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Allergic bronchopulmonary aspergillosis (ABPA) is caused by an exaggerated T(H)2 response to the ubiquitous mold Aspergillus fumigatus. ABPA develops in a small fraction of patients with cystic fibrosis and asthma, suggesting that intrinsic host defects play a major role in disease susceptibility. This article reviews current understanding of the immunopathology, clinical and laboratory findings, and diagnosis and management of ABPA. It highlights clinical and laboratory clues to differentiate ABPA from cystic fibrosis and asthma, which are challenging given clinical and serologic similarities. A practical diagnostic algorithm and management scheme to aid in the treatment of these patients is outlined.

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Background: Hereditary angioedema types I and II are caused by a functional deficiency of C1 inhibitor (C1-INH), leading to overproduction of bradykinin. The current functional diagnostic assays employ inhibition of activated C1s; however, an alternative, more physiologic method is desirable. Methods: ELISAs were developed using biotinylated activated factor XII (factor XIIa) or biotinylated kallikrein bound to avidin-coated plates. Incubation with plasma was followed by detection of bound C1-INH. Results: After standard curves were developed for quantification of C1-INH, serial dilutions of normal plasma were employed to validate the ability to detect known concentration of C1-INH in the plasma as a percent of normal. Hereditary angioedema (HAE) types I and II were then tested. The level of functional C1-INH in all HAE types I and II plasma tested was less than 40% of our normal control. This was evident regardless of whether we measured factor XIIa-C1-INH or kallikrein-C1-INH complexes, and the two assays were in close agreement. By contrast, testing the same samples utilizing the commercial method (complex ELISA, Quidel Corp.) revealed the levels of C1-INH between 0 and 57% of normal (mean, 38%), and 42 samples were considered equivocal (four controls and 38 patients). Conclusions: Diagnosis of HAE types I and II can be ascertained by inhibition of enzymes of the bradykinin-forming cascade, namely factor XIIa and kallikrein. Either method yields functional C1-INH levels in patients with HAE (types I and II) that are clearly abnormal with less variance or uncertainty than the commercial method.