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复发性多软骨炎伴孤立性气管支气管受累并合并干燥综合征:一例报告

Relapsing polychondritis with isolated tracheobronchial involvement complicated with Sjogren's syndrome: A case report.

作者信息

Chen Jun-Yan, Li Xiao-Yan, Zong Chen

机构信息

Department of Pulmonary and Critical Care Medicine, The Second Hospital of Shanxi Medical University, Taiyuan 030000, Shanxi Province, China.

出版信息

World J Clin Cases. 2022 Jul 6;10(19):6563-6570. doi: 10.12998/wjcc.v10.i19.6563.

Abstract

BACKGROUND

Relapsing polychondritis (RP) is a rare, long-term, and potentially life-threatening disease characterised by recurrent paroxysmal inflammation that can involve and destroy the cartilage of the external ear, nose, larynx, and trachea.

CASE SUMMARY

We here report a case of RP involving solely the tracheobronchial cartilage ring (and not the auricular. nasal or articular cartilage) complicated by Sjögren's syndrome in a 47-year-old female whose delayed diagnosis caused a sharp decline in pulmonary function. After corticosteroid treatment, her pulmonary function improved.

CONCLUSION

In such cases, our experience suggested that 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) and fiberoptic bronchoscopy should be used to diagnose airway chondritis as relapsing polychondritis in the early phase of disease.

摘要

背景

复发性多软骨炎(RP)是一种罕见的、长期的、可能危及生命的疾病,其特征为反复发作的阵发性炎症,可累及并破坏外耳、鼻、喉和气管的软骨。

病例摘要

我们在此报告一例仅累及气管支气管软骨环(而非耳廓、鼻或关节软骨)的复发性多软骨炎病例,该病例发生于一名47岁女性,并发干燥综合征,其延迟诊断导致肺功能急剧下降。经皮质类固醇治疗后,她的肺功能得到改善。

结论

在这类病例中,我们的经验表明,应使用18F-氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描(18F-FDG PET/CT)和纤维支气管镜在疾病早期诊断气道软骨炎为复发性多软骨炎。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6bc1/9294918/0fb8df7577a0/WJCC-10-6563-g001.jpg

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