Recombinant Human Interferon- as Adjunct Therapy for Aspergillus Infection in a Patient with Chronic Granulomatous Disease

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Abstract
The hallmark of chronic granulomatous disease (CaD) is defective killing of ingested microorganisms by phagocytic cells. Invasive aspergillosis in CaD patients is particularly virulent and has a mortality rate of ∼ 50%. A patient with autosomal recessive CaD was identified who had progressive pulmonary aspergillosis that was unresponsive to conventional antifungal therapy. She was treated with recombinant human interferon-γ (rHuIFN-γ) and had a dramatic improvement in clinical symptoms, sedimentation rate, and radiographic scans. No consistent improvement in bactericidal function or neutrophil oxidative capacity could be demonstrated. However, serum neopterin levels, a measure of macrophage activation, increased in a dose-dependent manner with rHuIFN-γ therapy; increased levels mirrored the improved clinical parameters. This patient's treatment illustrates the usefulness of the single-photon emission computed tomography (SPECT) gallium scan for following pulmonary inflammatory lesions in the presence of fibrosis and indicates that rHuIFN-γ may be of benefit toCaDpatients with serious infectionsunresponsive to conventional therapy.

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