Meb. Vilar et al., Allergic bronchopulmonary aspergillosis as presenting sign of cystic fibrosis in an elderly man, ANN ALLER A, 85(1), 2000, pp. 70-73
Background: Although cystic fibrosis is the most common genetic disorder of
children, its heterogeneous spectrum of severity lends itself to underdiag
nosis in the older adult patient population where the index of suspicion is
not high.
Methods: We report a 60-year-old Hispanic man with asthma who presented wit
h progressive dyspnea and wheezing unresponsive to inhaled corticosteroid t
reatment. Additionally, he had clinical findings and a past history suggest
ive of cystic fibrosis. Skin testing, radiography and laboratory studies we
re completed to evaluate for allergic bronchopulmonary aspergillosis (ABPA)
and cystic fibrosis.
Results: Test results revealed peripheral eosinophilia and hyper IgE. Skin
testing to Aspergillus fumigatus (Af) was positive. IgG, lgM, and Af specif
ic antibodies were present. High resolution CT scan showed central bronchie
ctasis. Sweat tests were positive on two separate occasions and gene analys
is showed our patient to have a positive gene mutation at D127ON/D127ON.
Conclusion: Cystic fibrosis should be suspected in the older adult patient
with a compatible clinical presentation.