Association of CXCL10 and CXCL13 Levels with Disease Activity and Cutaneous Manifestation in Active Adult-Onset Still's Disease

Hyoun-Ah Kim¹, In Je Kim², Jae Ho Han³, Chang-Hee Suh¹ and Juyang Jung¹, ¹Department of Rheumatology, Ajou University School of Medicine, Suwon, South Korea, ²Division of Rheumatology, Department of Internal Medicine, Ewha Womans University School of Medicine, Seoul, South Korea, ³Department of Pathology, Ajou University School of Medicine, Suwon, South Korea

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: adult-onset Still's disease and chemokines, Disease Activity

Session Information

Date: Sunday, November 8, 2015

Title: Miscellaneous Rheumatic and Inflammatory Diseases Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: CXCL10 is produced in response to interferon-γ, and tumor necrosis factor-α (TNF-α) triggers the accumulation of activated lymphocytes. CXCL13 is constitutively expressed in secondary lymphoid tissues, and the expression is upregulated by TNF-α, via T cell stimulation. It appears that CXCL10 and CXCL13 could play a potential role in the pathogenesis of adult-onset Still’s disease (AOSD), therefore, we investigated the associations between CXCL10 and CXCL13 levels and clinical manifestations in patients with active AOSD.

Methods: Blood samples were collected from 39 active AOSD patients and 40 healthy controls (HC). Of the AOSD patients, follow-up samples were collected from 15 9.6 ± 9.2 months later. CXCL10, CXCL13, and CXCR3 expression levels in biopsy specimens obtained from 26 AOSD patients with skin rashes were investigated via immunohistochemistry.

Results: The CXCL10 level of AOSD patients (1,031.3 ± 2,019.6 pg/mL) was higher than that of HC (104.4 ± 47.9 pg/mL, p=0.006). Also, the CXCL13 level of AOSD patients (158.8 ± 151.2 pg/mL) was higher than that of HC (23.5 ± 18.1 pg/mL, p<0.001). Serum CXCL10 levels correlated with ferritin and systemic scores. Serum CXCL13 levels correlated with those of hemoglobin, C-reactive protein, ferritin, and albumin, and systemic scores. In follow-up AOSD patients, the levels of CXCL10 and CXCL13 fell significantly (153.7 ± 130.1 pg/mL, p=0.002, and 89.1 ± 117.4 pg/mL, p=0.001, respectively). On immunohistochemistry, the percentages of inflammatory cells expressing CXCL10 ranged from 1 to 85%, CXCL13 from 1 to 72%, and CXCR3 from 2 to 65%. The percentage of CXCL10-positive inflammatory cells was higher in skin biopsy samples exhibiting mucin deposition than in those that did not (p=0.01). CXCL13 levels were correlated with those of CD4 and CD68.

Conclusion: Serum CXCL10 and CXCL13 levels may serve as clinical markers for assessment of disease activity in AOSD. CXCL10/CXCR3 and CXCL13 may contribute to the inflammatory response, especially skin manifestations thereof, in AOSD.

Disclosure: H. A. Kim, None; I. J. Kim, None; J. H. Han, None; C. H. Suh, None; J. Jung, None.

To cite this abstract in AMA style:

Kim HA, Kim IJ, Han JH, Suh CH, Jung J. Association of CXCL10 and CXCL13 Levels with Disease Activity and Cutaneous Manifestation in Active Adult-Onset Still’s Disease [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/association-of-cxcl10-and-cxcl13-levels-with-disease-activity-and-cutaneous-manifestation-in-active-adult-onset-stills-disease/. Accessed .

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