Current Approach in the Diagnosis and Management of Allergic Bronchopulmonary Aspergillosis in Children With Cystic Fibrosis

Abstract

Allergic bronchopulmonary aspergillosis (ABPA) is a complex pulmonary disorder characterized by a hypersensitivity reaction to Aspergillus fumigatus, and almost always seen in patients with cystic fibrosis (CF) and asthma. Fungal hyphae leads to an ongoing inflammation in the airways that may result in bronchiectasis, fibrosis, and eventually loss of lung function. Despite the fact that ABPA is thought to be more prevalent in CF than in asthma, the literature on ABPA in CF is more limited. The diagnosis is challenging and may be delayed because it is made based on a combination of clinical features, and radiologic and immunologic findings. With clinical deterioration of a patient with CF, ABPA is important to be kept in mind because clinical manifestations mimic pulmonary exacerbations of CF. Early diagnosis and appropriate treatment are important in preventing complications related to ABPA. Treatment modalities involve the use of anti-inflammatory agents to suppress the immune hyperreactivity and the use of antifungal agents to reduce fungal burden. Recently, in an effort to treat refractory patients or to reduce adverse effects of steroids, other treatment options such as monoclonal antibodies have started to be used. Intensive research of these new agents in the treatment of children is being conducted to address insufficient data.

New Perspectives in the Diagnosis and Management of Allergic Fungal Airway Disease

“…Most patients with AFAD present after the fourth decade, and ABPA was regarded as rare in childhood in association with asthma, although it is well recognised in cystic fibrosis. 130 , 131 Fungal allergy is, however, a feature of severe asthma in children, and it is reasonable to assume that the sensitisation and possibly the damaging effects of AFAD start at this age. 132 Castanhinha et al found increased IL-33 expression in the airways of children with SAFS compared to non-fungal sensitised patients with therapy-resistant asthma, although they did not report how many were only sensitised to A. fumigatus rather than Alternaria and Cladosporium spp.…”

Allergic Bronchopulmonary Aspergillosis With or Without Asthmatic Symptoms?

“…Plugged and inflamed airways ultimately lead to the development of bronchiectasis [ 10 ]. The thick, golden-brown mucus expectorated, as the one seen in our case, is typical of ABPA and contains eosinophils, desquamated epithelial cells, and mucin [ 1 ].…”

“…Remission is recognized when the patient has remained asymptomatic and with stably low IgE levels for six months after terminating corticosteroid and antifungal therapy. On the other hand, an increase in total serum IgE would represent a relapse of the disease [ 1 ].…”

“…BAL did not recover and grow other infectious organisms and other fungal markers such as GM index and ß-1,3-Galactomannan were within normal parameters. Importantly, the presence of A. fumigatus or positive GM index on BAL fluid cannot be used to differentiate between ABPA or colonization by itself [ 1 , 17 ].…”

“…This disease, known as allergic bronchopulmonary aspergillosis (ABPA), typically manifests in patients who have aspergillus sensitization (AS), as well as those who suffer from other chronic pulmonary diseases including asthma and cystic fibrosis (CF). ABPA presents as an acute or subacute clinical deterioration from baseline with increased cough, sputum production or sputum color change, wheezing, dyspnea, as well as constitutional symptoms such as weight loss and fever, that fails to respond to appropriate medical therapy [ 1 ].…”