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Effectiveness of IFN-gamma for liver abscesses in chronic granulomatous disease
D Conte1, M Fraquelli, F Capsoni
1Cattedra di Gastroenterologia, IRCCS Ospedale Maggiore, Milano, Italy. dconte@imiucca.csi.unimi.it
Abstract:
In chronic granulomatous disease, interferon-gamma (IFN-gamma) significantly reduces the incidence and severity of recurrent infections, but its effectiveness administered ex novo during acute infection has been reported in only one case. In this report, we describe two adult brothers with chronic granulomatous disease treated successfully with IFN-gamma for acute liver abscesses. Two brothers with severe recurrent infections of unknown origin were hospitalized for septic fever, malnutrition, and ultrasonographic evidence of liver abscess. Autosomal recessive chronic granulomatous disease was diagnosed based on lack of superoxide anion production by phagocytes and absence of p47-phox protein. An antibiotic regimen specifically directed against Staphylococcus aureus was ineffective, whereas treatment with 50 microg/m2 IFN-gamma s.c. thrice weekly induced complete healing with scarring within 3 months. No septic recurrence was observed during a 4-year follow-up period. In chronic granulomatous disease, IFN-gamma is effective not only in preventing but also in healing life-threatening acute infections.
Insights
Interferon-gamma (IFN-gamma) successfully treated severe liver abscesses in two brothers with chronic granulomatous disease. This demonstrates IFN-gamma
Area of Science:
- Immunology
- Infectious Diseases
- Genetics
Background:
- Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by recurrent, severe infections.
- Interferon-gamma (IFN-gamma) is a standard therapy for CGD to reduce infection incidence and severity.
- Limited data exist on IFN-gamma's efficacy when initiated during acute infections.
Observation:
- Two adult brothers presented with severe recurrent infections, including septic fever, malnutrition, and liver abscesses.
- Diagnosis of autosomal recessive CGD was confirmed by phagocyte superoxide anion production and p47-phox protein absence.
- Standard antibiotic therapy targeting Staphylococcus aureus was ineffective.
Findings:
- Treatment with subcutaneous IFN-gamma (50 microg/m2 thrice weekly) led to complete healing of liver abscesses within 3 months.
- Complete resolution of abscesses with scarring was observed.
- No septic recurrences occurred during a 4-year follow-up period.
Implications:
- IFN-gamma is effective in treating life-threatening acute infections in patients with CGD, not just for prevention.
- This case series expands the therapeutic role of IFN-gamma in managing severe infections in CGD.
- Early and effective intervention with IFN-gamma can lead to favorable long-term outcomes in CGD patients with severe infections.