Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.
Divisão de Pneumologia, Instituto do Coração (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil.
Chest. 2020 Dec;158(6):2440-2448. doi: 10.1016/j.chest.2020.05.609. Epub 2020 Jun 29.
Pulmonary Langerhans cell histiocytosis (PLCH) determines reduced exercise capacity. The speculated mechanisms of exercise impairment in PLCH are ventilatory and cardiocirculatory limitations, including pulmonary hypertension (PH).
What are the mechanisms of exercise limitation, the exercise capacity, and the prevalence of dynamic hyperinflation (DH) and PH in PLCH?
In a cross-sectional study, patients with PLCH underwent an incremental treadmill cardiopulmonary exercise test with an evaluation of DH, pulmonary function tests, and transthoracic echocardiography. Those patients with lung diffusing capacity for carbon monoxide (Dlco) < 40% predicted and/or transthoracic echocardiogram with tricuspid regurgitation velocity > 2.5 m/s and/or with indirect PH signs underwent right heart catheterization.
Thirty-five patients were included (68% women; mean age, 47 ± 11 years). Ventilatory and cardiocirculatory limitations, impairment suggestive of PH, and impaired gas exchange occurred in 88%, 67%, 29%, and 88% of patients, respectively. The limitation was multifactorial in 71%, exercise capacity was reduced in 71%, and DH occurred in 68% of patients. FEV and Dlco were 64 ± 22% predicted and 56 ± 21% predicted. Reduction in Dlco, an obstructive pattern, and air trapping occurred in 80%, 77%, and 37% of patients. FEV and Dlco were good predictors of exercise capacity. The prevalence of PH was 41%, predominantly with a precapillary pattern, and mean pulmonary artery pressure correlated best with FEV and tricuspid regurgitation velocity.
PH is frequent and exercise impairment is common and multifactorial in PLCH. The most prevalent mechanisms are ventilatory, cardiocirculatory, and suggestive of PH limitations.
ClinicalTrials.gov; No.: NCT02665546; URL: www.clinicaltrials.gov.
肺朗格汉斯细胞组织细胞增生症（PLCH）导致运动能力下降。推测 PLCH 运动障碍的机制是通气和心肺循环受限，包括肺动脉高压（PH）。
PLCH 患者的运动受限机制、运动能力以及动态过度充气（DH）和 PH 的患病率是什么？
在一项横断面研究中，PLCH 患者接受递增式跑步机心肺运动试验，评估 DH、肺功能检查和经胸超声心动图。那些一氧化碳弥散量（Dlco）<40%预计值和/或经胸超声心动图三尖瓣反流速度>2.5m/s和/或有间接 PH 征象的患者进行右心导管检查。
共纳入 35 例患者（68%为女性；平均年龄 47±11 岁）。通气和心肺循环受限、提示 PH 的损害、气体交换受损分别发生在 88%、67%、29%和 88%的患者中。71%的患者存在多因素限制，71%的患者运动能力降低，68%的患者出现 DH。FEV 和 Dlco 分别为预计值的 64±22%和 56±21%。80%、77%和 37%的患者出现 Dlco 降低、阻塞模式和空气滞留。FEV 和 Dlco 是运动能力的良好预测指标。PH 的患病率为 41%，主要为毛细血管前模式，平均肺动脉压与 FEV 和三尖瓣反流速度相关性最好。
PH 在 PLCH 中很常见，运动障碍也很常见且多因素，常见机制为通气、心肺循环受限和提示 PH 受限。
ClinicalTrials.gov；编号：NCT02665546；网址：www.clinicaltrials.gov。
