Infectious Disease Compendium

Chronic Granulomatous Disease

Diagnosis

X-Linked defect in nicotinamide adenine dinucleotide phosphate oxidase activity.  Diagnose with the nitroblue-tetrazolium (NBT) test. There is also genetic testing.

Five types

X-linked chronic granulomatous disease (CGD), autosomal recessive cytochrome b-negative CGD, autosomal recessive cytochrome b-positive CGD, type I autosomal recessive cytochrome b-positive CGD, type II atypical granulomatous disease.

Syndromes

Lung infection occurs in 87% of patients and liver abscess in 32% (PubMed). They can get a variety of other, often deep, infections

Microbiology

Aspergillus incidence is 2.6 cases per 100 patient-years; A. nidulans is uniquely a pathogen in Chronic Granulomatous Disease (PubMed).

Listeria.

E. coli. Klebsiella.

Burkholderia, 1.06 per 100 patient-years; Nocardia, 0.81 per 100 patient-years; Serratia, 0.98 per 100 patient-years, and severe Staphylococcus infection, 1.44 per 100 patient-years.

They also get infections with Methylotrophs:  Granulibacter bethesdensis (9 cases), Acidomonas methanolica (2 cases), and Methylobacterium lusitanum (1 case) (PubMed).  To quote the EID article "Methylotroph infections in CGD patients typically result in elevated inflammatory markers and lymphadenopathy, which may progress to necrotizing lymphadenitis with or without abscess formation. The clinical course may be protracted because of infection persistence, antimicrobial drug resistance, and relapse. Culturing these bacteria is difficult, requiring atypical media and prolonged incubation."

Treatment

Interferon gamma 1b decreases infections.

Liver abscesses do better with steroids (PubMed).

Last Update: 06/08/18.