Idiopathic bronchiectasis

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ORPHA:60033OMIM:211400J47
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Overview

Idiopathic bronchiectasis is a long-term lung condition where the airways (the tubes that carry air in and out of your lungs) become permanently widened, scarred, and damaged. 'Idiopathic' means that doctors cannot find a clear underlying cause for why this happened. The damaged airways have trouble clearing mucus normally, which leads to mucus building up and creating a breeding ground for repeated lung infections. Over time, this cycle of infection and inflammation causes more damage to the lungs. The most common symptoms include a daily cough that brings up mucus or phlegm, frequent chest infections, shortness of breath, and sometimes coughing up blood. Some people also feel very tired and may lose weight over time. The condition can range from mild — where a person lives a fairly normal life with some daily management — to more severe, where lung function is significantly reduced. There is no cure for idiopathic bronchiectasis, but treatments can help manage symptoms, reduce infections, and slow the progression of lung damage. Treatment usually involves airway clearance techniques, inhaled medications, and antibiotics during flare-ups. With good management, many people with bronchiectasis can maintain a reasonable quality of life.

Key symptoms:

Daily cough that produces mucus or phlegmFrequent chest infections or pneumoniaShortness of breath, especially with activityCoughing up blood (small or large amounts)Wheezing or a rattling sound when breathingChest pain or tightnessFeeling very tired or fatiguedUnintended weight lossClubbing of the fingers (fingertips become rounded and enlarged)Fever and chills during infection flare-upsReduced ability to exercise or be physically active

Clinical phenotype terms (19)— hover any for plain English
Abnormal respiratory system physiologyHP:0002795Respiratory tract infectionHP:0011947Productive coughHP:0031245Recurrent lower respiratory tract infectionsHP:0002783Recurrent Haemophilus influenzae infectionsHP:0005376CracklesHP:0030830Reduced FEV1/FVC ratioHP:0030877EmphysemaHP:0002097Acute infectious pneumoniaHP:0011949HalitosisHP:0100812ClubbingHP:0001217
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

10 events
Sep 2025A Cohort for Inflammatory Respiratory Diseases: From Phenotyping to Personalised Medicine

CHU de Reims — NA

TrialRECRUITING
May 2025Validation and Clinical Utility of the Lung Sliding Index (LSI) for Differentiating Pulmonary Diseases

Assiut University — NA

TrialRECRUITING
Apr 2025Perioperative Risk Factors Related to the Prognosis of Lung Transplantation: A Retrospective Study

Zhejiang University

TrialNOT YET RECRUITING
Nov 2024Expand Pulmonary Rehabilitation to Other Chronic Respiratory Diseases Than COPD

Copenhagen University Hospital, Hvidovre — NA

TrialACTIVE NOT RECRUITING
Sep 2024Comparison of Two Antibiotic Regimens for the Treatment of Early Airways Infection With PA in Adults With Bronchiectasis

Centre Hospitalier Intercommunal Creteil — PHASE2

TrialRECRUITING
Apr 2023Clinical Outcomes of BronchiEctaSis in Taiwan: A Multicenter Prospective Cohort (BEST Study)

National Taiwan University Hospital

TrialACTIVE NOT RECRUITING
Apr 2023A Study of the Clinical Benefit of Tobramycin Inhalation Solution

Qianfoshan Hospital

TrialACTIVE NOT RECRUITING
Mar 2023Study of Bronchiectasis and Associated Cardiovascular Comorbidities

Assiut University

TrialRECRUITING
Jan 2023Development of a Canadian Bronchiectasis and NTM Database

University of Calgary

TrialRECRUITING
Apr 2017Controls for Respiratory Diseases

Seoul National University Hospital

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

1 available

Acetylcysteine

ACETYLCYSTEINE· Hospira, Inc.

adjuvant therapy for patients with abnormal, viscid, or inspissated mucous secretions in bronchiectasis

Clinical Trials

10 recruitingView all trials with filters →
Phase 21 trial
Comparison of Two Antibiotic Regimens for the Treatment of Early Airways Infection With PA in Adults With Bronchiectasis
Phase 2
Actively Recruiting
PI: Camille JUNG, MD · Sites: Amiens; Bordeaux +16 more · Age: 1899 yrs
N/A3 trials
Expand Pulmonary Rehabilitation to Other Chronic Respiratory Diseases Than COPD
N/A
Active
PI: Henrik Hansen, PhD, Msc, PT (Dept. of Respiratory Medicine, University Hospital) · Sites: Copenhagen, Greater Copenhagen; Copenhagen, Greater Copenhagen +3 more · Age: 4099 yrs
A Cohort for Inflammatory Respiratory Diseases: From Phenotyping to Personalised Medicine
N/A
Actively Recruiting
PI: Jeanne-Marie PEROTIN-COLLARD · Sites: Reims · Age: 1899 yrs
Validation and Clinical Utility of the Lung Sliding Index (LSI) for Differentiating Pulmonary Diseases
N/A
Actively Recruiting
PI: Ahmad M. Shaddad, MD (Assiut University) · Sites: Asyut · Age: 1899 yrs
Other6 trials
Study of Bronchiectasis and Associated Cardiovascular Comorbidities
Actively Recruiting
PI: Ahmad Shaddad (Assuit University Faculty of Medicine) · Sites: Asyut · Age: 1880 yrs
Seoul National University Airway Registry
Actively Recruiting
PI: Chul-Gyu Yoo, M.D. (Seoul National University Hospital) · Sites: Seongnam-si, Gyeonggi-do; Seoul +1 more · Age: 2090 yrs
Controls for Respiratory Diseases
Actively Recruiting
PI: Chang-Hoon Lee, M.D. (Seoul National University Hospital) · Sites: Seoul, Seoul · Age: 1990 yrs
Clinical Outcomes of BronchiEctaSis in Taiwan: A Multicenter Prospective Cohort (BEST Study)
Active
PI: Hao-Chien Wang (National Taiwan University Hospital) · Sites: Taipei, Taipei · Age: 2099 yrs
Development of a Canadian Bronchiectasis and NTM Database
Actively Recruiting
· Sites: Calgary, Alberta · Age: 18120 yrs
A Study of the Clinical Benefit of Tobramycin Inhalation Solution
Active
PI: Rui Yang, MD (Qianfoshan Hospital) · Sites: Jinan, Shandong

Specialists

11 foundView all specialists →
MM
Michele Mondoni, MD
Specialist
PI on 1 active trial
SP
Stefano Centanni, MD, PhD
Specialist
PI on 1 active trial
DF
Dr W Flowers
Specialist
PI on 1 active trial1 Idiopathic bronchiectasis publication
JD
Jin-fu Mr Xu, Doctor
Specialist
PI on 1 active trial
EM
Elbert P Trulock III, MD
Specialist
PI on 1 active trial
HP
Henrik Hansen, PhD, Msc, PT
FAIRFIELD, CA
Specialist
PI on 1 active trial
CM
Chang-Hoon Lee, M.D.
Specialist
PI on 1 active trial
CM
Camille JUNG, MD
Amiens
Specialist

Rare Disease Specialist

YO
Yeon-Mok Oh
Specialist
2 Idiopathic bronchiectasis publications
HL
Hyun Lee
Specialist
2 Idiopathic bronchiectasis publications

Treatment Centers

8 centers
⚗️ Trial Site

CHU Nantes

📍 Nantes

👤 Sebastien HASCOËT

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

Travel Grants

No travel grants are currently matched to Idiopathic bronchiectasis.

Search all travel grants →NORD Financial Assistance ↗

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Latest news about Idiopathic bronchiectasis

Disease timeline:

New recruiting trial: Controls for Respiratory Diseases

A new clinical trial is recruiting patients for Idiopathic bronchiectasis

New trial: Comparison of Two Antibiotic Regimens for the Treatment of Early Airways Infection With PA in Adults

Phase PHASE2 trial recruiting. Antibiotic monotherapy treatment and follow-up

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What tests do I need to make sure there is no underlying cause for my bronchiectasis that has been missed?,What airway clearance technique is best for me, and can I be referred to a respiratory physiotherapist?,Should I have antibiotics at home to start early if I get a chest infection, and which antibiotic should I use?,Is long-term antibiotic therapy (like azithromycin) appropriate for me to reduce the number of infections I get?,How often should I have lung function tests and CT scans to monitor my condition?,Are there any clinical trials for bronchiectasis that I might be eligible for?,What vaccinations do I need, and how can I reduce my risk of catching respiratory infections?

Common questions about Idiopathic bronchiectasis

What is Idiopathic bronchiectasis?

Idiopathic bronchiectasis is a long-term lung condition where the airways (the tubes that carry air in and out of your lungs) become permanently widened, scarred, and damaged. 'Idiopathic' means that doctors cannot find a clear underlying cause for why this happened. The damaged airways have trouble clearing mucus normally, which leads to mucus building up and creating a breeding ground for repeated lung infections. Over time, this cycle of infection and inflammation causes more damage to the lungs. The most common symptoms include a daily cough that brings up mucus or phlegm, frequent chest

How is Idiopathic bronchiectasis inherited?

Idiopathic bronchiectasis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Are there clinical trials for Idiopathic bronchiectasis?

Yes — 10 recruiting clinical trials are currently listed for Idiopathic bronchiectasis on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Idiopathic bronchiectasis?

11 specialists and care centers treating Idiopathic bronchiectasis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.