肉样瘤病中的囊性肺:临床放射学特征和演变。

IF 6.6 2区 医学 Q1 RESPIRATORY SYSTEM
Respirology Pub Date : 2024-12-01 Epub Date: 2024-08-28 DOI:10.1111/resp.14823
Giovanni Franco, Marie-Pierre Debray, Niccolò Anzani, Almerico Marruchella, Aurélie Cazes, Pierre Le Guen, Camille Taillé, Paola Faverio, Raphaël Borie, Fabrizio Luppi, Bruno Crestani
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引用次数: 0

摘要

背景和目的:肉样瘤病在胸部计算机断层扫描(CT)中可有不典型的表现。囊肿是肺肉样瘤病的一种罕见表现。本研究旨在描述一系列囊性肉样瘤病患者及其临床放射学特征和病情进展:在这项回顾性双中心研究中,我们招募了所有经胸部 CT 检查发现肺部囊性病变的肉样瘤病患者。我们收集了临床特征、肺部检查结果,并追踪了确诊时和最后一次评估时囊肿的数量、分布和大小:共发现 12 名患者(6 名男性,中位年龄 53 岁)(发病率:1.9%;95% 置信区间:0.8%-2.9%)。所有患者均出现多发性囊性病变(中位数:14 [范围:2-216]),其中 10/12 例为双侧分布,11/12 例为微小结节和结节,4/12 例为纤维化病变。7 名患者的肺功能检查正常,3 名患者患有阻塞性综合征,1 名患者患有限制性综合征,1 名患者同时患有阻塞性综合征和限制性综合征。在随访期间(中位数:10 年[1-16 年]),4 名患者的囊肿数量有所增加。在最后一次评估中,3/12 名患者的用力肺活量下降>10%,3/12 名患者的一氧化碳弥散容量(DLCO)下降>10%。诊断时DLCO降低、CT上出现结节或纤维化病变与囊肿数量增加有关:结论:肺囊肿病变在肉样瘤病患者中很少见,不会影响长期预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Cystic lung in sarcoidosis: Clinico-radiologic characteristic and evolution.

Background and objective: Sarcoidosis can manifest with atypical findings on chest computed tomography (CT). Cysts are a rare manifestation of lung sarcoidosis. The aim of the study was to describe a series of patients with cystic sarcoidosis and their clinical-radiological characteristics and progression.

Methods: In this retrospective, bicentric study we recruited all patients affected by sarcoidosis with lung cystic lesions at chest CT. We collected clinical characteristics, pulmonary tests and tracked number, distribution and size of the cysts at diagnosis and at the last evaluation.

Results: Twelve patients (6 males, median age 53 years) were identified (prevalence: 1.9%; 95% Confidence Interval: 0.8%-2.9%). All patients presented multiple cystic lesions (median number: 14 [range: 2-216]) with a bilateral distribution in 10/12, micronodules and nodules in 11/12 and fibrotic lesions in 4/12. Seven patients had normal lung function test, three had an obstructive syndrome, one had a restrictive syndrome and one had coexistence of both. During follow-up (median: 10 years [range 1-16 years]), an increase of the number of cysts was observed in four patients. At last evaluation, 3/12 patients experienced a decline of forced vital capacity >10% and 3/12 patients a decline of diffusing capacity for carbon monoxide (DLCO) >10%. A lower DLCO at diagnosis, and the presence of nodules or fibrotic lesions on CT were associated with an increase in the number of cysts.

Conclusion: Cystic lung lesions are rare in patients with sarcoidosis and do not influence long term prognosis.

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来源期刊
Respirology
Respirology 医学-呼吸系统
CiteScore
10.60
自引率
5.80%
发文量
225
审稿时长
1 months
期刊介绍: Respirology is a journal of international standing, publishing peer-reviewed articles of scientific excellence in clinical and clinically-relevant experimental respiratory biology and disease. Fields of research include immunology, intensive and critical care, epidemiology, cell and molecular biology, pathology, pharmacology, physiology, paediatric respiratory medicine, clinical trials, interventional pulmonology and thoracic surgery. The Journal aims to encourage the international exchange of results and publishes papers in the following categories: Original Articles, Editorials, Reviews, and Correspondences. Respirology is the preferred journal of the Thoracic Society of Australia and New Zealand, has been adopted as the preferred English journal of the Japanese Respiratory Society and the Taiwan Society of Pulmonary and Critical Care Medicine and is an official journal of the World Association for Bronchology and Interventional Pulmonology.
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