Elevated IL-1 secretion from CGD monocytes can be counteracted with IL-1Ra. (A-B) Caspase-1 activation in monocytes determined by caspase-1 FLICA. (A) Unstimulated (−) and LPS-treated (6 hours) monocytes from an asymptomatic CGD patient (p47) and a healthy control (C1). (B) Active caspase-1 in monocytes from 2 asmyptomatic CGD patients with the indicated mutation and 2 healthy controls (C1, C2) quantified by caspase-1 FLICA. (C-D) IL-1β (C) and IL-1α (D) release from monocytes of 2 asymptomatic CGD patients and a healthy control (C1) treated with LPS for the indicated time points determined by enzyme-linked immunosorbent assay. (E) Caspase-1 activation in unstimulated and LPS-treated (6 hours) monocytes from a symptomatic CGD patient with colitis determined by caspase-1 FLICA. (F-G) IL-1β (F) and IL-1α (G) release from monocytes of the indicated symptomatic CGD patient before (pre-anakinra) and after (post-anakinra) treatment with anakinra compared with a healthy control (C1). Data are representative of 4 (A-D) or 3 (E) experiments with cells from at least 2 different CGD patients (error bars indicate SEM of triplicate wells).