Université Paris Cité, Inserm, PHERE, Hôpital Bichat, AP-HP, Service de Pneumologie A, Centre Constitutif du Centre de Référence des Maladies Pulmonaires Rares, FHU APOLLO, Paris, France.
University of Milano Bicocca, School of Medicine and Surgery, UOC Pneumologia, Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.
Respirology. 2024 Dec;29(12):1067-1076. doi: 10.1111/resp.14823. Epub 2024 Aug 28.
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.
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.
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.
Cystic lung lesions are rare in patients with sarcoidosis and do not influence long term prognosis.
结节病在胸部计算机断层扫描（CT）上可表现出非典型表现。囊肿是肺部结节病的一种罕见表现。本研究旨在描述一组囊性结节病患者及其临床-影像学特征和进展。
在这项回顾性、双中心研究中，我们招募了所有胸部 CT 显示肺部囊性病变的结节病患者。我们收集了临床特征、肺功能检查结果，并跟踪了诊断时和最后评估时囊肿的数量、分布和大小。
共确定了 12 名患者（6 名男性，中位年龄 53 岁）（患病率：1.9%；95%置信区间：0.8%-2.9%）。所有患者均存在多个囊性病变（中位数：14 [范围：2-216]），10/12 例为双侧分布，11/12 例存在微结节和结节，4/12 例存在纤维性病变。7 名患者的肺功能检查正常，3 名患者存在阻塞性综合征，1 名患者存在限制性综合征，1 名患者同时存在这两种综合征。在随访期间（中位数：10 年[范围 1-16 年]），4 名患者的囊肿数量增加。最后评估时，3/12 名患者用力肺活量下降>10%，3/12 名患者一氧化碳弥散量（DLCO）下降>10%。诊断时 DLCO 较低，以及 CT 上存在结节或纤维性病变与囊肿数量增加相关。
囊性肺部病变在结节病患者中较为罕见，不影响长期预后。
