The Changing Paradigm of Management of Liver Abscesses in Chronic Granulomatous Disease

Abstract

Background

Chronic granulomatous disease (CGD) is a rare genetic disorder causing recurrent infections. Over one quarter of patients develop hepatic abscesses and liver dysfunction. Recent reports suggest disease-modifying treatment with corticosteroids is effective for these abscesses. Comparison of corticosteroid therapy to traditional invasive treatments has not been performed.

Methods

Records of 268 patients with CGD treated at the National Institutes of Health (NIH) from 1980 to 2014 were reviewed. Patients with liver involvement and complete records were included. We recorded residual reactive oxygen intermediate (ROI) production by neutrophils, NADPH oxidase germline mutation status, laboratory values, imaging characteristics, time to repeat hepatic interventions, and overall survival among three treatment cohorts: open liver surgery (OS), percutaneous liver-directed interventional radiology therapy (IR), and high-dose corticosteroid management (CM).

Results

88 of 268 patients with CGD suffered liver involvement. 26 patients with median follow-up of 15.5 years (8.5-32.9) had complete records and underwent 100 standard interventions, 42 IR and 58 OS. Eight patients received high-dose corticosteroids only. There were no differences in NADPH genotype, size, or number of abscesses between patients treated with OR, IR, or CM. Time to repeat intervention was extended in OS compared to IR (18.8 vs. 9.5 months; p=0.04) and further increased in CM alone (median time to recurrence not met). Impaired macrophage and neutrophil function measured by ROI production correlated with shorter time to repeat intervention (r=0.6; p=0.0019).

Conclusions

Treatment of CGD-associated liver abscesses with corticosteroids was associated with fewer subsequent hepatic interventions and improved outcome compared to invasive treatments.