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酷似弥漫性泛细支气管炎的特发性细支气管炎

Idiopathic bronchiolitis mimicking diffuse panbronchiolitis.

作者信息

Poletti Venerino, Chilosi Marco, Trisolini Rocco, Cancellieri Alessandra, Zompatori Maurizio, Lazzari Agli Luigi, Boaron Maurizio, Schulte W, Theegarten D, Guzman J, Costabel Ulrich

机构信息

Dipartimento di Malattie dell'Apparato Respiratorio e del Torace, Ospedale G.B. Morgagni, Forlì, Italy.

出版信息

Sarcoidosis Vasc Diffuse Lung Dis. 2003 Mar;20(1):62-8.

PMID:12737282
Abstract

Diffuse panbronchiolitis (DPB) is a distinct sinobronchial syndrome which has been almost exclusively reported in East Asia; only a few cases have been observed in non-Asians so far. Since 1990, when one of us described the first European case, we have screened all patients presenting with a clinical, physiopathological and radiological pattern compatible with DPB, in order to assess if the paucity of proven cases in Europe could reflect a lack of awareness of this disease among both pulmonologists and pathologists. Over a 10-years period we identified four additional DPBs, but also interestingly observed five cases of "idiopathic" bronchiolitis (IB) that had both clinical symptoms and high-resolution computed tomography (HRCT) findings indistinguishable from those of DPB, but did not show the typical histological changes. All these patients were Caucasians and presented with dyspnea, productive cough, an obstructive or mixed impairment on pulmonary function tests and chest HRCT features strongly suggesting a grade II to IV DPB. We suggest that diseases classified as IB in this study might be homogeneous entities which are clinically and radiologically similar to DPB.

摘要

弥漫性泛细支气管炎(DPB)是一种独特的鼻窦支气管综合征,几乎仅在东亚地区有报道;迄今为止,在非亚洲人中仅观察到少数病例。自1990年我们中的一人描述了首例欧洲病例以来,我们对所有临床表现、生理病理和放射学特征与DPB相符的患者进行了筛查,以评估欧洲确诊病例稀少是否反映了肺科医生和病理学家对这种疾病认识不足。在10年期间,我们又发现了4例DPB,但有趣的是还观察到5例“特发性”细支气管炎(IB),这些病例的临床症状和高分辨率计算机断层扫描(HRCT)表现与DPB难以区分,但未显示出典型的组织学变化。所有这些患者均为白种人,表现为呼吸困难、咳痰、肺功能检查显示阻塞性或混合性损伤,胸部HRCT特征强烈提示为II至IV级DPB。我们认为,本研究中归类为IB的疾病可能是在临床和放射学上与DPB相似的同质实体。

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