Overview
Respiratory bronchiolitis-interstitial lung disease syndrome (RB-ILD) is a rare lung condition that almost always occurs in people who smoke cigarettes, though it can occasionally affect former smokers. It is sometimes called 'smokers' lung disease' or 'respiratory bronchiolitis-associated interstitial lung disease.' In this condition, pigmented immune cells called macrophages build up inside the small airways of the lungs, causing inflammation and scarring of the delicate lung tissue. This makes it harder for the lungs to move oxygen into the bloodstream. The most common symptoms are a persistent cough and gradually worsening shortness of breath, especially during physical activity. Some people also notice crackle-like sounds when a doctor listens to their lungs. Because the symptoms come on slowly, many people do not realize something is wrong until the disease has already progressed. The good news is that RB-ILD often responds well to the most important treatment: stopping smoking. Many patients see significant improvement after quitting. In more severe cases, doctors may also prescribe corticosteroids to reduce lung inflammation. With early diagnosis and smoking cessation, the outlook for most patients is much better than for other types of interstitial lung disease.
Also known as:
Key symptoms:
Persistent dry or mild coughShortness of breath, especially during exercise or physical activityCrackling sounds in the lungs heard through a stethoscopeReduced ability to exercise or do physical tasksFatigue and low energyMild decrease in lung function on breathing testsOccasional mild chest discomfortClubbing of the fingers (thickening and rounding of fingertips) in some cases
Clinical phenotype terms (22)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Respiratory bronchiolitis-interstitial lung disease syndrome.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Respiratory bronchiolitis-interstitial lung disease syndrome.
Community
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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.How certain are you that I have RB-ILD, and do I need a lung biopsy to confirm the diagnosis?,How much improvement can I realistically expect if I stop smoking?,Do I need to take corticosteroids, and what are the risks and benefits for my specific situation?,How often should I have follow-up visits and repeat breathing tests to monitor my lung function?,Are there pulmonary rehabilitation programs you can refer me to?,What symptoms should prompt me to seek emergency care?,Are there any clinical trials for RB-ILD that I might be eligible for?
Common questions about Respiratory bronchiolitis-interstitial lung disease syndrome
What is Respiratory bronchiolitis-interstitial lung disease syndrome?
Respiratory bronchiolitis-interstitial lung disease syndrome (RB-ILD) is a rare lung condition that almost always occurs in people who smoke cigarettes, though it can occasionally affect former smokers. It is sometimes called 'smokers' lung disease' or 'respiratory bronchiolitis-associated interstitial lung disease.' In this condition, pigmented immune cells called macrophages build up inside the small airways of the lungs, causing inflammation and scarring of the delicate lung tissue. This makes it harder for the lungs to move oxygen into the bloodstream. The most common symptoms are a persi
How is Respiratory bronchiolitis-interstitial lung disease syndrome inherited?
Respiratory bronchiolitis-interstitial lung disease syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Respiratory bronchiolitis-interstitial lung disease syndrome typically begin?
Typical onset of Respiratory bronchiolitis-interstitial lung disease syndrome is adult. Age of onset can vary across affected individuals.
Which specialists treat Respiratory bronchiolitis-interstitial lung disease syndrome?
3 specialists and care centers treating Respiratory bronchiolitis-interstitial lung disease syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.