ABSTRACT: Background: Guillain Barré Syndrome (GBS) is an autoimmune disorder caused by the immune-mediated damage of the peripheral nervous system. Increasing evidence suggests that inflammatory cytokines are important mediators for the onset and progression of GBS. A number of clinical studies have demonstrated elevated levels of T helper-1 (Th1-), Th2-, and Th17-related cytokines in patients with GBS; however, the results were inconsistent across studies. Methods: We performed a systematic review and a meta-analysis of studies comparing the levels of inflammatory cytokines in the cerebrospinal fluid and peripheral blood between patients with GBS and healthy individuals, using Comprehensive Meta-Analysis Version 2 software. A database search identified 30 studies comprising 1,302 patients with GBS and 1,073 healthy controls. Results: The random-effects meta-analysis demonstrated that peripheral blood tumor necrosis factor-? (Hedges g, 1.544; 95% confidence interval (CI), 0.923-2.165; p < 0.001), interleukin-1? (IL-1?; Hedges g, 0.678; 95% CI, 0.183-1.172; p = 0.007), IL-6 (Hedges g, 0.630; 95% CI, 0.100-1.160; p = 0.02), IL-4 (Hedges g, 0.822; 95% CI, 0.220-1.423; p = 0.007), IL-17 (Hedges g, 1.452; 95% CI, 0.331-2.573; p = 0.011), interferon-? (Hedges g, 1.104; 95% CI, 0.490-1.719; p < 0.001), and C-reactive protein (Hedges g, 0.909; 95% CI, 0.453-1.365; p < 0.001) levels were significantly increased in patients with GBS when compared with healthy controls. Contrastingly, the blood IL-10 and transforming growth factor-? levels were not significantly associated with GBS. Furthermore, the meta-analysis found that cerebrospinal fluid IL-17 levels were significantly associated with GBS (Hedges g, 1.882; 95% CI, 0.104-3.661; p = 0.038). Conclusion: Altogether, our results clarified the circulating inflammatory cytokine profile in patients with GBS, and revealed that Th1-, Th2-, and Th17-related cytokines were highly elevated in the GBS patients, suggesting the potential use of these cytokines as biomarkers for GBS.