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复发性多软骨炎的喉部受累:173例患者的临床及CT表现

Laryngeal involvement in relapsing polychondritis: clinical and CT findings in 173 patients.

作者信息

Bai Xiao, Yu Ruohan, Wang Zhengang

机构信息

Department of Rheumatology and Immunology, Beijing Tsinghua Changgung Hospital, Beijing, China.

Department of Rheumatology and Immunology, Peking University International Hospital, Beijing, China.

出版信息

RMD Open. 2025 May 8;11(2):e005397. doi: 10.1136/rmdopen-2024-005397.

Abstract

INTRODUCTION/OBJECTIVES: Relapsing polychondritis (RP) is a rare autoimmune disorder primarily affecting cartilaginous structures. We aimed to characterise the clinical features and CT findings of laryngeal involvement in RP, hypothesising that specific CT patterns correlate with clinical manifestations.

METHODS

We retrospectively analysed 173 patients with confirmed RP. Demographic and clinical data were collected, and laryngeal, tracheal, and bronchial CT findings were reviewed. Statistical analyses identified factors associated with laryngeal involvement and airway stenosis.

RESULTS

Notably, 66% of asymptomatic patients displayed CT evidence of airway damage, with laryngeal involvement in 41.1% (44/107), tracheal involvement in 80.9% (140/173), and bronchial involvement in 36.4% (63/107). Cricoid erosion and broadening with mucosal hyperplasia were the predominant laryngeal findings. Significant associations with laryngeal involvement included younger age at disease onset (p<0.05), longer disease duration (p<0.01) and multiorgan manifestations (p<0.01).

CONCLUSIONS

Laryngeal involvement is common in RP patients with airway manifestations, second only to tracheal involvement. CT findings of cricoid erosion and broadening are characteristic. Vigilant clinical monitoring is recommended for RP patients with identified risk factors to facilitate early detection and management. Future research should focus on developing targeted interventions for this high-risk subgroup of RP patients.

摘要

引言/目的:复发性多软骨炎(RP)是一种罕见的自身免疫性疾病,主要影响软骨结构。我们旨在描述RP患者喉部受累的临床特征和CT表现,推测特定的CT模式与临床表现相关。

方法

我们回顾性分析了173例确诊为RP的患者。收集了人口统计学和临床数据,并对喉部、气管和支气管的CT表现进行了评估。统计分析确定了与喉部受累和气道狭窄相关的因素。

结果

值得注意的是,66%的无症状患者显示有气道损伤的CT证据,其中喉部受累占41.1%(44/107),气管受累占80.9%(140/173),支气管受累占36.4%(63/107)。环状软骨侵蚀和增宽伴黏膜增生是主要的喉部表现。与喉部受累显著相关的因素包括发病年龄较轻(p<0.05)、病程较长(p<0.01)和多器官表现(p<0.01)。

结论

喉部受累在有气道表现的RP患者中很常见,仅次于气管受累。环状软骨侵蚀和增宽的CT表现具有特征性。建议对具有已确定危险因素的RP患者进行密切的临床监测,以便于早期发现和管理。未来的研究应侧重于为这一RP高危亚组患者开发有针对性的干预措施。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8a9c/12067797/f01edb3744d7/rmdopen-11-2-g001.jpg

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