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Allergic bronchopulmonary aspergillosis

Diagnosis

ABPA is caused by an abnormal immune response to Apergillus fumigatus. It affects between 5 to 15% of patients with CF, with a peak in teenage years. Clinically it may present with an acute deterioration, and should be considered as a contributor to any respiratory exacerbation, or insidiously with no obvious symptoms initially. Untreated it can result in worsening bronchiectasis and loss of lung function.
We screen for ABPA each year at annual review and during every chest exacerbation needing ivs antibiotics by assessing: history of wheeze, changes on CXR and total IgE (if ABAP is present this will be more than 500iu/L or usually more than 1000iu/L). The presence of Aspergillus in the sputum in not discriminatory, but should be taken into account. The number of criteria required to make a diagnosis is still a matter for debate. If the child has symptoms of wheeze or new changes on the CXR then it is easier to justify treatment. If the child is asymptomatic it is more difficult. If the IgE is more than 1000iu, treatment should be considered.
Scediosporum apiospermum is the second most commonly isolated fungus and can also an allergic bronchopulmonary mycosis.

Treatment

Treatment is prednisolone plus itraconazole. Typically prednisolone (non-enteric coated) is used at 2mg/kg once daily for 2 weeks (max 40mg), and then half that dose once daily for 2 weeks. Review at 4 weeks for clinical response and repeat IgE. Further weaning can then either be by further halving of the dose every 2 weeks or moving to alternate day dosing (eg if on 20mg daily, initally move to 20mg alternate days). Dose reductions tend to occur every 2 weeks. If there is a poor response, check compliance and check that the prednisolone formulation used is non-enteric.

Itraconazole

We also routinely use Itraconazole. For patients less than 12 years give 5mg/kg bd (max 200mg bd), or over 12 years 200 mg bd orally (monitor liver function) and continue whilst they remain on steroids. Note that we recommend twice daily dosing (BNFc suggests once daily) - this is because of the very poor absorption of itraconazole, such that blood levels are frequently very low despite high doses. The capsules particularly are poorly absorbed and should be taken with an acidic liquid (e.g. coca-cola, orange juice) and food. The liquid formulation is absorbed better although it is quite unpalatable. The liquid is taken on an empty stomach. Liver function should be checked with repeat IgE levels to look for itraconazole toxicity.

Inhaled steroids are not effective in treating ABPA.

Recurrence or poor response to treatment

Recurrence of ABPA is common. Some children may either respond poorly to oral prednsiolone, or get steroid related side-effects (most teenagers are most bothered by the facial changes) and for these children iv methylprednisolone is an alternative. We use 15mg/kg (max 1gm) for 3 days every month, repeated on a minimum of 3 occasions. If Aspergillus cultures remain positive despite using itraconazole, and either ABPA or Aspergillus bronchitis is thought likely, 4 weeks of voriconazole can be considered. Absorption is better and is not influenced by gastric acid. A final alternative is nebulised amphotericin, which tastes awful, but may be tolerated for 2-4 weeks.

Steroid side effects

Patients on long-term oral steroids should be screened regularly for glucose intolerance, high blood pressure, sub-capsular cataract formation, poor growth (minimised on alternate day regime, dose taken in the morning), and warned of the increased risk of infection. It should be assumed that all children who have been on >0.5mg/kg prednisolone for >2 weeks will be adrenally suppressed, so appropriate warnings about the possible need for hydrocortisone replacement during intercurrent illness and for im hydrocortisone during vomiting illnesses when they are unable to take their steroids orally should be given. Children having prolonged (>3months) prednisolone may take longer to recover adrenal function and use of hydrocortisone replacement and synacthen tests to check recovery should be considered. Children who have not had an obvious episode of chickenpox should have their VZ titres measured, and if these are not protective should be given appropriate advice should they come into contact with chickenpox.