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结缔组织病相关间质性肺疾病患者支气管肺泡灌洗液和血液的免疫学特征。

Immunological characteristics of bronchoalveolar lavage fluid and blood across connective tissue disease-associated interstitial lung diseases.

机构信息

Inflammation and Immunology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.

Aimed Analytics GmbH, Bonn, Germany.

出版信息

Front Immunol. 2024 Oct 25;15:1408880. doi: 10.3389/fimmu.2024.1408880. eCollection 2024.

DOI:10.3389/fimmu.2024.1408880
PMID:39524435
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11543407/
Abstract

Interstitial lung disease (ILD) is a serious complication of connective tissue diseases (CTDs). The heterogeneity of ILDs reflects differences in pathogenesis among diseases. This study aimed to clarify the characteristics of CTD-ILDs via a detailed analysis of the bronchoalveolar lavage fluid (BALF) and blood immune cells. BALF and blood samples were collected from 39 Japanese patients with newly diagnosed ILD: five patients with Sjögren's syndrome (SS), eight patients with dermatomyositis (DM), six patients with rheumatoid arthritis (RA), six patients with systemic sclerosis, four patients with anti-neutrophil cytoplasmic antibody-associated vasculitis, and 10 patients with idiopathic interstitial pneumonia. We performed single-cell RNA sequencing to analyze the gene expression profiles in these patients' immune cells. In patients with SS, B cells in the BALF were increased and genes associated with the innate and acquired immunity were enriched in both the BALF and blood. In contrast, patients with DM showed an upregulation of genes associated with viral infection in both the BALF and blood. In patients with RA, neutrophils in the BALF tended to increase, and their gene expression patterns changed towards inflammation. These disease-specific characteristics may help us understand the pathogenesis for each disease and discover potential biomarkers.

摘要

间质性肺病 (ILD) 是结缔组织病 (CTD) 的严重并发症。ILD 的异质性反映了疾病之间发病机制的差异。本研究旨在通过详细分析支气管肺泡灌洗液 (BALF) 和血液免疫细胞来阐明 CTD-ILD 的特征。从 39 名新诊断为 ILD 的日本患者中采集 BALF 和血液样本：5 名干燥综合征 (SS) 患者、8 名皮肌炎 (DM) 患者、6 名类风湿关节炎 (RA) 患者、6 名系统性硬化症患者、4 名抗中性粒细胞胞质抗体相关性血管炎患者和 10 名特发性间质性肺炎患者。我们进行了单细胞 RNA 测序，以分析这些患者免疫细胞中的基因表达谱。在 SS 患者中，BALF 中的 B 细胞增加，BALF 和血液中均富集了与先天和获得性免疫相关的基因。相比之下，DM 患者的 BALF 和血液中与病毒感染相关的基因表达上调。在 RA 患者中，BALF 中的中性粒细胞倾向于增加，其基因表达模式向炎症转变。这些疾病特异性特征可能有助于我们了解每种疾病的发病机制并发现潜在的生物标志物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/4c59dc117d93/fimmu-15-1408880-g009.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/f1cf7dfe830f/fimmu-15-1408880-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/eec1e1acea97/fimmu-15-1408880-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/1f0587dda121/fimmu-15-1408880-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/2d94e088262e/fimmu-15-1408880-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/e4c5e502e755/fimmu-15-1408880-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/b3280e57f524/fimmu-15-1408880-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/27cdddd83c97/fimmu-15-1408880-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/347b4ab7fa9f/fimmu-15-1408880-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/4c59dc117d93/fimmu-15-1408880-g009.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/f1cf7dfe830f/fimmu-15-1408880-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/eec1e1acea97/fimmu-15-1408880-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/1f0587dda121/fimmu-15-1408880-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/2d94e088262e/fimmu-15-1408880-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/e4c5e502e755/fimmu-15-1408880-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/b3280e57f524/fimmu-15-1408880-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/27cdddd83c97/fimmu-15-1408880-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/347b4ab7fa9f/fimmu-15-1408880-g008.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/365a/11543407/4c59dc117d93/fimmu-15-1408880-g009.jpg

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