Underlying chronic granulomatous disease in a patient with bronchocentric granulomatosis

Abstract

We present a case of bronchocentric granulomatosis in a woman with no history of asthma who was colonised with Aspergillus fumigatus. A family history of chronic granulomatous disease prompted further testing that demonstrated severely depressed neutrophil oxidant production and gp91 phox deficiency compatible with the X linked carrier state of chronic granulomatous disease. Only one report of the association of these two rare diseases has previously appeared in the literature. We postulate that an ineffective immune response led to the prolonged colonisation of A fumigatus resulting in a hypersensitivity reaction that was manifest clinically as bronchocentric granulomatosis.