Different Features of Interleukin-37 and Interleukin-18 as Disase Activity Markers of Adult-Onset Still's Disease.

Seoung Wan Nam,Suman Kang,Dae Hyun Yoo,Jun Hyeok Lee

doi:10.3390/jcm10050910

Abstract

The aim of this study was to evaluate the usefulness of serum interleukin (IL)-37 and IL-18 as disease activity markers of adult-onset Still’s disease (AOSD) and to compare their related clinical features. Forty-five patients with a set of high and subsequent low disease activity status of AOSD were enrolled. Modified Pouchot (mPouchot) score and serologic disease activity markers including levels of IL-37 and IL-18 were compared between high and low disease activity status. The relationships between disease activity parameters and differences in levels of cytokines according to each disease manifestation were evaluated in high disease activity status. mPouchot score and all disease activity markers including IL-37 and IL-18 significantly declined after treatment. Though both cytokines positively correlated with mPouchot score, the two did not correlate with each other in high disease activity status. IL-18 positively correlated with ferritin, AST, and LDH while IL-37 correlated better with CRP. The expression level of IL-37 was related to leukocytosis while IL-18 was related to pleuritis, pneumonitis, abnormal LFT, and hyperferritinemia. In addition, patients in the IL-18 dominant group presented with higher LDH levels and required a higher mean corticosteroid dose. In conclusion, IL-37 and IL-18 are disease activity markers reflecting different aspects of AOSD that can complement each other.

Highlights

Adult-onset Still’s disease (AOSD) is a rare systemic auto-inflammatory disorder characterized by four cardinal symptoms of spiking fever, arthralgia or arthritis, evanescent salmon-colored rash, and leukocytosis with neutrophil predominance [1]
We evaluated how serum levels of IL-37 and IL-18 reflect the activity of AOSD using paired serum samples of high and low disease activity status in a cohort of patients with AOSD
Sixty patients with AOSD were enrolled in this study, all of whom met the criteria of Yamaguchi et al Four major criteria proposed by Yamaguchi include fever, arthralgia or arthritis, non-pruritic salmon-colored rash, and leukocytosis with granulocyte predominance

Summary

Introduction

Adult-onset Still’s disease (AOSD) is a rare systemic auto-inflammatory disorder characterized by four cardinal symptoms of spiking fever, arthralgia or arthritis, evanescent salmon-colored rash, and leukocytosis with neutrophil predominance [1]. It is difficult to assess disease activity of AOSD. Pouchot et al described a systemic disease activity score comprised of 12 main signs and symptoms of AOSD; this scoring system was later modified by Rau et al [3,4]. Several biomarkers were proposed as potential disease activity parameters of AOSD including. No reliable disease activity parameter exists yet [6]. Given the potential polycyclic evolution of AOSD, it is necessary to identify biomarkers for accurate assessment of disease activity to improve

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