title: Recombinant human interferon-gamma as adjunct therapy for Aspergillus infection in a patient with chronic granulomatous disease.
abstract: The hallmark of chronic granulomatous disease (CGD) is defective killing of ingested microorganisms by phagocytic cells. Invasive aspergillosis in CGD patients is particularly virulent and has a mortality rate of approximately 50%. A patient with autosomal recessive CGD was identified who had progressive pulmonary aspergillosis that was unresponsive to conventional antifungal therapy. She was treated with recombinant human interferon-gamma (rHuIFN-gamma) 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-gamma 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-gamma may be of benefit to CGD patients with serious infections unresponsive to conventional therapy.
authors.affiliation: Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: J
authors.initials: J
authors.lastname: Bernhisel-Broadbent
authors.forename: E E
authors.initials: EE
authors.lastname: Camargo
authors.forename: H S
authors.initials: HS
authors.lastname: Jaffe
authors.forename: H M
authors.initials: HM
authors.lastname: Lederman
mesh_terms: D000328:Adult; D001228:Aspergillosis / Q000209:etiology / Q000628:therapy*; D001799:Blood Sedimentation; D004306:Dose-Response Relationship, Immunologic; D005260:Female; D006105:Granulomatous Disease, Chronic / Q000150:complications* / Q000276:immunology; D006801:Humans; D007371:Interferon-gamma / Q000627:therapeutic use*; D008172:Lung Diseases, Fungal / Q000209:etiology / Q000628:therapy*; D010586:Phagocytes / Q000276:immunology; D011994:Recombinant Proteins
chemical_list: D011994:Recombinant Proteins; D007371:Interferon-gamma