[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"familial-pulmonary-fibrosis\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:familial-pulmonary-fibrosis":26},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,4,0,[8,42,67,90],{"id":9,"slug":10,"hasResults":11,"nctId":12,"briefTitle":13,"officialTitle":14,"acronym":4,"eligibilityCriteria":15,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":4,"enrollmentInfo":18,"targetDuration":4,"studyType":21,"phases":22,"briefSummary":24,"conditions":25,"keywords":4,"overallStatus":29,"whyStopped":4,"lastUpdateSubmitDate":30,"lastUpdatePostDateStruct":31,"startDateStruct":34,"completionDateStruct":36,"leadSponsor":38,"locationsCount":41},"100608466","phase-3-a-study-to-test-whether-nerandomilast-can-help-slow-down-changes-in-the-lung-in-people-with-a-family-history-of-pulmonary-fibrosis-100608466",false,"NCT07201922","A Study to Test Whether Nerandomilast Can Help Slow Down Changes in the Lung in People With a Family History of Pulmonary Fibrosis","A Double Blind, Randomized, Placebo-controlled Exploratory Trial to Investigate the Efficacy and Safety of Nerandomilast Over 24 Months When Administered in Individuals With Interstitial Lung Abnormalities and a Family History of Pulmonary Fibrosis to Reduce the Risk of Worsening (DROP-FPF)","Inclusion Criteria:\n\n* Individuals ≥40 years of age at the time of first signed informed consent at Visit 1a\n* Participants must have at least 1 first-degree relative (biological parent, sibling, or child) with confirmed pulmonary fibrosis (idiopathic pulmonary fibrosis \\[IPF\\], idiopathic nonspecific interstitial pneumonia \\[NSIP\\], and\u002For pulmonary fibrosis due to known genetic cause \\[e.g. short telomere syndrome, mucin 5B (MUC5B) mutation, surfactant protein mutations\\])\n* High resolution computed tomography (HRCT) scan with evidence of interstitial lung abnormalities involving at least 5% of a single lung zone or interstitial lung disease (ILD), based on central evaluation\n* Forced vital capacity (FVC) ≥80% of predicted normal at Visit 1b\n* Diffusing capacity of the lungs for carbon monoxide (DLCO) corrected for hemoglobin ≥70% of predicted normal at Visit 1b Further inclusion criteria apply.\n\nExclusion Criteria:\n\n* Prior known pulmonary fibrosis that, in the opinion of the Investigator, requires treatment with approved therapies\n* Prebronchodilator forced expiratory volume in 1 second (FEV1)\u002FFVC \\\u003C0.7 at Visit 1b\n* HRCT findings consistent with probable or definite usual interstitial pneumonia (UIP) pattern\n* Any medical condition that is known to predispose to the development of pulmonary fibrosis (e.g. known connective tissue disease)\n* Prior or current use of nerandomilast, nintedanib, or pirfenidone Further exclusion criteria apply.","ALL","40 Years",{"count":19,"type":20},80,"ESTIMATED","INTERVENTIONAL",[23],"PHASE3","This study is open to people aged 40 years or older who have at least 1 family member with pulmonary fibrosis. Pulmonary fibrosis is a condition where lung tissue becomes scarred, making it harder to breathe. People can join if a lung scan shows early changes in the lung, called interstitial lung abnormalities, which may lead to lung scarring. People with family members who have pulmonary fibrosis are more likely to develop it themselves. That is why it is important to check early for lung changes and find ways to prevent the condition from getting worse. The purpose of this study is to find out whether a medicine called nerandomilast can help slow down changes in the lung in people with a family history of pulmonary fibrosis.\n\nParticipants are put into one of 2 groups randomly, which means the group is chosen by chance. One group takes nerandomilast tablets, and the other group takes placebo tablets. Placebo tablets look like nerandomilast tablets but do not contain any medicine. Participants take a tablet twice a day for about 2 to 3 years. There is a 3 out of 5 chance that participants will receive nerandomilast instead of the placebo.\n\nParticipants are in the study for about 2 to 3 years. Participants visit the study site multiple times: more frequently during the first 2 years (about every 3 months), and then every 6 months thereafter. In the 3rd year, participants also have phone calls with the site staff every 3 months.\n\nDoctors regularly test lung function and take chest scans to see if the treatment works. The results are compared between the 2 groups to see if nerandomilast helps. The doctors also check participants' health and take note of any unwanted effects.",[26,27,28],"Familial Pulmonary Fibrosis","Interstitial Lung Abnormalities","Interstitial Lung Diseases","RECRUITING","2026-06-23",{"date":32,"type":33},"2026-06-24","ACTUAL",{"date":35,"type":33},"2026-02-10",{"date":37,"type":20},"2029-05-23",{"name":39,"class":40},"Boehringer Ingelheim","INDUSTRY",56,{"id":43,"slug":44,"hasResults":11,"nctId":45,"briefTitle":46,"officialTitle":46,"acronym":4,"eligibilityCriteria":47,"healthyVolunteers":11,"sex":16,"minAge":17,"maxAge":48,"enrollmentInfo":49,"targetDuration":4,"studyType":51,"phases":4,"briefSummary":52,"conditions":53,"keywords":4,"overallStatus":29,"whyStopped":4,"lastUpdateSubmitDate":56,"lastUpdatePostDateStruct":57,"startDateStruct":59,"completionDateStruct":61,"leadSponsor":63,"locationsCount":66},"100319340","mechanisms-of-familial-pulmonary-fibrosis-100319340","NCT03437486","Mechanisms of Familial Pulmonary Fibrosis","Eligibility Requirements:\n\n1. Bloodline members of an affected individual from a family in which two or more members of a family are known to have Idiopathic Interstitial Pneumonia (IIP) and who have no personal diagnosis of IIP or IPF\n2. Sibling or adult child of an affected individual\n\nExclusion Criteria:\n\n1. Inability to understand the requirements of the study or be unwilling to provide written informed consent (as evidenced by signature on an informed consent document approved by the IRB).\n2. Inability to travel to Nashville for 1-2 outpatient visits and\u002For complete a written or online version of the Interstitial Lung Disease Questionnaire\n3. Age \\\u003C 40 or \\>75 years old. If the affected relative was younger than 50 years old at the time of IIP diagnosis, potential subjects between age 18 and 40 years may participate when they are up to 10 years younger than the age at relative's diagnosis.\n4. Underlying disease with signs and symptoms that could be confused with IIP or IPF symptoms (i.e., rheumatoid arthritis or other connective tissue diseases, occupational lung disease, chemotherapy, etc.)\n5. Thought to be unsuitable for participation in the study in the opinion of the investigator","75 Years",{"count":50,"type":20},750,"OBSERVATIONAL","This a prospective, longitudinal study of first-degree family members of patients diagnosed with familial interstitial pneumonia (FIP). FIP is the familial form of idiopathic pulmonary fibrosis (IPF), which is defined as 2 or more bloodline relatives which have a diagnosis of idiopathic interstitial pneumonia (IIP). The most common form of idiopathic interstitial pneumonia in FIP families is IPF (approximately 70%). The inheritance pattern in FIP is consistent with autosomal dominant inheritance with incomplete penetrance. Therefore, individuals in this study have approximately 50% risk of carrying a disease-associated allele. The causative gene is currently only known approximately 20% of families. The main goal of this longitudinal study is to better establish the natural history of FIP and to identify risk factors for later development of symptomatic disease. The investigators' plan is to follow these at-risk individuals with yearly questionnaires and planned in person 2 year follow-ups through age 75 or until they develop symptomatic FIP.",[26,54,55],"Idiopathic Pulmonary Fibrosis","Familial Interstitial Pneumonia","2025-12-26",{"date":58,"type":33},"2025-12-31",{"date":60,"type":33},"2009-01-01",{"date":62,"type":20},"2030-01-30",{"name":64,"class":65},"Vanderbilt University Medical Center","OTHER",1,{"id":68,"slug":69,"hasResults":11,"nctId":70,"briefTitle":71,"officialTitle":72,"acronym":73,"eligibilityCriteria":74,"healthyVolunteers":75,"sex":16,"minAge":76,"maxAge":4,"enrollmentInfo":77,"targetDuration":4,"studyType":51,"phases":4,"briefSummary":79,"conditions":80,"keywords":4,"overallStatus":29,"whyStopped":4,"lastUpdateSubmitDate":81,"lastUpdatePostDateStruct":82,"startDateStruct":84,"completionDateStruct":86,"leadSponsor":88,"locationsCount":66},"100612295","gen-fpf-genetic-exploration-of-familial-pulmonary-fibrosis-100612295","NCT07251725","GEN-FPF: Genetic Exploration of Familial Pulmonary Fibrosis","Unravelling the Genetic Basis of Familial Pulmonary Fibrosis: A Next-Generation Sequencing Approach to Fibrogenesis and Surfactant Disorder Genes","GEN-FPF","Inclusion Criteria:\n\nDiagnosis of Familial Pulmonary Fibrosis (FPF):\n\nAt least two individuals from the same family (first-degree relatives) diagnosed with pulmonary fibrosis based on clinical, radiological, or histopathological criteria (e.g., HRCT pattern consistent with usual interstitial pneumonia, UIP).\n\nDefinite or probable FPF diagnosis, according to international classification criteria and verified family history of disease.\n\nAge:\n\nAdults aged 18 years or older at the time of enrollment.\n\nInformed Consent:\n\nAbility and willingness to provide written informed consent (or consent provided by a legally authorized representative).\n\nWillingness to participate in genetic testing, clinical evaluations, and longitudinal follow-up.\n\nAvailability of Family Members:\n\nAffected family members with pulmonary fibrosis willing to provide blood samples and clinical information.\n\nUnaffected first-degree relatives willing to participate in genetic testing and family history documentation.\n\nIdiopathic Pulmonary Fibrosis (IPF) Cohort:\n\nIndividuals with a confirmed diagnosis of idiopathic pulmonary fibrosis (IPF) according to ATS\u002FERS 2018 criteria, enrolled as a comparative (non-familial) cohort.\n\nExclusion Criteria:\n\nNon-Familial Pulmonary Fibrosis:\n\nIndividuals with isolated, sporadic pulmonary fibrosis (without a family history) who are not part of the defined IPF control group.\n\nOther Significant Pulmonary Diseases:\n\nPresence of pulmonary diseases unrelated to fibrosis (e.g., chronic obstructive pulmonary disease, asthma, cystic fibrosis, or active pulmonary infection).\n\nRefusal or Withdrawal of Consent:\n\nIndividuals unwilling to provide or maintain informed consent for participation, genetic testing, or long-term data use.",true,"18 Years",{"count":78,"type":20},126,"Pulmonary fibrosis (PF) is a progressive lung disease marked by tissue scarring and impaired breathing. Familial pulmonary fibrosis (FPF) makes up 10-20% of PF cases and shares features with idiopathic PF (IPF), but the genetic causes of FPF are not fully understood.\n\nThis study focuses on uncovering the genetic basis of FPF by analyzing families with multiple affected members. It targets genes involved in fibrogenesis and surfactant disorders, as familial cases often appear earlier and progress more rapidly than sporadic ones.\n\nUnderstanding FPF genetics could:\n\n1. Identify new genetic markers for early diagnosis and prognosis.\n2. Improve genetic counseling and preventive strategies for affected families.\n3. Reveal therapeutic targets for personalized treatments.\n4. Highlight shared molecular pathways between familial and idiopathic PF, potentially benefiting a broader patient group.\n\nIn summary, the study aims to deepen our understanding of FPF genetics to improve diagnosis, counseling, and treatment for both familial and idiopathic forms of pulmonary fibrosis.",[26],"2025-11-24",{"date":83,"type":33},"2025-11-26",{"date":85,"type":33},"2025-09-17",{"date":87,"type":20},"2028-09",{"name":89,"class":65},"Fondazione IRCCS Policlinico San Matteo di Pavia",{"id":91,"slug":92,"hasResults":11,"nctId":93,"briefTitle":94,"officialTitle":94,"acronym":95,"eligibilityCriteria":96,"healthyVolunteers":75,"sex":16,"minAge":76,"maxAge":4,"enrollmentInfo":97,"targetDuration":4,"studyType":51,"phases":4,"briefSummary":99,"conditions":100,"keywords":4,"overallStatus":101,"whyStopped":4,"lastUpdateSubmitDate":102,"lastUpdatePostDateStruct":103,"startDateStruct":105,"completionDateStruct":107,"leadSponsor":109,"locationsCount":4},"100556133","clinical-genetics-and-screening-for-idiopathic-pulmonary-fibrosis-100556133","NCT06521125","Clinical Genetics and Screening for Idiopathic Pulmonary Fibrosis","GENESI","Criteria for PATIENTS:\n\nInclusion Criteria:\n\n1. patients aged ≥18 years when signing the informed consent\n2. diagnosis of IPF based on 2022 ATS\u002FERS\u002FJRS\u002FALAT Guidelines as confirmed by the investigator based on chest HRCT scan and if available surgical lung biopsy\n3. diagnosis of FPF defined as the presence of fibrotic ILD in at least two members of the same biological family\n4. at least one 1st degree relative \\>40 years of age.\n\nExclusion Criteria:\n\n1. patients with Interstitial Lung Diseases other than Idiopathic Pulmonary Fibrosis, including but not limited to patients with granulomatous lung disease, autoimmune\u002Fcollagen vascular disease associated interstitial lung disease, and drug induced interstitial lung disease\n2. unwilling or unable to sign informed consent\n\nCriteria for FIRST DEGREE BIOLOGICAL RELATIVES:\n\nInclusion Criteria:\n\na. subjects aged ≥40 years\n\nExclusion Criteria:\n\n1. previous diagnosis of IPF\n2. a history of severe or poorly controlled anxiety, severe or poorly controlled depression according to the opinion of the investigators, suicidal ideation, or other psychiatric illness requiring hospitalization\n3. unwilling or unable to sign informed consent 400 first-degree relatives of participating patients will be recruited",{"count":98,"type":20},600,"Background:\n\nIdiopathic pulmonary fibrosis (IPF) is the most common and severe form of interstitial lung disease. Between 2% and 20% of patients with IPF have a family history of the disease, which is considered the strongest risk factor. Therefore, genetic testing has been increasingly considered as a potential tool to identify patients at risk of developing IPF.\n\nAccording to some studies, genetic testing (particularly of MUC5B and TERT mutations) could be useful to rapidly identify unidentified and\u002For asymptomatic individuals (in families as well as in the general population) who have interstitial lung anomalies (ILA) that may indicate a initial stage of pulmonary fibrosis. Finding efficient screening methods and associated targeted treatments for IPF may be essential to improving the prognosis and quality of life of those suffering from this disease.\n\nObjectives of the study:\n\nThe study involves two populations of study subjects:\n\n* patients with FPF and sporadic IPF\n* first-degree relatives of patients with FPF and sporadic IPF (biological relatives, not spouses)\n\nThe primary objective is to determine the prevalence rates of interstitial lung abnormalities in at-risk relatives of patient with IPF and FPF.\n\nStudy design:\n\nMulticenter, cross-sectional study without drug and without device conducted in two major Italian tertiary referral hospitals.\n\nThe entire project is expected to last 24 months.",[26,54],"NOT_YET_RECRUITING","2024-07-22",{"date":104,"type":33},"2024-07-25",{"date":106,"type":20},"2024-09-01",{"date":108,"type":20},"2026-09-01",{"name":110,"class":65},"Fondazione Policlinico Universitario Agostino Gemelli IRCCS"]