PBMC from TRAPS patients are hyperresponsive to low-dose LPS. (A) PBMC from patients with TRAPS heterozygous for structural TNFR1 mutations (n = 13, black bars), R92Q or P46L functional polymorphisms (n = 8, gray bars), or healthy controls (n = 13, white bars) were stimulated with the indicated dose of LPS for 24 h, and cytokine concentrations in supernatants were measured by cytometric bead array. P values obtained for differences vs. controls for samples from patients with structural TNFR1 mutations at 0.01 ng/mL LPS were 0.038 for TNF; 0.034 for IL-1β; and 0.011 for IL-6 (Student's unpaired t test). (B) Data from A were reanalyzed in subsets of patients treated with the indicated biologic agents. (C) Data from A were reanalyzed for patients having low (<2 mg/dL) or high (>2 mg/dL) CRP at the time of sample collection. *, P < 0.05 for the indicated comparisons.