Ultrasound-guided transbronchial biopsy in the diagnosis of fibrosing mediastinitis-associated pulmonary hypertension.

IF 3.4 2区 医学 Q2 GENETICS & HEREDITY
Yu Zhang, Han-Xiang Song, Yong-Jia Qi, Nan-Nan Sun, Zan-Sheng Huang, Wan-Lei Fu, Jing Zhang, Felix J F Herth, Ye Fan
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Abstract

Background: Fibrosing mediastinitis is a rare benign disease frequently complicated by pulmonary hypertension. A definitive diagnosis for fibrosing mediastinitis-associated pulmonary hypertension (FM-PH) and its etiologies necessitates mediastinal biopsy and subsequent pathological assessment. Endobronchial ultrasound (EBUS)-guided transbronchial mediastinal cryobiopsy is a recently developed technique that provides diagnostic advantages over standard needle biopsy, particularly in benign mediastinal disorders. Nevertheless, their safety and efficacy in diagnosing FM-PH remain elusive.

Methods: We retrospectively studied patients with mediastinal lesion and pulmonary vascular compression who underwent both transbronchial needle aspiration and mediastinal cryobiopsy with EBUS guidance. Diagnostic yields of FM-PH and its etiologies, along with procedure-related adverse events, were analyzed. Immunohistochemical study was conducted to identify immunological properties of FM-PH.

Results: Of the 529 patients with mediastinal lesions, 80 exhibited pulmonary vessel compression, including 10 who were ultimately diagnosed with FM-PH following mediastinal biopsy and right heart catheterization. Cryobiopsy showed a higher diagnostic yield for FM-PH compared to needle aspiration (100% versus 40%, p = 0.011). Disease etiologies included pneumoconiosis in 5 cases, tuberculosis in 3, and idiopathic FM-PH in the remaining 2. Cryobiopsy appeared to be superior to needle biopsy for etiological diagnosis, although this difference was not statistically significant (80% versus 60%, p = 0.628). Immunohistochemical analyses of cryosamples revealed mixed inflammatory infiltrates of B and T lymphocytes, as well as macrophages, surrounding or within FM-PH lesions. There was no significant bleeding or other complications.

Conclusion: Transbronchial mediastinal cryobiopsy might be a safe and effective diagnostic tool for FM-PH, offering valuable information for personalized treatment.

超声引导下经支气管活检诊断纤维化性纵隔炎相关性肺动脉高压。
背景:纤维性纵隔炎是一种罕见的良性疾病,常并发肺动脉高压。纤维化性纵隔炎相关性肺动脉高压(FM-PH)的明确诊断及其病因需要纵隔活检和随后的病理评估。支气管内超声(EBUS)引导下的经支气管纵隔低温活检是一项最近发展起来的技术,它比标准的穿刺活检具有诊断优势,特别是在良性纵隔疾病中。然而,它们在诊断FM-PH中的安全性和有效性仍然难以捉摸。方法:我们回顾性研究了纵膈病变和肺血管压迫的患者,这些患者在EBUS指导下接受了经支气管穿刺和纵膈低温活检。分析了FM-PH的诊断率及其病因,以及与手术相关的不良事件。通过免疫组化研究确定FM-PH的免疫学特性。结果:529例纵隔病变患者中,80例表现为肺血管受压,其中10例在纵隔活检和右心导管置管后最终诊断为FM-PH。冷冻活检对FM-PH的诊断率高于针吸法(100% vs 40%, p = 0.011)。病因包括尘肺病5例,肺结核3例,特发性FM-PH 2例。冷冻活检在病因诊断方面似乎优于针活检,尽管这种差异没有统计学意义(80%对60%,p = 0.628)。冷冻标本的免疫组织化学分析显示,在FM-PH病变周围或内部有B淋巴细胞和T淋巴细胞以及巨噬细胞的混合炎症浸润。没有明显出血或其他并发症。结论:经支气管纵隔冷冻活检可能是一种安全有效的诊断FM-PH的工具,为个性化治疗提供有价值的信息。
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来源期刊
Orphanet Journal of Rare Diseases
Orphanet Journal of Rare Diseases 医学-医学:研究与实验
CiteScore
6.30
自引率
8.10%
发文量
418
审稿时长
4-8 weeks
期刊介绍: Orphanet Journal of Rare Diseases is an open access, peer-reviewed journal that encompasses all aspects of rare diseases and orphan drugs. The journal publishes high-quality reviews on specific rare diseases. In addition, the journal may consider articles on clinical trial outcome reports, either positive or negative, and articles on public health issues in the field of rare diseases and orphan drugs. The journal does not accept case reports.
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