National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Diffuse panbronchiolitis


Other Names:
Panbronchiolitis, diffuse; PBLT; DPB
Categories:
Diffuse panbronchiolitis (DPB) is a rare condition characterized by inflammation of the small airways of the lungs (bronchiolitis) and chronic sinusitis. It mainly occurs among the Japanese but has been reported in other populations. Symptoms typically develop anywhere from the teenage years to the fifth decade of life and are slowly progressive over months to years. Common symptoms include chronic sinusitis, a productive cough (producing mucus), breathlessness with exertion, wheezing, and weight loss (especially as symptoms worsen). The exact cause of DPB is not known, but a variety of genetic, environmental, and systemic factors appear to contribute to the condition. Treatment involves long-term use of macrolide antibiotics, which reportedly dramatically improves the long-term outcome. If untreated, DPB leads to bronchiectasis, progressive respiratory failure and death over the course of a few years.[1]
Last updated: 3/21/2017

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
Percent of people who have these symptoms is not available through HPO
Bronchiectasis
Permanent enlargement of the airways of the lungs
0002110
Cough
Coughing
0012735
Crackles 0030830
Foam cells 0003651
Hypoxemia
Low blood oxygen level
0012418
Progressive
Worsens with time
0003676
Rhonchi 0030831
Wheezing 0030828
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Last updated: 7/1/2020
The main treatment that has been recommended for diffuse panbronchiolitis (DPB) is the use of macrolide antibiotics.[2] Erythromycin is typically the first drug of choice.[1] A different macrolide antibiotic may be used if erythromycin is not well-tolerated or if a person doesn't improve after several weeks of therapy.[1]

The best duration of macrolide therapy is not known, but most patients are treated for a minimum of six months. Therapy is continued until symptoms, imaging findings, and lung function measurements are improved or stable. In the majority of patients, therapy is stopped after two years. However, in patients with advanced disease, therapy is continued indefinitely.[1]

Other therapies, such as bronchodilator medications and bronchial hygiene (airway clearance techniques), may be useful for some people.[1]
Last updated: 3/21/2017

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Diffuse panbronchiolitis. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Talmadge E King, Jr. Diffuse panbronchiolitis. UpToDate. Waltham, MA: UpToDate; July, 2016;
  2. Lin X, Lu J, Yang M, Dong BR, Wu HM. Macrolides for diffuse panbronchiolitis. Cochrane Database Syst Rev. January, 2015; 1: