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利妥昔单抗成功治疗常见可变免疫缺陷中的肉芽肿性/淋巴细胞间质性肺病。

Successful rituximab treatment of granulomatous/lymphocytic interstitial lung disease in common variable immunodeficiency.

作者信息

Králíčková P, Kubcová Š, Kočová E, Bartoš V, Souček O, Rozsíval P, Vaníček H, Krčmová I, Ravčuková B, Grombiříková H, Freiberger T

出版信息

Epidemiol Mikrobiol Imunol. 2018 Winter;67(3):142-148.

PMID:30602282
Abstract

Successful rituximab treatment of granulomatous/lymphocytic interstitial lung disease in common variable immunodeficiency Common variable immunodeficiency, a heterogeneous group of diseases, represents a clinically relevant form of antibody immunodeficiency. Granulomatous/lymphocytic interstitial lung disease is among the most serious complications. A case report is presented of a young women with granulomatous/lymphocytic interstitial lung disease and splenomegaly accompanied by pancytopenia. Intravenous rituximab treatment in monotherapy (at a weekly dose of 375 mg/m2 for four consecutive weeks, repeated six months later) not only led to a significant improvement in clinical symptoms but also to positive morphological and functional lung changes, mitigation of pancytopenia, considerable reduction of alkaline phosphatase level, and disappearance of splenic granulomas. The treatment was well tolerated without any side effects. The case report presented suggests possible efficacy and safety of rituximab monotherapy in patients with a complicated form of common variable immunodeficiency. KEYWORDS Rituximab - antibody immunodeficiency - lung disease - treatment Epidemiol. Mikrobiol. Imunol., 67, 2018, č. 3, s. 142-148.

摘要

利妥昔单抗成功治疗常见变异型免疫缺陷中的肉芽肿性/淋巴细胞间质性肺病 常见变异型免疫缺陷是一组异质性疾病,是临床上一种具有重要意义的抗体免疫缺陷形式。肉芽肿性/淋巴细胞间质性肺病是最严重的并发症之一。本文报告一例年轻女性患者,患有肉芽肿性/淋巴细胞间质性肺病及脾肿大并伴有全血细胞减少。采用利妥昔单抗单药静脉治疗(每周剂量375mg/m²,连续四周,六个月后重复),不仅使临床症状显著改善,还使肺部出现积极的形态学和功能改变,全血细胞减少症得到缓解,碱性磷酸酶水平大幅降低,脾肉芽肿消失。治疗耐受性良好,未出现任何副作用。该病例报告提示利妥昔单抗单药治疗对于复杂型常见变异型免疫缺陷患者可能具有疗效和安全性。关键词 利妥昔单抗 - 抗体免疫缺陷 - 肺病 - 治疗 《流行病学、微生物学与免疫学》,67卷,2018年,第3期,第142 - 148页

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Front Immunol. 2023 Sep 19;14:1264482. doi: 10.3389/fimmu.2023.1264482. eCollection 2023.
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Managing Granulomatous-Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders: e-GLILDnet International Clinicians Survey.管理常见可变免疫缺陷疾病中的肉芽肿性淋巴细胞性间质性肺病:e-GLILDnet 国际临床医生调查。
Front Immunol. 2020 Nov 26;11:606333. doi: 10.3389/fimmu.2020.606333. eCollection 2020.