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IgG4相关性肺病的临床特征:基于单中心的经验

Clinical characteristics of IgG4-related lung disease: a single-center based experience.

作者信息

Han Dong-Ge, Ying Chun-Lin, Zhao Tian-Qi, Tong Qiao-Yun, Liu Wei

机构信息

The First College of Clinical Medical Science, Three Gorges University, Yichang, China.

Institute of Digestive Disease, Three Gorges University, 8 Daxue Road, Yichang, 443000, China.

出版信息

Clin Rheumatol. 2025 Sep 10. doi: 10.1007/s10067-025-07678-4.

Abstract

BACKGROUND

IgG4-related lung disease (IgG4-RLD) is a rare autoimmune condition. This study aims to systematically analyze the clinical characteristics of IgG4-RLD to enhance clinicians' awareness and improve patient outcomes.

METHODS

This retrospective analysis investigates the clinical data of 20 patients diagnosed with IgG4-RLD at the Yichang Central People's Hospital between January 2019 and April 2025.

RESULTS

The mean age at diagnosis among the 20 patients with IgG4-RLD was 60.10 ± 10.34 years, with a male-to-female ratio of 3:1. The most frequent clinical manifestations were hemoptysis and abnormalities in pulmonary imaging. Eight patients (40.00%) presented with isolated pulmonary involvement, while 12 patients (60.00%) exhibited extra-pulmonary organ involvement, with lymph node involvement being the most prevalent. The pulmonary imaging characteristics primarily included solid nodular patterns in 10 patients (50.00%) and round ground-glass opacity (GGO) patterns in 9 patients (45.00%). Patients with round GGO patterns exhibited a higher incidence of hemoptysis and predominantly had a history of respiratory conditions (P < 0.05). Patients with solid nodular patterns exhibited significantly higher percentages of eosinophils and lymphocytes, as well as elevated serum IgG levels, compared to those with round GGO patterns (P < 0.05). By contrast, patients with round GGO patterns demonstrated significantly higher percentages of neutrophils and a markedly increased neutrophil-to-lymphocyte ratio (NLR) compared to those with solid nodular patterns (P < 0.05).

CONCLUSIONS

The clinical manifestations of IgG4-RLD are complex and heterogeneous. Clinicians need to acquire a thorough understanding of these features in order to develop personalized diagnostic and therapeutic approaches, thereby enhancing patient outcomes. Key Points • The pulmonary imaging characteristics of IgG4-RLD were primarily manifested as solid nodular patterns and round GGO patterns. • Patients with round GGO patterns were more likely to experience hemoptysis, and the majority of these patients presented with a history of respiratory conditions. • Patients with solid nodular patterns demonstrated significantly higher percentages of eosinophils, lymphocytes, and serum IgG levels compared to those with round GGO patterns. • Patients with round GGO patterns demonstrated significantly higher percentages of neutrophils and a markedly increased NLR compared to those with solid nodular patterns.

摘要

背景

IgG4相关性肺病(IgG4-RLD)是一种罕见的自身免疫性疾病。本研究旨在系统分析IgG4-RLD的临床特征,以提高临床医生的认识并改善患者预后。

方法

本回顾性分析调查了2019年1月至2025年4月在宜昌市中心人民医院确诊为IgG4-RLD的20例患者的临床资料。

结果

20例IgG4-RLD患者的诊断时平均年龄为60.10±10.34岁,男女比例为3:1。最常见的临床表现为咯血和肺部影像学异常。8例(40.00%)表现为孤立性肺部受累,12例(60.00%)表现为肺外器官受累,其中淋巴结受累最为常见。肺部影像学特征主要包括10例(50.00%)的实性结节型和9例(45.00%)的圆形磨玻璃影(GGO)型。圆形GGO型患者咯血发生率较高,且主要有呼吸系统疾病史(P<0.05)。与圆形GGO型患者相比,实性结节型患者的嗜酸性粒细胞和淋巴细胞百分比以及血清IgG水平显著更高(P<0.05);相比之下,圆形GGO型患者的中性粒细胞百分比显著更高,中性粒细胞与淋巴细胞比值(NLR)明显升高(P<0.05)。

结论

IgG4-RLD的临床表现复杂且具有异质性。临床医生需要全面了解这些特征,以制定个性化的诊断和治疗方法,从而改善患者预后。要点:•IgG4-RLD的肺部影像学特征主要表现为实性结节型和圆形GGO型。•圆形GGO型患者更容易咯血,且这些患者大多有呼吸系统疾病史。•与圆形GGO型患者相比,实性结节型患者的嗜酸性粒细胞、淋巴细胞百分比和血清IgG水平显著更高。•与实性结节型患者相比,圆形GGO型患者的中性粒细胞百分比显著更高,NLR明显升高。

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