Background/Purpose: Behcet’s disease (BD) is an inflammatory disease characterized by ocular, mucosal and skin lesions. However, the pathogenesis of BD is still unknown. Characteristics of histologic findings show granulocyte-dominant immune cell infiltration, suggesting dominancy of innate immune response or “inflammation-like immune response” rather than acquired immune response. Thus, BD is recently proposed to categorize as an autoinflammatory disease. To clarify the pathogenesis of BD and have an answer for activation status of immune system, levels of multiple chemokines in the circulating milieu were assessed as a reflection of the local production of chemokines for the recruitment of immune cells.

Methods: Twenty-seven patients with BD (12 active and 15 inactive patients) and 20 healthy controls were involved. Levels of chemokine in plasma were examined by enzyme-linked immunosorbent assay. Various chemokines were assessed, including RANTES (recruitment of general T cells), CX3CL1 (cytotoxic T cells, NK cells, gamma-delta T cells), MCP-1 (monocytes), CXCL9 (Th1 cells including Th1/Th17 cells, effector CD8+ cells), MIP3a (Th17)、CCL27 (Th22) to cover recruitment of various lymphocytes. Comparison between 2 groups was assessed by non-parametric Mann-Whitney U-test.

Results: Levels of circulating RANTES, CX3CL1, MCP-1, and CXCL9 were comparable in both groups, suggesting that recruitment of general T cells, monocytes, cytotoxic lymphocytes were not expected based on profiles of these chemokines. On the other hand, plasma concentration of MIP3a and CCL27 were higher in BD compared to healthy controls (MIP3a: 39.6 ± 54.3 versus 14.5 ± 8.9, P < 0.05, CCL27: 354.2 ± 150.6 versus 238.3 ± 155.5, P < 0.05, respectively), suggesting the association with recruitment of Th17 and Th22. Interestingly, levels of CCL20 was comparable between patients with active disease and those without, whereas CCL27 was higher in patients with inactive disease compared to those with active disease.

Conclusion: In patients with BD, expression profile of the chemokine in circulation is skewed rather than global upregulation, suggesting that pathophysiological process in BD is not a non-specific inflammation from the point of view of chemokines. Upregulation of CCL20 in BD patients and downregulation of CCL27 in active patients might associate with regulation of infiltration of Th17 or Th22 to the lesions, and with pathogenesis of BD.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/expression-profile-of-chemokines-is-skewed-to-th17th22-recruitment-in-circulation-of-patients-with-behcets-disease/