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Abstract：
UNASSIGNED: Increased serum IgG and autoantibodies suggest involvement of B cells in autoimmune hepatitis (AIH). The aim of this study was to assess levels of B cell activating factor of the tumour necrosis family (BAFF), IL-21, and circulating B cell populations in AIH and correlate these to treatment response.
UNASSIGNED: BAFF and IL-21 levels were determined in 66 patients with AIH before treatment and 10 healthy controls. Flow cytometry was performed on circulating B cells of 10 patients with AIH and 12 healthy controls.
UNASSIGNED: Based on BAFF and IL-21 levels, untreated patients with AIH were divided into 3 groups: 27 (41%) patients with normal BAFF and IL-21 (normal BAFF), 27 (41%) patients with elevated BAFF but normal IL-21 (high BAFF), and 12 (18%) patients with elevated IL-21 (high IL-21). The high BAFF group presented with higher bilirubin compared with the normal BAFF and high IL-21 groups (159 vs. 26 vs. 89 μmol/L; p = 0.001; Mann-Whitney U test). After 12 months of treatment, 54% of the high BAFF group reached remission compared with 34% of the normal BAFF group and 0% of the high IL-21 group (p = 0.006, Chi-square test). During follow-up, 3 patients (25%) with high IL-21 developed primary sclerosing cholangitis (PSC) variant syndrome. Autoimmune-associated B cells were increased in patients with AIH compared with healthy controls (4.4 vs. 1.4%; p = 0.003, Mann-Whitney U test). BAFF levels were correlated positively with naïve B cells (p = 0.01) and negatively with class-switched B cells (p = 0.003) and nonclass-switched B cells (p = 0.005, Spearman correlation).
UNASSIGNED: Using BAFF and IL-21, we identified different immunological phenotypes of AIH with a different presentation, treatment response, and outcome. Patients with high IL-21 had the poorest treatment response and a risk of developing PSC variant syndrome. BAFF level was related to shifts in circulating B-cell populations.
UNASSIGNED: In patients with untreated autoimmune hepatitis (AIH), circulating B-cell activating factor of the tumour necrosis family (BAFF), IL-21, and B-cell populations were determined. Three subgroups were identified: with (1) normal BAFF and IL-21, (2) elevated BAFF and normal IL-21, and (3) elevated IL-21. Remission after 1-year treatment occurred in 54, 34, and 0% in Groups 1, 2, and 3, respectively. Group 2 had higher bilirubin, indicating more liver dysfunction. In 25% of patients with high IL-21, AIH-PSC variant syndrome developed, but none in the other groups. Autoimmune-associated B cells were elevated and BAFF levels correlated with certain B cells.
UNASSIGNED: BAFF and IL-21 levels were determined in 66 patients with AIH before treatment and 10 healthy controls. Flow cytometry was performed on circulating B cells of 10 patients with AIH and 12 healthy controls.
UNASSIGNED: Based on BAFF and IL-21 levels, untreated patients with AIH were divided into 3 groups: 27 (41%) patients with normal BAFF and IL-21 (normal BAFF), 27 (41%) patients with elevated BAFF but normal IL-21 (high BAFF), and 12 (18%) patients with elevated IL-21 (high IL-21). The high BAFF group presented with higher bilirubin compared with the normal BAFF and high IL-21 groups (159 vs. 26 vs. 89 μmol/L; p = 0.001; Mann-Whitney U test). After 12 months of treatment, 54% of the high BAFF group reached remission compared with 34% of the normal BAFF group and 0% of the high IL-21 group (p = 0.006, Chi-square test). During follow-up, 3 patients (25%) with high IL-21 developed primary sclerosing cholangitis (PSC) variant syndrome. Autoimmune-associated B cells were increased in patients with AIH compared with healthy controls (4.4 vs. 1.4%; p = 0.003, Mann-Whitney U test). BAFF levels were correlated positively with naïve B cells (p = 0.01) and negatively with class-switched B cells (p = 0.003) and nonclass-switched B cells (p = 0.005, Spearman correlation).
UNASSIGNED: Using BAFF and IL-21, we identified different immunological phenotypes of AIH with a different presentation, treatment response, and outcome. Patients with high IL-21 had the poorest treatment response and a risk of developing PSC variant syndrome. BAFF level was related to shifts in circulating B-cell populations.
UNASSIGNED: In patients with untreated autoimmune hepatitis (AIH), circulating B-cell activating factor of the tumour necrosis family (BAFF), IL-21, and B-cell populations were determined. Three subgroups were identified: with (1) normal BAFF and IL-21, (2) elevated BAFF and normal IL-21, and (3) elevated IL-21. Remission after 1-year treatment occurred in 54, 34, and 0% in Groups 1, 2, and 3, respectively. Group 2 had higher bilirubin, indicating more liver dysfunction. In 25% of patients with high IL-21, AIH-PSC variant syndrome developed, but none in the other groups. Autoimmune-associated B cells were elevated and BAFF levels correlated with certain B cells.
摘要：
未经证实：血清IgG和自身抗体升高提示B细胞参与自身免疫性肝炎（AIH）。这项研究的目的是评估肿瘤坏死家族（BAFF）的B细胞活化因子的水平，IL-21和AIH中的循环B细胞群体并将这些与治疗反应相关联。
UNASSIGNED：在治疗前66例AIH患者和10例健康对照中测定了BAFF和IL-21水平。对10例AIH患者和12例健康对照者的循环B细胞进行流式细胞术。
未经评估：基于BAFF和IL-21水平，将未经治疗的AIH患者分为3组:BAFF正常和IL-21正常的患者27例(41%),27例（41%）BAFF升高但IL-21正常（高BAFF）的患者，和12名（18％）IL-21升高（高IL-21）的患者。与正常BAFF和高IL-21组相比，高BAFF组呈现较高的胆红素（159vs.26vs.89μmol/L；p=0.001；Mann-WhitneyU检验)。经过12个月的治疗，高BAFF组54％达到缓解，而正常BAFF组为34％，高IL-21组为0％（p=0.006，卡方检验）。随访期间,3例（25％）高IL-21患者发生原发性硬化性胆管炎（PSC）变异综合征。与健康对照组相比，AIH患者的自身免疫相关B细胞增加（4.4vs.1.4%；p=0.003，曼-惠特尼U检验)。BAFF水平与初始B细胞呈正相关（p=0.01），与类别转换B细胞（p=0.003）和非类别转换B细胞呈负相关（p=0.005，Spearman相关性）。
UNASSIGNED：使用BAFF和IL-21，我们鉴定了具有不同表现的AIH的不同免疫表型，治疗反应,和结果。高IL-21患者的治疗反应最差，并且有发生PSC变异综合征的风险。BAFF水平与循环B细胞群的变化有关。
未经证实：在未经治疗的自身免疫性肝炎（AIH）患者中，肿瘤坏死家族的循环B细胞活化因子（BAFF），测定IL-21和B细胞群。确定了三个亚组:(1)正常的BAFF和IL-21,(2)升高的BAFF和正常的IL-21,以及(3)升高的IL-21。治疗1年后，第1、2和3组的缓解率分别为54％、34％和0％。第2组胆红素较高，表明更多的肝功能障碍。在25％的高IL-21患者中，AIH-PSC变异综合征发展，但在其他组中没有。自身免疫相关的B细胞升高，BAFF水平与某些B细胞相关。
UNASSIGNED：在治疗前66例AIH患者和10例健康对照中测定了BAFF和IL-21水平。对10例AIH患者和12例健康对照者的循环B细胞进行流式细胞术。
未经评估：基于BAFF和IL-21水平，将未经治疗的AIH患者分为3组:BAFF正常和IL-21正常的患者27例(41%),27例（41%）BAFF升高但IL-21正常（高BAFF）的患者，和12名（18％）IL-21升高（高IL-21）的患者。与正常BAFF和高IL-21组相比，高BAFF组呈现较高的胆红素（159vs.26vs.89μmol/L；p=0.001；Mann-WhitneyU检验)。经过12个月的治疗，高BAFF组54％达到缓解，而正常BAFF组为34％，高IL-21组为0％（p=0.006，卡方检验）。随访期间,3例（25％）高IL-21患者发生原发性硬化性胆管炎（PSC）变异综合征。与健康对照组相比，AIH患者的自身免疫相关B细胞增加（4.4vs.1.4%；p=0.003，曼-惠特尼U检验)。BAFF水平与初始B细胞呈正相关（p=0.01），与类别转换B细胞（p=0.003）和非类别转换B细胞呈负相关（p=0.005，Spearman相关性）。
UNASSIGNED：使用BAFF和IL-21，我们鉴定了具有不同表现的AIH的不同免疫表型，治疗反应,和结果。高IL-21患者的治疗反应最差，并且有发生PSC变异综合征的风险。BAFF水平与循环B细胞群的变化有关。
未经证实：在未经治疗的自身免疫性肝炎（AIH）患者中，肿瘤坏死家族的循环B细胞活化因子（BAFF），测定IL-21和B细胞群。确定了三个亚组:(1)正常的BAFF和IL-21,(2)升高的BAFF和正常的IL-21,以及(3)升高的IL-21。治疗1年后，第1、2和3组的缓解率分别为54％、34％和0％。第2组胆红素较高，表明更多的肝功能障碍。在25％的高IL-21患者中，AIH-PSC变异综合征发展，但在其他组中没有。自身免疫相关的B细胞升高，BAFF水平与某些B细胞相关。
