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.
Highlights
Aspergillus fumigatus (A. fumigatus) is the most common ubiquitous airborne fungus, which causes allergic bronchopulmonary aspergillosis (ABPA) [1]
Acute/subacute clinical worsening defined as cough, increased amount of sputum or changing in color of sputum, wheeze, dyspnea, the onset of new fever, weight loss, exercise-induced asthma and decrease in pulmonary function, that does not respond to appropriate treatment and is not explained with another etiology [3]
In a recent study on children with cystic fibrosis (CF) reported that 75% of ABPA patients had eosinophil count >400 cells/μL and 40% of them having counts >1,000 cells/μL, while none of the patients in the A. fumigatus sensitized and non-sensitized groups had eosinophilia and these findings suggested that eosinophil count could be a specific biomarker for ABPA in children [56]
Summary
Aspergillus fumigatus (A. fumigatus) is the most common ubiquitous airborne fungus, which causes allergic bronchopulmonary aspergillosis (ABPA) [1]. ABPA is believed to be underdiagnosed, especially in developing countries, because its clinical features are much the same as cystic fibrosis (CF). In asthmatic patients the prevalence is reported to be about 1 to 2% and is more common in adults than in children [4, 5]. The prevalence is higher in CF patients than in asthmatic patients. Immunopathogenesis, clinical features, diagnosis, and current treatment modalities have been tried to be summarized. This review is based on the studies and case reports with the pediatric age group, some studies in adults and asthmatics have been mentioned due to limited number of publications in children. By the way the diagnosis and treatment in children are not much different from adults and the treatment in CF is similar to asthmatics [8]
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