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What

Allergic Bronchopulmonary Aspergillosis (ABPA) is an allergic reaction to a type of fungus called Aspergillus fumigatus. ABPA is characterized by inflammation and narrowing of the bronchi and alveoli, which are parts of the airway. ABPA occurs only in those who are hypersensitive to this type of fungus.

Who

ABPA occurs only in those who have asthma or cystic fibrosis. It affects males and females equally, and tends to affect adults more than children.

Signs and Symptoms

Symptoms of ABPA may include: 

1. Worsening of asthma or cystic fibrosis
   
2. Wheezing (difficulty breathing)
   
3. Cough which may produce brownish colored plugs or bloody sputum
   
4. Fever ABPA can lead to complications such as widening and formation of scar tissue in the airways that may interfere with oxygen delivery to the body (bronchiectasis), coughing up large amounts of blood (hemoptysis), formation of mucus plugs that may cause some parts of the lung tissue to collapse (atelectasis), and respiratory failure. More commonly, it can lead to a persistent worsening of asthma syndrome that needs oral steroid medicines to control.

Possible Causes

ABPA is caused by inhaling the spores of A. fumigatus, a type of fungus. When the spores reach the airway, the body’s immune system attacks the spores by sending white blood cells, antibodies, and other cells that try to kill the invading spores. The airway also reacts by becoming narrower and more obstructed. A. fumigatus is very common and is found on decaying vegetation such as dead leaves, hay and compost stacks, stored grain, bird droppings, wet paint, construction materials, and in air conditioning systems. However, only people who develop the specific kind of antibody response in this disease get the syndrome. This is why the disease is rare, even though the fungus is common.

Diagnosis

The disease is similar to asthma, and occurs only in asthma and cystic fibrosis patients, so the diagnosis is sometime difficult. It is usually identified by discovering abnormalities on chest X ray in an asthma patient, or by having positive skin tests (a kind of allergy testing) to A. fumigatus. To make the diagnosis requires that specific criteria be met, including: - A history of asthma - A complete blood count (CBC) showing an increased presence of white blood cells called eosinophils - Increased eosinophils in the sputum - Chest x-ray showing an accumulation of fluid in the lungs - Positive aspergillus antigen skin test - Positive serum Aspergillus antibody test - CT scan indicating the presence of brochiectasis - Increased IgE levels in the blood (IgE activates allergic reactions to foreign substances) Not everyone will have all of these features though, so it important that the diagnosis be made by someone who feels comfortable diagnosing and treating this disease, usually an allergist or pulmonologist (lung specialist).

Treatment

ABPA is treated with prednisone or other corticosteroid medications, as well as medications that open the airway (bronchodilators). Patients need to take the steroids for at least two to four months for each exacerbation. Periodic checkups with chest x-ray and spirometry (a test that measures lung capacity) should be conducted to track the progress of treatment. Anti-fungal medication is not recommended since ABPA is an allergic reaction and not a direct fungal infection.

Prognosis

Most patients respond well to corticosteroid therapy and improve over time. However, relapse is common and requires repeat treatment. There can be periods of many years between relapses, though. Those who develop complications are at risk for persistent asthma symptoms, or bronchiectasis, a widening of the airways that can lead to respiratory failure. Treatment can prevent or limit this progression. One sign of successful treatment and a good prognosis is a long-term fall in IgE levels, though they rarely return to normal.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Community Health Care Library
Educational site that contains valuable information and definitions of related medical terms.

Medline Plus
An easy to understand resource; excellent for learning about a disease or condition.

Google Search for Allergic Bronchopulmonary Aspergillosis (ABPA)

References and Sources

www.chclibrary.org/micromed/00036780.html www.nlm.nih.gov/medlineplus/ency/article/000070.htm www.merck.com/pubs/mmanual/section6/chapter76/76d.htm