Rare Oncology News

Disease Profile

Bronchiolitis obliterans

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

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ICD-10

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Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Obliterative bronchiolitis

Categories

Immune System Diseases; Lung Diseases; RDCRN

Summary

Bronchiolitis obliterans is an inflammatory condition that affects the lung's tiniest airways, the bronchioles. In affected people, the bronchioles may become damaged and inflamed leading to extensive scarring that blocks the airways. Signs and symptoms of the condition include a dry cough; shortness of breath; and/or fatigue and wheezing in the absence of a cold or asthma.[1][2][3] Many different chemicals (such as nitrogen oxides, ammonia, welding fumes or food flavoring fumes) and respiratory infections can cause lung injury that leads to bronchiolitis obliterans. It can also be associated with rheumatoid arthritis and graft-versus-host disease following a lung or hematopoietic cell transplantation.[3] While there is no way to reverse the disease, treatments are available that may stabilize or slow the progression.[1][3]

Another similarly named disease, bronchiolitis obliterans organizing pneumonia, is a completely different disease.

Symptoms

Signs and symptoms of bronchiolitis obliterans generally develop approximately two to eight weeks after exposure to toxic fumes or a respiratory illness. Affected people may experience a dry cough, shortness of breath, and/or wheezing. Fatigue and wheezing in the absence of a cold or asthma may also be noted.[1][2][3] Symptoms generally progress slowly over weeks to months.[3]

Cause

Bronchiolitis obliterans may result from lung injury caused by a variety of different chemicals and respiratory infections. Inhaled chemicals known to irritate the lungs and lead to the condition include chlorine; ammonia; oxides of nitrogen or sulfur dioxide; welding fumes; or food flavoring fumes (such as diacetyl). Respiratory infections caused by respiratory syncytial virus, adenovirus, or Mycoplasma pneumonia have been linked with the condition.[1][3]

Bronchiolitis obliterans can also be associated with rheumatoid arthritis and graft-versus-host disease following a lung or hematopoietic cell transplantation.[3]

Diagnosis

A diagnosis of bronchiolitis obliterans is often suspected based on the presence of characteristic signs and symptoms. Additional testing can then be ordered to support and confirm the diagnosis. These tests may include:[1][3]

Treatment

Although there is no cure for bronchiolitis obliterans, treatment may help stabilize or slow its progression. The best treatment options and the effectiveness of the selected treatments vary based on the underlying cause and the severity of the condition. Medications often prescribed to people with bronchiolitis obliterans include certain types of antibiotics (called macrolide antibiotics), corticosteroids, and immunosuppressive drugs. In severe cases, lung transplantation may be recommended. Therapies such as cough suppressors or supplemental oxygen can also be given to manage the symptoms associated with the condition.[1][3]

Organizations

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 Supporting this Disease

    Learn more

    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.

    Where to Start

      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.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Bronchiolitis obliterans. Click on the link to view a sample search on this topic.

        References

        1. Bronchiolitis Obliterans. National Jewish Health. February 2016; https://www.nationaljewish.org/conditions/bronchiolitis.
        2. Weigt SS, DerHovanessian A, Wallace WD, Lynch JP 3rd, Belperio JA. Bronchiolitis obliterans syndrome: the Achilles' heel of lung transplantation. Semin Respir Crit Care Med. June 2013; 34(3):336-351. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4768744/.
        3. King TE. Bronchiolitis in adults. UpToDate. March 2016; https://www.uptodate.com/contents/bronchiolitis-in-adults.

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