Ministry of Education Key Laboratory of Child Development and Disorders, Key Laboratory of Pediatrics in Chongqing, Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, Children's Hospital of Chongqing Medical University, Chongqing, China.
Pediatr Allergy Immunol. 2013 Nov;24(7):691-6. doi: 10.1111/pai.12130.
Juvenile idiopathic arthritis (JIA) has three major onset types with widely varying clinical features: systemic, polyarticular and pauciarticular. We assessed natural killer (NK) cell function and killer cell immunoglobulin-like receptor (KIR) genotypes in patients with different JIA subtypes.
Peripheral blood samples from 72 children with active JIA (systemic, 25; polyarticular, 24; pauciarticular, 23) and 25 controls were used for flow cytometric assessments of NK cell count, cytotoxicity, perforin, granzyme B, interferon (IFN)-γ and tumour necrosis factor (TNF)-α. Samples from 220 children with JIA (systemic, 84; polyarticular, 72; pauciarticular, 64) and 150 controls were used for KIR2DS2, KIR2DS4, KIR3DS1, KIR2DL1, KIR2DL2, KIR2DL3 and KIR3DL1 typing by polymerase chain reaction with sequence-specific oligonucleotide probes.
Compared with the controls, the patients with systemic JIA showed lower NK cell counts, cytotoxicity and perforin and granzyme B expression (p < 0.05), while the patients with pauci- and polyarticular JIA showed higher perforin and granzyme B expression (p < 0.05). NK cells produced higher level of TNF-α while lower level of IFN-γ in the pauci- and polyarticular JIA groups than in the systemic JIA group (p < 0.05). No significant differences in KIR gene frequencies were found between the JIA subgroups and healthy controls, except for the positive frequency and locus frequency of KIR2DS4, which were lower in the systemic JIA group.
Compared with poly- and pauciarticular JIA, systemic JIA is associated with decreased NK cell function, more IFN-γ and less TNF-α secretion of NK cell and lower KIR2DS4 frequency.
幼年特发性关节炎（JIA）有三种主要的发病类型，临床表现差异很大：全身型、多关节型和少关节型。我们评估了不同 JIA 亚型患者的自然杀伤（NK）细胞功能和杀伤细胞免疫球蛋白样受体（KIR）基因型。
采集 72 例活动期 JIA 患儿（全身型 25 例，多关节型 24 例，少关节型 23 例）和 25 例对照者外周血样本，采用流式细胞术检测 NK 细胞计数、细胞毒性、穿孔素、颗粒酶 B、干扰素（IFN）-γ和肿瘤坏死因子（TNF）-α。采集 220 例 JIA 患儿（全身型 84 例，多关节型 72 例，少关节型 64 例）和 150 例对照者样本，采用聚合酶链反应-序列特异性寡核苷酸探针法检测 KIR2DS2、KIR2DS4、KIR3DS1、KIR2DL1、KIR2DL2、KIR2DL3 和 KIR3DL1 基因型。
与对照组相比，全身型 JIA 患儿 NK 细胞计数、细胞毒性、穿孔素和颗粒酶 B 表达降低（p<0.05），而少关节型和多关节型 JIA 患儿穿孔素和颗粒酶 B 表达升高（p<0.05）。少关节型和多关节型 JIA 患儿 NK 细胞 TNF-α分泌水平升高，IFN-γ 分泌水平降低，与全身型 JIA 患儿比较差异均有统计学意义（p<0.05）。除 KIR2DS4 的阳性频率和基因座频率在全身型 JIA 患儿中较低外，JIA 各亚组与健康对照者间 KIR 基因频率无显著差异。
与多关节型和少关节型 JIA 相比，全身型 JIA 与 NK 细胞功能下降、IFN-γ 分泌增加、TNF-α 分泌减少以及 KIR2DS4 频率降低有关。
