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CTLA-4 缺陷患者的肉芽肿性淋巴细胞性间质性肺病的成功治疗。

Successful Treatment of Granulomatous-lymphocytic Interstitial Lung Disease in a Patient with CTLA-4 Deficiency.

机构信息

Division of Infectious Diseases and Respiratory Medicine, Department of Internal Medicine, National Defense Medical College, Japan.

Department of Pediatrics, National Defense Medical College, Japan.

出版信息

Intern Med. 2023 Mar 15;62(6):871-875. doi: 10.2169/internalmedicine.0076-22. Epub 2022 Aug 10.

DOI:10.2169/internalmedicine.0076-22
PMID:35945007
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10076143/
Abstract

Common variable immunodeficiency (CVID) causes granulomatous-lymphocytic interstitial lung disease (GLILD) and has a poor prognosis. We herein report a case of GLILD in a 49-year-old woman with CTLA-4 deficiency-associated CVID. The patient presented with dyspnea that had worsened over the past two years. A laboratory examination revealed hypoglobulinemia and pancytopenia. Chest computed tomography showed diffuse infiltrative and granular shadows in the bilateral interstitium. A flow cytometric analysis of blood cells and genetic testing confirmed CTLA-4 deficiency. We performed video-assisted thoracoscopic surgery for the pathological diagnosis of GLILD and to exclude infection and malignancy. Corticosteroid treatment successfully improved the condition of the patient.

摘要

普通变异性免疫缺陷病（CVID）可引起肉芽肿性淋巴细胞性间质性肺病（GLILD），且预后较差。我们在此报告一例 CTLA-4 缺陷相关 CVID 导致的 GLILD 患者。该患者因呼吸困难就诊，症状在过去两年中逐渐加重。实验室检查发现低球蛋白血症和全血细胞减少。胸部计算机断层扫描显示双侧肺间质弥漫性浸润性和颗粒状阴影。血细胞流式细胞分析和基因检测证实 CTLA-4 缺陷。我们为明确 GLILD 的病理诊断、排除感染和恶性肿瘤而行电视辅助胸腔镜手术。皮质类固醇治疗成功改善了患者的病情。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c333/10076143/63eefa811e69/1349-7235-62-0871-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c333/10076143/bfd7fd10b135/1349-7235-62-0871-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c333/10076143/63eefa811e69/1349-7235-62-0871-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c333/10076143/bfd7fd10b135/1349-7235-62-0871-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c333/10076143/63eefa811e69/1349-7235-62-0871-g002.jpg

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本文引用的文献

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Treatment Strategies for GLILD in Common Variable Immunodeficiency: A Systematic Review.常见可变免疫缺陷中的 GLILD 的治疗策略：系统评价。
Front Immunol. 2021 Apr 15;12:606099. doi: 10.3389/fimmu.2021.606099. eCollection 2021.
2
Longitudinal observation and decline of neutralizing antibody responses in the three months following SARS-CoV-2 infection in humans.人类感染 SARS-CoV-2 后三个月内中和抗体反应的纵向观察和下降。
Nat Microbiol. 2020 Dec;5(12):1598-1607. doi: 10.1038/s41564-020-00813-8. Epub 2020 Oct 26.
3
The Lung in Primary Immunodeficiencies: New Concepts in Infection and Inflammation.
原发性免疫缺陷中的肺部疾病：感染与炎症的新概念。
Front Immunol. 2018 Aug 8;9:1837. doi: 10.3389/fimmu.2018.01837. eCollection 2018.
4
Clinical efficacy of a next-generation sequencing gene panel for primary immunodeficiency diagnostics.新一代测序基因面板在原发性免疫缺陷诊断中的临床疗效。
Clin Genet. 2018 Mar;93(3):647-655. doi: 10.1111/cge.13163. Epub 2018 Feb 2.
5
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Intern Med. 2017 Nov 1;56(21):2899-2902. doi: 10.2169/internalmedicine.7757-16. Epub 2017 Sep 15.
6
Immune deficiency and autoimmunity in patients with CTLA-4 (CD152) mutations.携带CTLA-4（CD152）突变患者的免疫缺陷与自身免疫
Clin Exp Immunol. 2017 Oct;190(1):1-7. doi: 10.1111/cei.12997. Epub 2017 Jul 21.
7
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8
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J Med Genet. 2016 Sep;53(9):575-90. doi: 10.1136/jmedgenet-2015-103690. Epub 2016 Jun 1.
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Nat Med. 2014 Dec;20(12):1410-1416. doi: 10.1038/nm.3746. Epub 2014 Oct 20.