Elsevier

Paediatric Respiratory Reviews

Volume 24, September 2017, Pages 35-38
Paediatric Respiratory Reviews

Cystic Fibrosis: Frequently Asked Questions
Question 11: How should Allergic Bronchopulmonary Aspergillosis [ABPA] be managed in Cystic Fibrosis?

https://doi.org/10.1016/j.prrv.2016.10.001Get rights and content

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Clinical signs and symptoms of ABPA

Patients may be initially asymptomatic or minimally symptomatic with everyday subtle symptoms consistent with established CF lung disease such as slight cough, increased sputum and exercise related dyspnoea. Alternatively, the onset may be more obvious, and rarely fulminant [7]. Suspicion should be raised if there is no clinical response to conventional nebulised or intravenous antibiotic therapy. Symptoms may include increased wheezing, fever, malaise and thick sputum with brown or black

Diagnosis of ABPA

The diagnosis of ABPA is based on the presence of a combination of clinical, laboratory, and radiological findings (Figure 1) [8]. In the context of CF, the diagnosis can be difficult as most of the diagnostic criteria can be positive in the absence of ABPA. The Cystic Fibrosis Foundation Consensus Conference has established diagnostic and screening criteria for ABPA (Table 1) [7], [8]. Staging of ABPA is not often applied in CF with ABPA, although a staging system has been used for patients

Current therapeutic options

Successful management of ABPA requires an early diagnosis and prompt intervention. There are three important therapeutic considerations: Treatment of ABPA, treatment of the underlying CF lung disease and environmental control.

Intravenous pulse methylprednisolone

High-dose intravenous (IV) pulsed methylprednisolone (10-20 mg/kg/day for 3 days every 3-4 weeks) has been investigated as a potential effective alternative therapy for ABPA. In 2009, Cohen-Cymberknoh et al. performed a retrospective study in nine CF-ABPA patients (ages 7-36 years) [26]. The study compared IV methylprednisolone to conventional oral prednisolone treatment. It was concluded that pulse methylprednisolone is as effective as oral prednisone and it is relatively safe. However,

Conclusion

Oral corticosteroids are the mainstay of therapy for ABPA complicating CF. However, the use of additional treatments including oral and nebulised antifungals should be considered in relapsing or steroid-dependent ABPA cases. In cases of nonresponse, an absence of improving lung function and a lack of falling IgE levels, as well as drug-induced side effects, anti-IgE treatment could be considered [27], [28], [32].

However, more data is required to clarify the role of omalizumab before this

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