Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis

被引：180

作者：

Shimizu, Masaki ^{[1
]}

Yokoyama, Tadafumi ^{[1
]}

Yamada, Keiko ^{[2
]}

Kaneda, Hisashi ^{[3
]}

Wada, Hideo ^{[4
]}

Wada, Taizo ^{[1
]}

Toma, Tomoko ^{[1
]}

Ohta, Kazuhide ^{[5
]}

Kasahara, Yoshihito ^{[1
]}

Yachie, Akihiro ^{[1
]}

机构：

[1] Kanazawa Univ, Dept Pediat, Sch Med, Inst Med Pharmaceut & Hlth Sci, Kanazawa, Ishikawa 9208641, Japan

[2] Tonami Gen Hosp, Dept Pediat, Tonami, Toyama, Japan

[3] Toyama City Hosp, Dept Pediat, Yoyama, Japan

[4] Noto Gen Hosp, Dept Pediat, Nanao, Japan

[5] Kanazawa Med Ctr, Dept Pediat, Kanazawa, Ishikawa, Japan

来源：

RHEUMATOLOGY | 2010年 / 49卷 / 09期

关键词：

Macrophage activation syndrome; Systemic juvenile idiopathic arthritis; Interleukin-18; KILLER-CELL DYSFUNCTION; RHEUMATOID-ARTHRITIS; KAWASAKI-DISEASE; ADULT-ONSET; HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS; GAMMA PRODUCTION; STILLS-DISEASE; MARKER; DISORDERS; SEVERITY;

D O I：

10.1093/rheumatology/keq133

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Methods. Five patients with MAS complicating s-JIA (MAS/s-JIA), 10 with HLH due to EBV infection (EBV-HLH), 22 with KD and 28 healthy controls were analysed. Cytokine concentrations (IL-18, IL-6, neopterin and TNF-alpha receptor Types I and II) were quantified in serum by ELISA. Results were compared with clinical features of MAS/s-JIA, including ferritin concentrations. Results. Serum IL-18 concentrations in MAS/s-JIA patients were significantly higher than those in EBV-HLH or KD patients (P < 0.05). Serum IL-6 concentrations in KD patients were significantly higher than those in EBV-HLH or MAS/s-JIA patients. Serum neopterin concentrations in EBV-HLH patients were significantly higher than those in MAS/s-JIA or KD patients. Serum IL-18 correlated positively with the following measurements of disease activity: CRP, ferritin, lactate dehydrogenase and other cytokines (P < 0.05). Serum concentrations of IL-18 in s-JIA patients remained elevated in the inactive phase of disease, whereas clinical parameters and other cytokines normalized. Conclusions. IL-18 may be an important mediator in s-JIA. Although serum Il-18 concentrations correlated with markers of the disease activity, IL-18 concentrations remained elevated even when other markers of disease activity normalized. Serum IL-18 concentration may be a promising indicator of the disease activity. The cytokine release pattern in MAS/HLH is different among patients with different aetiologies. Monitoring the cytokine profile, including IL-18, may be useful for differentiation of MAS/HLH and evaluation of disease activity in s-JIA.

引用

页码：1645 / 1653

页数：9

共 33 条

[11] Natural killer cell dysfunction - A common pathway in systemic-onset juvenile rheumatoid arthritis, macrophage activation syndrome, and hemophagocytic lymphohistiocytosis? [J].