Diagnostic or therapeutic dilemma in patients with neutrophil disorders and allergic bronchopulmonary aspergillosis

T. M. Eppinger, P. A. Greenberger, C. Cunningham-Rundles

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Allergic bronchopulmonary aspergillosis (ABPA) is an inflammatory process of the airway caused by host response mechanisms to noninvasive colonisation of the bronchial wall with Aspergillus fumigatus. The treatment of ABPA is geared toward modifying host response rather than eliminating fungal colonisation per se; corticosteroids are considered highly effective treatment. The process usually occurs in patients who have underlying pulmonary disease such as asthma or cystic fibrosis. Diagnosis is established by the presence of radiological and/or serological criteria. Among 14 patients with primary disorders of neutrophil function we have identified 2 of 7 patients with hyper IgE syndrome (HIgE) and 2 of 7 patients with chronic granulomatous disease (CGD) who meet all serological diagnostic requirements for ABPA (IgE>1000ng/ml, serum precipitins to A.fumigatus, elevated serum IgE-a-Af, elevated serum IgG-a-Af). An additional 2 patients with CGD met all 4 criteria but their Af-specific IgE and IgG titers were lower and another two partially fulfilled the criteria. Due to their underlying disease most of these patients fulfill the radiologic criteria of bronchiectasis, central bronchiectasis or pulmonary infiltrates. Some have reversible bronchial obstruction. Thus there is diagnostic dilemma for patients with neutrophil disorders such as HIgE or CGD who fit criteria of ABPA. The therapeutic approach requires modification for patients with primary neutrophil disorders for whom drug therapy with corticosteroids alone imposes significant risks.

Original languageEnglish
Pages (from-to)A893
JournalFASEB Journal
Issue number5
StatePublished - 20 Mar 1998


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