogne Hôpital François Mitterand, Dijon, France|CHU de Grenoble - Hôpital André Michallon; Pole Pharmacie, La Tronche, France|Centre Hospitalier Regional Universitaire de Lille, Lille, France|Servie de Pneumologie et centre de Transplantation Pulmonaire, Marseille, France|CHU de Montpellier Hopital Arnaud de Villeneuve; de Génétique, Montpellier, France|CHU Nice - Hôpital Pasteur 2, Nice, France|Hopital Bichat Claude Bernard; Hepatologie Gastro Enterologie, Paris, France|European Hospital Georges Pompidou (HEGP), Paris, France|Hopital Haut Leveque, Pessac, France|Centre Hospitalier Universitaire de Reims; l'Hopital Maison Blanche, Reims, France|CHU de Rennes - Hopital de Pontchaillo, Rennes, France|CHU Tours - Hôpital Bretonneau; Pharmacie, Tours, France|Evang. Lungenklinik Berlin Klinik für Pneumologie, Berlin, Germany|Fachkrankenhaus Coswig GmbH Zentrum f.Pneumologie Beatmungsmedizin Thorax-u.Gefäßchirurgie, Coswig, Germany|MECS Cottbus, Cottbus, Germany|Klifeck GmbH, Delitzsch, Germany|Klinik Donaustauf Zentrum für Pneumologie, Donaustauf, Germany|Universitatsklinikum Essen; Innere Klinik, Essen, Germany|IFS - Interdisziplinäres Facharztzentrum; Institut für klinische Forschung Pneumologie, Frankfurt am Main, Germany|Universitatsklinikum Gießen und Marburg GmbH, Gießen, Germany|Universitatsklinikum Hamburg-Eppendorf; Klinik und Poliklinik fur Neurochirurgie, Hamburg, Germany|Fraunhofer-Institut für Toxikologie und Experimentelle Medizin ITEM, Hannover, Germany|Thoraxklinik-Heidelberg gGmbH; Apotheke der Thoraxklinik, Heidelberg, Germany|Universitätsklinikum des Saarlandes; Klinik für Augenheilkunde, Homburg/Saar, Germany|Lungenfachklinik Immenhausen, Immenhausen, Germany|Universitatsklinikum Leipzig; Apotheke, Leipzig, Germany|IKF Pneumologie Mainz Helix Medical Excellence Center Mainz, Mainz, Germany|Praxis für Pneumologie Dr. Silke Mronga & Dr. Lukas Jerrentrup, Marburg, Germany|Universitaetsklinikum Muenster, Muenster, Germany|Klinikum der Universität München - Großhadern; Apotheke, München, Germany|Lungenklinik Münnerstadt, Thoraxzentrum Bezirk Unterfranken, Münnerstadt, Germany|Krankenhaus Bethanien gGmbH, Solingen, Germany|Evangelismos Hospital; Endocrinology, Diabetes & Metabolism, Athens, Greece|Laiko General Hospital of Athens; A Propedeutical Clinic of Internal Medicine, Athens, Greece|National and Kapodistrian University of Athens EKPA Medical School, Athens, Greece|Sotiria Hospital for Diseases of the Chest, Academic Department of Pneumonology, Athens, Greece|"Attikon" University General Hospital; 3rd Department of Pediatrics, Chaidari, Greece|University General Hospital of Heraklion, Herakleion, Greece|University Hospital of Ioannina; Pulmonology Department, Ioannina, Greece|University General Hospital of Larisa; Neurology Clinic, Larisa, Greece|Athens Medical Center, Marousi, Greece|"General hospital of Chest Diseases ""Sotiria"", 7th Pulmonary Clinic ", Sotiria Athens, Greece|General Hospital of Thessalonikis G.Papanikolaou; Nephrology, Thessaloniki, Greece|The University of Hong Kong; Queen Mary Hospital, Hong Kong, Hong Kong|Prince of Wales Hosp; Dept. Of Clinical Onc, Shatin, Hong Kong|Semmelweis University, Budapest, Hungary|Trial Pharma Kft, Csorna, Hungary|Debreceni Egyetem, Debrecen, Hungary|Megyei Tudogyogyintezet - Pulmonologia, Farkasgyepu, Hungary|Borsod-Abauj-Zemplen (BAZ) Megyei Kozponti Korhaz es Egyetemi Oktatokorhaz, Miskolc, Hungary|Markusovszky Egyetemi Oktatokorhaz ; SZEMESZET, Szombathely, Hungary|Szent Borbala Korhaz, Tatabánya, Hungary|Tudogyogyintezet Torokbalint; Onkologiai és Jarobeteg Centrum, Törökbálint, Hungary|Barzilai Medical Center; Neurology Department, Ashkelon, Israel|Shamir medical center, Be'er Ya'akov, Israel|Soroka University Medical Center, Psychiatry; Psychiatry, Beer Sheva, Israel|Rambam Health Care Campus, Haifa, Israel|Bnai Zion Medical Center; Internal Medicine, Haifa, Israel|Carmel Medical Center; Obstetrics and Gynecology, Haifa, Israel|Edith Wolfson Medical Center, Holon, Israel|Shaare Zedek Medical Center; Nrphrology Dept., Jerusalem, Israel|Hadassah Medical Center, Jerusalem, Israel|Meir Medical Center, Kfar- Saba, Israel|Rabin Medical Center, Petach Tiqwa, Israel|The Chaim Sheba Medical Center, Ramat Gan, Israel|Kaplan Medical Center, Rehovot, Israel|Tel Aviv Sourasky Medical Center; Department of Neurology, Tel Aviv, Israel|Azienda Ospedaliera Papa Giovanni XXIII, Bergamo, Abruzzo, Italy|AORN Ospedali dei Colli Ospedale Monaldi; UOC Pneumologia ad indirizzo Oncologico, Napoli, Campania, Italy|Azienda Ospedaliera Dei Colli, Napoli, Campania, Italy|Azienda Ospedaliera Universitaria Federico II, Napoli, Campania, Italy|Università degli Studi di Napoli Federico II; Dipartimento di Scienze Oftalmologiche, Napoli, Campania, Italy|Ospedale Morgagni Pierantoni; Patologia clinica, Forlì, Emilia-Romagna, Italy|A.O.U. Policlinico di Modena, Modena, Emilia-Romagna, Italy|Azienda Ospedaliera San Camillo Forlanini; Farmacia Ospedale San Camillo, Roma, Lazio, Italy|Policlinico Tor Vergata - U.O.C. Malattie Apparato Respiratorio, Rome, Lazio, Italy|Fondazione Policlinico Universitario A Gemelli; Servizio di Farmacia, Rome, Lazio, Italy|"Asst Rhodense - Unità operativa di pneumologia Ospedale ""Guido Salvini"" ", Garbagnate Milanese, Lombardia, Italy|Ospedale San Giuseppe; U.O. di Pneumologia, Milano, Lombardia, Italy|Asst Santi Paolo E Carlo, Milano, Lombardia, Italy|Ospedale San Gerardo, Monza, Lombardia, Italy|Fondazione IRCCS Policlinico San Matteo ; Divisione di Ematologia Dipartimento di Onco-Ematologia, Pavia, Lombardia, Italy|Azienda Ospedaliero Universitaria Ospedali Riuniti, Torrette Di Ancona, Marche, Italy|A.O.U. S. Luigi Gonzaga; Interstiziopatie Malattie Rare Polmone, Orbassano (to), Piemonte, Italy|Azienda Ospedaliera Universitaria Citta della Salute e della Scienza di Torino; Ospedale Molinette, Torino, Piemonte, Italy|Policlinico Riuniti di Foggia - Department of Medical and Surgical Sciences - University of Foggia, Foggia, Puglia, Italy|Ospedale di Ostuni - Pneumologia, Ostuni, Puglia, Italy|A.O.U. Sassari; Dip. Medicina Clinica e Sperimentale, Sassari, Sardegna, Italy|Azienda Ospedaliero - Universitaria Policlinico - Vittorio Emanuele, Catania, Sicilia, Italy|Az. Osp. Univ. P. Giaccone; UOC di Pneumologia, Palermo, Sicilia, Italy|Ismett Istituto Mediterraneo Trapianti E Terapie Alta Specializzazione;Pneumologia, Palermo, Sicilia, Italy|AOUC Azienda Ospedaliero-Universitaria Careggi; Neurologia 2, Firenze, Toscana, Italy|A.O. Univ. Senese Policlinico S. Maria alle Scotte; Dip. Medicina clinica e Scienze Immunologiche, Siena, Toscana, Italy|Azienda Ospedale Universita Di Padova; Divisione Clinicizzata Di Nefrologia 1, Padova, Veneto, Italy|University of Padova Ca' Foncello Hospital Immunologic & Respiratory Rare Diseases Center, Treviso, Veneto, Italy|Soon Chun Hyang University Hospital Bucheon, Bucheon-si,, Korea, Republic of|The Catholic University of Korea Bucheon St. Mary's Hospital, Gyeonggi-do, Korea, Republic of|Gacheon University Gil Medical Center, Namdong-gu, Korea, Republic of|Seoul National University Bundang Hospital, Seongnam-si, Korea, Republic of|The Catholic University of Korea Yeouido St. Mary's Hospital; Hematology-Oncology, Seoul, Korea, Republic of|Seoul National University Hospital (SNUH) - Medical Oncology Center, Seoul, Korea, Republic of|Soonchunhyang University Hospital, Seoul, Korea, Republic of|Asan Medical Center, Seoul, Korea, Republic of|Samsung Medical Center, Seoul, Korea, Republic of|Korea University Guro Hospital, Seoul, Korea, Republic of|The Catholic University of Korea Seoul St. Mary's Hospital, Seoul, Korea, Republic of|Unidad Médica para la Salud Integral UMSI, San Nicolás de Los Garza, Nuevo LEON, Mexico|Unidad de Investigacion CIMA SC, Chihuahua, Mexico|Centro de Diagnóstico y Tratamiento de Enfermedades Respiratorias, Medica San Isidro, Metepec, Mexico|Centro Respiratorio de México, Mexico, Mexico|INER- Instituto Nacional de Enfermedades Respiratorias"Ismae, Mexico, Mexico|Hospital Uni Dr. Jose E. Gonzalez; Centro Regional de Prevencion Y Diagnostico de Osteoporosis, Monterrey, Mexico|Unidad de Investigacion Clinica En Medicina (Udicem) S.C., Monterrey, Mexico|CICC Morelia, Morelia, Mexico|VU Medisch Centrum, Amsterdam, Netherlands|LUMC, Leiden, Netherlands|Sint Antonius Ziekenhuis, locatie Nieuwegein, Nieuwegein, Netherlands|Erasmus Medisch Centrum, Rotterdam, Netherlands|Auckland City Hospital, Auckland, New Zealand|NZ Respiratory & Sleep Institute, Auckland, New Zealand|University of Otago, Christchurch, Christchurch, New Zealand|Waikato Hospital; Gastro Research, Hamilton, New Zealand|Tauranga Hospital, Tauranga, New Zealand|Soerlandet Sykehus Arendal; Medisinsk Avdeling, Arendal, Norway|Haukeland Universitetshospital; Medicine Dept, Bergen, Norway|Akershus universitetssykehus, Lørenskog, Norway|Oslo University Hospital HF, Rikshospitalet, Oslo, Norway|Hospital de Chancay, Chancay, Peru|Hospital Militar Central del Ejercito del Perú, Jesús María, Peru|Hospital Guillermo Almenara Irigoyen Hospital Guillermo Almenara Irigoyen Hospital Guillermo Almen, La Victoria, Peru|Clinica San Borja; Servicio De Reumatologia, Lima, Peru|Clinica Delgado, Lima, Peru|Hospital Nacional Cayetano Heredia, Lima, Peru|Clinica San Pablo, Lima, Peru|Hospital Central FAP, Miraflores, Peru|Centro de Investigacion en Reumatologia de la Clinica Belen, Piura, Peru|Clinica Internacional - Sede San Borja, San Borja, Peru|Centrum Medycyny Oddechowej Mroz sp. j., Białystok, Poland|Nasz Lekarz Osrodek Badan Klinicznych, Bydgoszcz, Poland|Gornoslaskie Centrum Medyczne, Katowice, Poland|Jagiellonskie Centrum Innowacji, Kraków, Poland|Uniwersytecki Szpital Kliniczny Nr 1 im.N.Barlickiego Oddzial Kliniczny Pneumonologii i Alergologii, Lodz, Poland|PULMAG Arkadiusz Brodowski, Grzegorz Gasior S. C., Sosnowiec, Poland|Instytut Gruzlicy i Chorob Pluc; Apteka Zakladowa, Warszawa, Poland|Centrum Badań Klinicznych, Wrocław, Poland|Samodzielny Publiczny Szpital Kliniczny nr 1, Zabrze, Poland|Hospital de Braga; Centro Clínico Académico (Piso 1, Ala E), Braga, Portugal|Centro Hospitalar Algarve, Faro, Portugal|Hospital Senhora da Oliveira - Guimarses, E.P.E, Guimaraes, Portugal|Centro Hospitalar Universitario Lisboa Norte - Hospital Pulido Valente, Lisboa, Portugal|Centro Hospitalar do Porto - Hospital de Santo António, Porto, Portugal|Hospital de Sao Joao; Centro de Investigacao, Porto, Portugal|Hospital de Santa Luzia, Porto, Portugal|CHVNG/E_Unidade 1; Servico de Pneumologia, Vila Nova De Gaia, Portugal|Regional State Budgetary Institution of Healthcare "Regional Clinical Hospital", Barnaul, Altaj, Russian Federation|Moscow State Medical and Dental University n. a. A.I. Evdokimova, Moscow, Moskovskaja Oblast, Russian Federation|National Medical Research Center for Phthisiopulmonology and Infectious Diseases, Moscow, Moskovskaja Oblast, Russian Federation|Central NII tuberkuleza RAMN, Moscow, Moskovskaja Oblast, Russian Federation|SBHI of Stavropol region "City Hospital #2" of Pyatigorsk, Pyatigorsk, Stavropol, Russian Federation|Chelyabinsk Regional Clinical Hospital; Hematology, Chelyabinsk, Russian Federation|State Budgetary Educational Institution of Higher Professional Education, Sankt Peterburg, Russian Federation|SBIH at Vladimir Region "Regional Clinical Hospital", Vladimir, Russian Federation|National University Hospital, Singapore, Singapore|Singapore General Hospital, Singapore, Singapore|Tan Tock Seng Hospital, Singapore, Singapore|University of Cape Town Lung Institute; Lung Clinical Research, Cape Town, South Africa|Johese Clinical Research, Centurion, South Africa|Into Research; Life Groenkloof Hospital Medical Centre, Groenkloof, South Africa|Vergelegen Medi-Clinic, Somerset West, South Africa|HUA - Txagorritxu, Vitoria, Alava, Spain|Hospital Universitari de Bellvitge, L'hospitalet de Llobregat, Barcelona, Spain|Corporació Sanitaria Parc Taulí; Servicio de Neurología, Sabadell, Barcelona, Spain|Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Spain|CHUS - H. Clinico U. de Santiago, Santiago de Compostela, LA Coruña, Spain|Hospital Universitario del Henares, Coslada, Madrid, Spain|Hospital Universitario Puerta de Hierro - Majadahonda, Majadahonda, Madrid, Spain|Hospital Universitario Quironsalud Madrid, Pozuelo de Alarcón, Madrid, Spain|Universidad de Navarra - Clinica Universitaria de Navarra (CUN), Pamplona, Navarra, Spain|Hospital Galdakao-Usansolo, Galdakao, Vizcaya, Spain|Hospital de la Santa Creu i Sant Pau; Servicio de Neumologia, Barcelona, Spain|Hospital Univ Vall d'Hebron; Servicio de Oncologia, Barcelona, Spain|Hospital Clinic de Barcelona, Barcelona, Spain|Hospital Germans Trias i Pujol, Barcelona, Spain|Hospital Virgen de las Nieves; Servicio de Neumologia, Granada, Spain|Hospital Universitario de La Princesa; Aparato Digestivo, Madrid, Spain|Clínica Universidad de Navarra; Servicio de Farmacia, Madrid, Spain|Universidad Autonoma de Madrid (UAM) - Hospital Universitario La Paz, Madrid, Spain|Hospital Ramón y Cajal;Servicio de Neumología, Madrid, Spain|Fundacion Jimenez Diaz-UTE, Madrid, Spain|Hospital Clinico San Carlos; Servicio de Farmacia, Madrid, Spain|Hospital Universitario 12 de Octubre, Madrid, Spain|Hospital Clinico Universitario de Salamanca, Salamanca, Spain|CEIC del Hospital Virgen del Rocío, Sevilla, Spain|Hospital Clinico Universitario de Valencia, Valencia, Spain|Hospital Universitari i Politecnic La Fe de Valencia, Valencia, Spain|Sahlgrenska Universitetssjukhuset, Goteborg, Sweden|Skånes universitetssjukhus Malmö, Malmö, Sweden|Karolinska University Hospital, Stockholm, Sweden|Uppsala Universitet - Akademiska Sjukhuset, Uppsala, Sweden|Universitaetsspital Basel, Basel, Switzerland|Universitatsspital Bern, Bern, Switzerland|Centre Hospitalier Universitaire Vaudois (CHUV) ; Département d'Oncologie médicale - BH09 - Bureau 7, Lausanne, Switzerland|Hopital Neuchatelois Pourtales (Hopitaux Cadolles Pourtales), Neuchâtel, Switzerland|Buddhist Tzu Chi Dalin General Hospital; Rheumatology, Chia-yi, Taiwan|Kaohsiung Medical University Hospital; Chung-Ho Memorial Hospital, Kaoshiung City, Taiwan|China Medical University Hospital, Taichung, Taiwan|National Cheng Kung University Hospital, Tainan, Taiwan|National Taiwan University Hospital, Taipei, Taiwan|Far Eastern Memorial Hospital; Department of Clinical Pathology, Taipei, Taiwan|Veterans General Hospital - Taichung, Xitun Dist., Taiwan|Cukurova Universitesi Tip Fakultesi Balcali Hastanesi; Balcali; Onkologi, Adana, Turkey|Ankara Universitesi Tip Fakultesi Hastaneleri - Cebeci Hastanesi, Ankara, Turkey|Uludag Universitesi - Saglik Uygulama ve Arastirrma Merkezi, Bursa, Turkey|Gaziantep Universitesi Tip Fakultesi, Gaziantep, Turkey|Yedikule Gogus Hastaliklari ve Gogus Cerrahisi EAH;Gogus Hastaliklari, Istanbul, Turkey|Istanbul Universitesi Onkoloji Enstitüsü Hastanesi, İstanbul, Turkey|Ege Universitesi Tip Fakultesi Hastanesi, Izmir, Turkey|Dokuz Eylul University Faculty of Medicine; Chest Diseases, Izmir, Turkey|Kocaeli Universitesi Tip Fakultesi, Kocaeli, Turkey|Eriyes universitesi Tıp Fakultesi Hastanesi, Melikgazi/Kayser, Turkey|Mersin Universitesi Tip Fakultesi Hastanesi; Tibbi Onkoloji Birimi, Mersin, Turkey|Akdeniz University Faculty of Medicine Department of Pulmonology, Muratpasa/Antalya, Turkey|Koc Universitesi (KU) Tip Fakultesi (Koc University School of Medicine), Sariyer, Turkey|Marmara Universitesi Hastanesi Marmara University Hospital, Ueskuedar, Turkey|Haydarpaşa Sultan Abdülhamidhan Training and Research Hospital Department of Pulmonology, Üsküdar, Turkey|Municipal Non-Profit Enterprise City Clinical Hospital #16 of Dnipro City Council, Dnipro, Katerynoslav Governorate, Ukraine|National Academy of Medical Science of Ukraine (NAMS); NRCRM, Kyiv, KIEV Governorate, Ukraine|CHI Kharkiv City Clinical Hospital #13, Kharkiv, Ukraine|Yanovskyy Inst of Phth. & Pulm; Clinical-functional, Kyiv, Ukraine|Odessa regional clinical Hospital, Odessa, Ukraine|Edinburgh Royal Infirmary; Respiratory Department, Edinburgh, United Kingdom|Royal Devon & Exeter Hospital; Cardiology Department, Exeter, United Kingdom|Forth Valley Royal Hospital, Larbert, United Kingdom|University College London Hospital; Respiratory Medicine, London, United Kingdom|Guys and St Thomas NHS Foundation Trust, Guys Hospital, London, United Kingdom|North Manchester General Hospital, Manchester, United Kingdom|Queen Alexandra Hospital, Portsmouth, Portsmouth, United Kingdom|South Tyneside and Sunderland NHS Foundation trust, South Shields, United KingdomMore Information
Study of Efficacy and Safety of Inhaled Treprostinil in Subjects With Idiopathic Pulmonary Fibrosis Study RIN-PF-301 is designed to evaluate the safety and efficacy of inhaled treprostinil in subjects with idiopathic pulmonary fibrosis (IPF) over a 52-week period.
Phase 3RecruitingIdiopathic Pulmonary Fibrosis|Interstitial Lung DiseaseDrug: Placebo|Drug: Inhaled Treprostinil|Device: Treprostinil Ultrasonic NebulizerUnited TherapeuticsArizona Pulmonary Specialists, Ltd., Phoenix, Arizona, United States|David Geffen School of Medicine at UCLA, Los Angeles, California, United States|University of Florida, Jacksonville, Florida, United States|Ascension Medical Group St. Vincent's Lung Institute, Jacksonville, Florida, United States|Pulmonary Disease Specialists, PA d/b/a PDS Research, Kissimmee, Florida, United States|Central Florida Pulmonary Group, PA, Orlando, Florida, United States|University of South Florida, Tampa, Florida, United States|Loyola University Medical Center, Maywood, Illinois, United States|Maine Medical Center/Chest Medicine Associates, South Portland, Maine, United States|Tufts Medical Center, Boston, Massachusetts, United States|The Lung Research Center, Chesterfield, Missouri, United States|J&L Research, LLC, Saint Louis, Missouri, United States|University of Rochester Medical Center, Rochester, New York, United States|Pinehurst Medical Clinic, Inc., Pinehurst, North Carolina, United States|University of Cincinnati, Cincinnati, Ohio, United States|Cleveland Clinic, Cleveland, Ohio, United States|University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States|Medical University of South Carolina, Charleston, South Carolina, United States|Clinical Trials Center of Middle Tennessee, Franklin, Tennessee, United States|University of Utah Health, Salt Lake City, Utah, United States|Pulmonary Associates of Richmond, Inc., Richmond, Virginia, United StatesMore Information
Prospective Treatment Efficacy in IPF Using Genotype for Nac Selection (PRECISIONS) Trial The purpose of this study is to compare the effect of n-acetylcysteine (NAC) plus standard care with matched placebo plus standard of care in patients diagnosed with idiopathic pulmonary fibrosis (IPF) who have the TOLLIP rs3750920 TT genotype. The study will compare the time to a composite endpoint of relative decline in lung function [10% relative decline in forced vital capacity (FVC), first respiratory hospitalization, lung transplantation, or all-cause mortality]

The secondary objectives will be to examine the effect of NAC on the components of the primary composite endpoint, the rates of clinical events, change in physiology, change in health status, and change in respiratory symptoms.
Phase 3RecruitingIdiopathic Pulmonary FibrosisDrug: N-acetyl cysteine|Drug: PlaceboWeill Medical College of Cornell University|University of Virginia|University of Michigan|Pulmonary Fibrosis Foundation|University of Washington|National Heart, Lung, and Blood Institute (NHLBI)|Three Lakes FoundationStanford University, Stanford, California, United States|Piedmont Healthcare, Austell, Georgia, United States|Tulane University, New Orleans, Louisiana, United States|University of Michigan, Ann Arbor, Michigan, United States|University of Rochester, Rochester, New York, United States|Temple University, Philadelphia, Pennsylvania, United States|Medical University of South Carolina, Charleston, South Carolina, United StatesMore Information

Top treatments in development

AgentClass/Mechanism of ActionDevelopment StatusCompanyCompany ContactClinical StudiesMore Information
PamrevlumabPamrevlumab is a fully-human monoclonal antibody that inhibits the activity of connective tissue growth factor (CTGF). CTGF is a common factor in chronic fibrotic and proliferative disorders, characterized by persistent and excessive fibrous tissue which can lead to organ dysfunction and failure, and in cancer, characterized by promotion of tumor growth.Phase 3FibrogenGustavo Lorente 650-273-2264 Zephyrus@Fibrogen.com
https://clinicaltrials.gov/ct2/results?cond=IPF&term=Pamrevlumab&cntry=US&state=&city=&dist=More Information
rhPTX-2 (PRM-151)A recombinant human serum amyloid P/pentraxin 2 protein. Pentraxin-2 is an endogenous human protein that plays an important role in regulating the response to fibrosis. It directs the immune system to naturally turn off and reverse the process of fibrosis, which occurs as a result of excess collagen secretion and cellular growth and differentiation. Unlike other formulations that work by stopping a single target on the downstream side of fibrosis, this protein works by reversing and possibly healing the fibrotic tissue.Phase 3Hoffmann-La Roche
n/ahttps://clinicaltrials.gov/ct2/results?cond=ipf&term=PRM-151&cntry=&state=&city=&dist=n/a
Tyvaso® Treprostinil inhalation solutionTYVASO (treprostinil) is a prostacyclin vasodilator indicated for the treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to improve exercise ability. Studies establishing effectiveness included predominately patients with NYHA Functional Class III symptoms and etiologies of idiopathic or heritable PAH (56%) or PAH associated with connective tissue diseases (33%). The effects diminish over the minimum recommended dosing interval of 4 hours; treatment timing can be adjusted for planned activities.
While there are long-term data on use of treprostinil by other routes of administration, nearly all controlled clinical experience with inhaled treprostinil has been on a background of bosentan (an endothelin receptor antagonist) or sildenafil (a phosphodiesterase type 5 inhibitor). The controlled clinical experience was limited to 12 weeks in duration.
Phase 3United Therapeutics CorporationUnited Therapeutics Global Medical Information 919-485-8350 clinicaltrials@unither.comhttps://clinicaltrials.gov/ct2/results?cond=ipf&term=Treprostinil+inhalation+solution&cntry=&state=&city=&dist=More Information
N-acetyl cysteineN-acetyl cysteine (NAC) is a medication used to loosen thick mucus. NAC was initially licensed for use in 1968. It is on the World Health Organization's List of Essential Medicines, the most effective and safe medicines needed in a health system, and it is available as a generic medication and is not very expensive. Inhaled NAC has been used as a mucus dissolving therapy in respiratory conditions with excessive and/or thick mucus production.Phase 3NIH | US Federal Agencyn/ahttps://clinicaltrials.gov/ct2/results?cond=IPF&term=N-acetyl+cysteine&cntry=&state=&city=&dist=&Search=SearchMore Information