Liver disease in primary antibody deficiencies.

IF 3 4区医学 Q2 ALLERGY

Current Opinion in Allergy and Clinical Immunology Pub Date : 2024-12-01 Epub Date: 2024-09-25 DOI:10.1097/ACI.0000000000001035

Patrick Bez, Klaus Warnatz

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Abstract

Purpose of review: Liver disease has emerged as a major risk factor for increased mortality in patients with common variable immunodeficiency (CVID). This is mostly due to presinusoidal portal hypertension (PHTN) frequently secondary to nodular regenerative hyperplasia (NRH). Its pathogenesis is still poorly understood and treatment strategies for its various stages are often guided by trial and error. This review summarizes the most recent findings in the light of previous literature.

Recent findings: In the last 2 years, different groups have addressed pathology, diagnostics, treatment, and liver transplantation. Histological examinations seem to support the pathogenetic sequence of T-cell mediated infiltration and damage of the sinusoidal space with secondary development of NRH, pericellular fibrosis, and the manifestation of PHTN. While markers of the early phase - beyond slight elevation of cholestatic enzymes - are still missing, elevated liver stiffness and splenomegaly above 16 cm longitudinal diameter have been suggested as warning signs for PHTN in CVID patients. Data on immunosuppressive treatment of this manifestation is still very heterogeneous, but a recent report on liver transplantation was encouraging for end stage liver disease.

Summary: Liver disease deserves higher attention in the management of CVID. More studies are needed to understand its pathogenesis and optimal treatment.

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原发性抗体缺乏症的肝病。

审查目的：肝脏疾病已成为常见变异性免疫缺陷病（CVID）患者死亡率升高的主要风险因素。这主要是由于窦前门静脉高压症（PHTN）经常继发于结节性再生增生（NRH）。目前对其发病机理仍知之甚少，针对不同阶段的治疗策略也往往是在反复试验的基础上制定的。本综述根据以往文献总结了最新研究结果：在过去两年中，不同的研究小组对病理学、诊断、治疗和肝移植进行了探讨。组织学检查似乎支持由 T 细胞介导的窦状空间浸润和损伤、继发 NRH、细胞周纤维化和 PHTN 表现的发病顺序。虽然除了胆汁淤积酶的轻微升高外，早期阶段的标志物仍然缺失，但肝脏硬度升高和脾脏纵径超过 16 厘米已被认为是 CVID 患者 PHTN 的预警信号。有关这种表现的免疫抑制治疗的数据仍很不一致，但最近一份有关肝移植治疗终末期肝病的报告令人鼓舞。需要进行更多的研究，以了解其发病机制和最佳治疗方法。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Current Opinion in Allergy and Clinical Immunology 医学-过敏

CiteScore

5.90

自引率

3.60%

发文量

109

审稿时长

6-12 weeks

期刊介绍： This reader-friendly, bimonthly resource provides a powerful, broad-based perspective on the most important advances from throughout the world literature. Featuring renowned guest editors and focusing exclusively on one to three topics, every issue of Current Opinion in Allergy and Clinical Immunology delivers unvarnished, expert assessments of developments from the previous year. Insightful editorials and on-the-mark invited reviews cover key subjects such as upper airway disease; mechanisms of allergy and adult asthma; paediatric asthma and development of atopy; food and drug allergies; and immunotherapy.