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一名曾接受胸腺瘤治疗的白种男性出现弥漫性泛细支气管炎,表现为副胸腺综合征。

Diffuse panbronchiolitis as parathymic syndrome in a Caucasian man previously treated for thymoma.

作者信息

Carnevale Aldo, Lucioni Elisa, Daniele Marta Maria, Contoli Marco, Giganti Melchiore, Marku Brunilda

机构信息

Section of Radiology, Department of Translational Medicine and for Romagna, University of Ferrara, Ferrara, Italy.

Research Centre on Asthma and COPD, Department of Medical Sciences, University of Ferrara, Ferrara, Italy.

出版信息

Radiol Case Rep. 2021 Aug 9;16(10):3029-3033. doi: 10.1016/j.radcr.2021.07.026. eCollection 2021 Oct.

DOI:10.1016/j.radcr.2021.07.026
PMID:34408804
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8361232/
Abstract

Diffuse panbronchiolitis (DPB) is a rare disease characterized by bronchiolitis and chronic sinusitis. Being largely restricted to East Asia, its actual incidence in Caucasian patients is probably underestimated. DPB has been described in association with thymic neoplasms, mainly arising as a consequence of immune dysregulation. We present a rare case of DPB diagnosed in a 69-year-old Caucasian man who had undergone surgery for stage 2A thymoma a year before. The patient came to our hospital complaining of exertional dyspnea and productive cough, with a persistent lung consolidation described at chest X-rays. High resolution computed tomography (CT) showed diffuse centrilobular micronodules and solid nodules, tree-in-bud opacities, peripheral consolidations and cylindrical bronchiectasis. Sinus disease was also demonstrated by CT. Analysis of bronchoalveolar lavage showed marked granulocyte inflammation and allowed the isolation of Haemophilus Influenzae. Consequently, the diagnosis of DPB was reached by integrating clinical, and radiological data. Long-term therapy with azithromycin was prescribed, and was found to be effective in controlling symptoms and reducing radiological abnormalities at 6-month clinical and CT follow-up. Confidence with the radiological presentation and clinical significance of DPB is necessary, since the condition is responsive and reversible to long-term macrolide treatment, the effect of which is mainly attributed to an anti-inflammatory, and immunoregulatory action.

摘要

弥漫性泛细支气管炎(DPB)是一种以细支气管炎和慢性鼻窦炎为特征的罕见疾病。该病主要局限于东亚地区,其在白种人患者中的实际发病率可能被低估。DPB已被描述与胸腺肿瘤有关,主要是由于免疫失调所致。我们报告一例罕见的DPB病例,患者为一名69岁的白种男性,一年前因2A期胸腺瘤接受了手术。患者因劳力性呼吸困难和咳痰前来我院就诊,胸部X线显示肺部持续实变。高分辨率计算机断层扫描(CT)显示弥漫性小叶中心性微结节和实性结节、树芽征、外周实变和柱状支气管扩张。CT也显示了鼻窦疾病。支气管肺泡灌洗分析显示明显的粒细胞炎症,并分离出流感嗜血杆菌。因此,通过整合临床和放射学数据得出DPB的诊断。给予阿奇霉素长期治疗,在6个月的临床和CT随访中发现其对控制症状和减少放射学异常有效。由于DPB对长期大环内酯类治疗有反应且可逆转,其作用主要归因于抗炎和免疫调节作用,因此有必要了解DPB的放射学表现和临床意义。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9313/8361232/a7dcf97bf2ca/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9313/8361232/514a9db952c8/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9313/8361232/a809d557225c/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9313/8361232/a7dcf97bf2ca/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9313/8361232/514a9db952c8/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9313/8361232/a809d557225c/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9313/8361232/a7dcf97bf2ca/gr3.jpg

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Front Med (Lausanne). 2019 Oct 31;6:246. doi: 10.3389/fmed.2019.00246. eCollection 2019.
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Prognostic Value of Concomitant Bronchiectasis in Newly Diagnosed Diffuse Panbronchiolitis Patients on a Maintenance Therapy with Macrolides.大环内酯维持治疗新发弥漫性泛细支气管炎患者支气管扩张症的预测价值。
Can Respir J. 2019 Mar 10;2019:4913814. doi: 10.1155/2019/4913814. eCollection 2019.
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Thymic neoplasms patients complicated with bronchiectasis: Case series in a Chinese hospital and literature review.
胸腺癌胸腺切除术后弥漫性泛细支气管炎和重症肌无力的序贯发展:病例报告。
BMC Pulm Med. 2024 Jul 2;24(1):310. doi: 10.1186/s12890-024-03134-2.
胸腺瘤患者合并支气管扩张症:一家中国医院的病例系列及文献复习
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The effects of azithromycin on patients with diffuse panbronchiolitis: a retrospective study of 29 cases.阿奇霉素治疗弥漫性泛细支气管炎患者的疗效:29 例回顾性研究。
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