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Disease Profile

Allergic bronchopulmonary aspergillosis

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

US Estimated

Europe Estimated

Age of onset

Childhood

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ICD-10

B44.1+ J99.8*

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)

ABPA; Allergic bronchopulmonary mycosis; Aspergillosis, allergic bronchopulmonary;

Categories

Fungal infections; Immune System Diseases; Lung Diseases

Summary

Allergic bronchopulmonary aspergillosis is an allergic lung reaction to a type of fungus (Aspergillus fumigatus).[1][2] Symptoms vary, but may include wheezing, bronchial hyperreactivity, hemoptysis, productive cough, low-grade fever, malaise, and weight loss.[2][3] It is more common in people who have asthma or cystic fibrosis.[1][2] The recommended treatment for allergic bronchopulmonary aspergillosis is itraconazole, a prescription antifungal medication. Oral corticosteroids, like prednisone, may also be helpful.[2][3] The clinical course of allergic bronchopulmonary aspergillosis is variable. Many people with this condition can be stabilized for long periods when treated. However, only about 50% of patients achieve long-lasting remission and many require recurrent courses of treatment.[2]

Symptoms

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.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Abnormal eosinophil morphology
0001879
Asthma
0002099
Cough
Coughing
0012735
30%-79% of people have these symptoms
Bronchiectasis
Permanent enlargement of the airways of the lungs
0002110
Cerebral cortical atrophy
Decrease in size of the outer layer of the brain due to loss of brain cells
0002120
Low-grade fever
Mild fever
0011134
Weight loss
0001824
5%-29% of people have these symptoms
Abnormal fingernail morphology
Abnormal fingernails
Abnormality of the fingernails

[ more ]

0001231
Emphysema
0002097
Hemoptysis
Coughing up blood
0002105
Pulmonary arterial hypertension
Increased blood pressure in blood vessels of lungs
0002092
Respiratory insufficiency
Respiratory impairment
0002093
Percent of people who have these symptoms is not available through HPO
Abnormality of the immune system
Immunological abnormality
0002715
Autosomal dominant inheritance
0000006

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

    • You can obtain information on this topic from the Centers for Disease Control and Prevention (CDC). The CDC is recognized as the lead federal agency for developing and applying disease prevention and control, environmental health, and health promotion and education activities designed to improve the health of the people of the United States.
    • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
    • The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • 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 Allergic bronchopulmonary aspergillosis. Click on the link to view a sample search on this topic.

        References

        1. Miles MC, Peters SP. Allergic Bronchopulmonary Aspergillosis. Merck Manual Consumer Version. https://www.merckmanuals.com/home/lung-and-airway-disorders/asthma/allergic-bronchopulmonary-aspergillosis. Accessed 5/21/2015.
        2. Agarwal R. Allergic bronchopulmonary aspergillosis. Orhanet. May 2012; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=1164. Accessed 5/21/2015.
        3. Aspergillosis. Centers for Disease Control and Prevention (CDC). September 8, 2014; https://www.cdc.gov/fungal/diseases/aspergillosis/index.html. Accessed 5/21/2015.