Aspergillus and CF
Aspergillus is very common in our environment. Most people breathe in Aspergillus spores every day without getting sick. However, people with cystic fibrosis or other diseases that weaken their immune systems, are at a higher risk of developing health problems because of Aspergillus. Some types of allergic bronchopulmonary aspergillosis (ABPA) are mild and others are very serious.
ABPA affects about 2% to 19% of the people with CF. It causes inflammation in the lungs and can cause further scarring and fibrosis if untreated.
ABPA is more common in people older than 6 years. People with CF with the following characteristics are more likely to be diagnosed with ABPA compared to those who are not:
- Have decreased lung function
- Have allergies
- Have asthma
- Have sputum that grows a Pseudomonas species
Although the symptoms of ABPA are similar to CF symptoms, if you have ABPA, your health often gets worse without a clear reason or you may not respond to antibiotics that are used to treat typical CF exacerbations. Wheezing is more common in people with CF who have ABPA, but because the symptoms of ABPA and CF overlap, symptoms alone cannot be used to make the diagnosis.
Diagnosing ABPA in people with CF is difficult. It may often take longer because many of the criteria used to diagnose ABPA are the same as the usual symptoms of CF. Below is a list of the criteria:
- Symptoms that are not related to other problems (see above)
- Total serum immunoglobulin E (IgE) greater than 500 international units (IU) per milliliter
- Immediate positive skin test to an Aspergillus species
- Positive laboratory test (precipitin to Aspergillus fumigatus [A. fumigatus] or immunoglobulin G [IgG] antibody against A. fumigatus)
- An abnormal chest X-ray or computed tomography (CT) scan or change from previous exam that suggests ABPA
- Blood work showing an elevation in eosinophilia, suggesting an allergy
It is important to mention that many people with CF have a sputum culture with Aspergillus. This does not mean you have ABPA. Your body's (immune system) response to Aspergillus and your symptoms will determine if you have ABPA.
Treatment for ABPA involves prevention and treatment of acute episodes, or flare-ups. Treatment of ABPA has two parts. Inflammation in the lung is treated with corticosteroids. Fungal colonization is treated with antifungal medicines.
Although there are no studies for the use of anti-fungals in people with CF with ABPA flare-ups, reducing the fungal burden in the respiratory tract may reduce long-term risk of disease progression. Further research is needed.
Oral corticosteroid pills or liquid, such as prednisolone (brand names include Pediapred® and Prelone®), prednisone tablets, and methylprednisolone (brand names include Medrol®) tablets, have anti-inflammatory effects. The course of medicine is much longer than the short courses used for asthma. An individual with ABPA may use steroids for many weeks and be slowly weaned off of them while symptoms and lung function studies are checked. Once all the symptoms have gone away, the steroids are often stopped. Although inhaled steroids are not used to treat ABPA flare-ups, they are often used between flare-ups to help control symptoms.
Side effects of oral and liquid steroids are related to the amount and length of time a person takes them. Side effects of taking corticosteroids may include:
- Increased appetite
- Weight gain
- Round moon face
- Red cheeks
- Changes in mood
- Increased risk of infection
- Lower bone density
Antifungal medicines like itraconazole (brand names include Sporanox®) reduce the amount of fungus in the lungs. This may lower the chance of the disease getting worse over time. Itraconazole is used one to two times a day for about three to six months, or until all symptoms have gone away. It is better absorbed when taken on an empty stomach. If you are on an acid-reducing medicine (brand names Prilosec, Nexium®, Protonix®, Pepcid®, Zantac®), you should take itraconazole with eight ounces of an acidic beverage, such as cola or orange juice to make sure you are able to absorb all of the medicine.
Side effects of taking itraconazole may include:
- Stomach upset
Because steroids can have significant side effects and ABPA can be difficult to treat, CF providers may offer a therapy that targets one particular part of the immune system (so-called “biologic” therapies). One such therapy, omalizumab (Xolair®), has been used to target IgE, which is often very elevated in patients with ABPA. This therapy has not been formally studied in people with CF with ABPA.
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