III Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Plocka 26 st., 01-138, Warsaw, Poland.
Radiology Department, National Tuberculosis and Lung Diseases Research Institute, Warsaw, Poland.
Lung. 2018 Dec;196(6):715-720. doi: 10.1007/s00408-018-0155-1. Epub 2018 Sep 5.
Pneumothorax often develops in pulmonary Langerhans cell histiocytosis (PLCH), but some patients take a long time to be correctly diagnosed.
This study assessed the frequency of pneumothorax in PLCH and analysed the role of chest computed tomography (CT) in the prompt diagnosis.
Of the 90 patients with PLCH seen from 2000 to 2015, 29 (32%) had pneumothorax as the initial finding. In this group, 18 (62%) patients were diagnosed within 1 month, whereas the diagnosis was delayed for 4-120 months in 11 (38%) patients.
Patients who had pneumothorax as the initial sign of PLCH tended to be younger (mean age 27.7 ± 7.92 vs. 39.9 ± 13.21 years; P = 0.0001), male (69% vs. 43%; P = 0.028), smoked less (mean pack/years 8.4 ± 6.85 vs. 19 ± 17.16; P = 0.003), and had a significantly lower mean FVC (77.96 ± 19.62 vs. 89.47 ± 21.86% pred.; P = 0.015) and FEV (68.6 ± 19.93 vs. 79.4 ± 21.48% pred.; P = 0.03 than patients who had no pneumothorax. Recurrent pneumothorax was diagnosed more frequently in the group with a delayed diagnosis (82% vs. 39%; P = 0.02). CT was performed in all of the patients who were diagnosed promptly, but in none of the patients with a delayed diagnosis.
Patients who had pneumothorax as the initial sign of PLCH were younger, more frequently men, and had greater respiratory impairment than those who had no pneumothorax. CT in patients with pneumothorax led to a correct diagnosis of this disease.
气胸常发生于肺朗格汉斯细胞组织细胞增生症（PLCH），但有些患者需要很长时间才能得到正确诊断。
本研究评估了 PLCH 中气胸的发生率，并分析了胸部计算机断层扫描（CT）在及时诊断中的作用。
2000 年至 2015 年间共 90 例 PLCH 患者，其中 29 例（32%）以气胸为首发表现。在这一组中，18 例（62%）患者在 1 个月内得到诊断，而 11 例（38%）患者的诊断延迟了 4-120 个月。
以气胸为首发表现的 PLCH 患者倾向于更年轻（平均年龄 27.7 ± 7.92 岁 vs. 39.9 ± 13.21 岁；P=0.0001）、男性（69% vs. 43%；P=0.028）、吸烟较少（平均包/年 8.4 ± 6.85 岁 vs. 19 ± 17.16 岁；P=0.003），且平均 FVC（77.96 ± 19.62% pred. vs. 89.47 ± 21.86% pred.；P=0.015）和 FEV（68.6 ± 19.93% pred. vs. 79.4 ± 21.48% pred.；P=0.03）明显较低，与无气胸的患者相比。在延迟诊断组中，更频繁地诊断出复发性气胸（82% vs. 39%；P=0.02）。所有及时诊断的患者均进行了 CT 检查，但无一例延迟诊断的患者进行了 CT 检查。
以气胸为首发表现的 PLCH 患者比无气胸的患者更年轻、更常为男性，且呼吸功能损害更大。气胸患者的 CT 检查可导致对该病的正确诊断。
