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自身免疫性肝病的B细胞耗竭治疗:适应症和结局的回顾性分析

B cell depletion for autoimmune liver diseases: A retrospective review of indications and outcomes.

作者信息

Costaguta Guillermo A, Álvarez Fernando

机构信息

Gastroenterology, Hepatology and Nutrition CHU Sainte-Justine Montreal Quebec Canada.

Department of Pediatrics University of Montreal Montreal Quebec Canada.

出版信息

JPGN Rep. 2024 Jun 14;5(3):326-333. doi: 10.1002/jpr3.12098. eCollection 2024 Aug.

Abstract

OBJECTIVES

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.

METHODS

We retrospectively reviewed rituximab-treated patients between 2017 and 2022. Demographics, previous treatments, reasons for B cell depletion, response, and adverse effects were noted.

RESULTS

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 ( = 0.02) and 36 IU/L ( = 0.008), respectively. Mean γ-glutamyl transpeptidase decreased from 105 to 25 IU/L ( = 0.01). Immunoglobulin G levels were normalized in all patients ( = 0.01). No severe adverse events were observed. One patient had persistent hypogammaglobulinemia, and another had lymphopenia.

CONCLUSION

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.

摘要

目的

在北美,儿童自身免疫性肝炎的发病率为每10万名儿童中有0.23例,若不治疗,预后不佳。类固醇是首选治疗方法,但并非总是有效。B细胞耗竭是一种安全有效的治疗方法,可采用类固醇节省方案,尤其是对于无法耐受副作用的患者。

方法

我们回顾性分析了2017年至2022年间接受利妥昔单抗治疗的患者。记录了人口统计学资料、既往治疗情况、B细胞耗竭的原因、反应及不良反应。

结果

纳入6例平均年龄为10.2岁的患者。所有患者均患有合并症,导致类固醇治疗未成功或不理想。利妥昔单抗在平均随访8个月时开始使用。6个月后,平均丙氨酸转氨酶和天冬氨酸转氨酶水平分别从575 IU/L和342 IU/L降至28 IU/L(P = 0.02)和36 IU/L(P = 0.008)。平均γ-谷氨酰转肽酶从105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)。未观察到严重不良事件。1例患者持续存在低丙种球蛋白血症,另1例患者出现淋巴细胞减少。

结论

B细胞耗竭是自身免疫性肝病的一种有效且安全的治疗方法,应作为一种选择,特别是对于复发患者,此类患者不适合使用类固醇或已出现不依从情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3cdf/11322033/9c45dcad84ab/JPR3-5-326-g001.jpg

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