B 细胞耗竭治疗自身免疫性肝病:适应症和结果的回顾性分析

JPGN Reports Pub Date : 2024-06-14 DOI:10.1002/jpr3.12098
Guillermo A. Costaguta, Fernando Álvarez
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引用次数: 0

摘要

在北美,小儿自身免疫性肝炎的发病率为 0.23/100.000,如果不及时治疗,预后很差。类固醇是首选疗法,但并非总是有效。B细胞耗竭是一种安全有效的疗法,可以采用节省类固醇的方案,尤其是对于不能耐受副作用的患者。我们回顾性地回顾了2017年至2022年间接受利妥昔单抗治疗的患者。我们对2017年至2022年期间接受利妥昔单抗治疗的患者进行了回顾性研究,记录了患者的人口统计学特征、既往治疗情况、B细胞耗竭的原因、反应和不良反应。所有患者都有合并症,导致类固醇治疗不成功或不理想。利妥昔单抗在平均随访 8 个月时开始使用。6 个月后,丙氨酸转氨酶和天冬氨酸转氨酶的平均水平分别从 575 IU/L 和 342 IU/L 降至 28 IU/L (p = 0.02) 和 36 IU/L (p = 0.008)。平均γGT从105 IU/L降至25 IU/L(p = 0.01)。所有患者的免疫球蛋白 G 水平均恢复正常(p = 0.01)。未发现严重不良反应。B细胞耗竭是治疗自身免疫性肝病的一种有效而安全的方法,应作为一种选择,尤其是对于那些不希望使用类固醇或表现出不依从性的复发患者。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
B cell depletion for autoimmune liver diseases: A retrospective review of indications and outcomes
Pediatric autoimmune hepatitis has an incidence of 0.23/100.000 children in North America, with a bleak prognosis if left untreated. Steroids are the therapy of choice but are not always effective. B cell depletion is a safe and effective therapy that allows for a steroid‐sparing protocol, especially in patients who do not tolerate side effects.We retrospectively reviewed rituximab‐treated patients between 2017 and 2022. Demographics, previous treatments, reasons for B cell depletion, response, and adverse effects were noted.Six patients with a mean age of 10.2 years were included. All patients had comorbidities that rendered treatment with steroids unsuccessful or undesirable. Rituximab was started at a mean follow‐up of 8 months. After 6 months, the mean alanine transaminase and aspartate transaminase levels decreased from 575 IU/L and 342 IU/L, respectively, to 28 IU/L (p = 0.02) and 36 IU/L (p = 0.008), respectively. Mean γGT decreased from 105 to 25 IU/L (p = 0.01). Immunoglobulin G levels were normalized in all patients (p = 0.01). No severe adverse events were observed. One patient had persistent hypogammaglobulinemia, and another had lymphopenia.B‐cell depletion is an effective and safe treatment for autoimmune liver diseases and should be included as an option, particularly for relapsing patients in whom steroids are undesirable or have shown nonadherence.
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