贝利单抗治疗自身免疫性肝炎和原发性胆管炎-一个病例系列

IF 4.7 Q2 IMMUNOLOGY
Mirjam Kolev , Adela-Cristina Sarbu , Burkhard Möller , Britta Maurer , Florian Kollert , Nasser Semmo
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引用次数: 1

摘要

背景:大多数自身免疫性肝炎(AIH)患者通过既定的治疗方案获得完全缓解。对于不耐受或对这些药物反应不足的患者,剩余的选择有限,需要新的治疗方法。在原发性胆道胆管炎(PBC)中,熊去氧胆酸(UDCA)和贝特类药物可显著改善预后,但仍有一部分患者难治性疾病。在难治性AIH和/或PBC患者中,我们使用了一种新的治疗策略,即抗b细胞活化因子贝利单抗。前三名患者同时患有Sjögren病。这三种疾病之间的联系因素是B细胞活化,包括B细胞活化因子(BAFF)水平升高。此外,belimumab已被证明对Sjögren的疾病有益。目的和方法回顾性研究在瑞士伯尔尼大学医院接受抗baff治疗的6例伴有或不伴有Sjögren病的AIH或PBC患者的治疗反应。结果在所有3例AIH患者中,贝利单抗改善了疾病控制,并有助于绕过或减少皮质类固醇和钙调磷酸酶抑制剂的问题副作用。在PBC患者(n = 3)中,尽管IgM降低或正常化,但肝功能检查没有明显改善。所有伴有Sjögren's疾病的患者(n = 3)都有sicca症状的改善,三分之二的患者最初的疲劳明显减轻,随着时间的推移逐渐减轻。结论belimumab可能是AIH患者的一种有希望的治疗选择,需要进一步的研究。然而,中国人民银行的回应并不令人信服。对干燥症状和疲劳的效果令人鼓舞。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Belimumab treatment in autoimmune hepatitis and primary biliary cholangitis – a case series

Belimumab treatment in autoimmune hepatitis and primary biliary cholangitis – a case series

Background

The majority of patients with autoimmune hepatitis (AIH) achieve complete remission with established treatment regiments. In patients with intolerance or insufficient response to these drugs, the remaining options are limited and novel treatment approaches necessary. In primary biliary cholangitis (PBC), ursodeoxycholic acid (UDCA) and fibrates have improved prognosis dramatically, but there remains a proportion of patients with refractory disease.

In patients with refractory AIH and/or PBC, we used a novel treatment strategy with the anti-B cell activating factor, belimumab. The first three patients had concomitant Sjögren's disease. The connecting element between all three diseases is B cell activation, including elevated levels of the B cell activating factor (BAFF). Furthermore, belimumab has been shown to be beneficial in Sjögren's disease.

Aims and methods

To retrospectively investigate treatment response in six patients with AIH or PBC with or without concomitant Sjögren's disease treated with the anti-BAFF therapy belimumab at the University Hospital in Bern, Switzerland.

Results

In all three patients with AIH, belimumab improved disease control and helped by-pass or reduce problematic side effects from corticosteroids and calcineurin inhibitors. In PBC patients (n = 3), there was no clear improvement of liver function tests, despite reduction or normalization of IgM. All patients with concomitant Sjögren's disease (n = 3) had an improvement of sicca symptoms and two out of three patients experienced an initially marked reduction in fatigue, which lessened over time.

Conclusions

Belimumab may be a promising treatment option for patients with AIH and further investigations are needed. In PBC however, response was not convincing. The effects on sicca symptoms and fatigue were encouraging.

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来源期刊
Journal of Translational Autoimmunity
Journal of Translational Autoimmunity Medicine-Immunology and Allergy
CiteScore
7.80
自引率
2.60%
发文量
33
审稿时长
55 days
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