肺功能接近正常的结节病相关性肺动脉高压患者。

Sarcoidosis-associated pulmonary hypertension in patients with near-normal lung function.

机构信息

Pulmonary Institute, Soroka University Medical Center, Beer Sheva, Israel.

出版信息

Int J Tuberc Lung Dis. 2013 Mar;17(3):406-11. doi: 10.5588/ijtld.12.0428.

DOI:10.5588/ijtld.12.0428

PMID:23407231

Abstract

SETTING

Tertiary care medical centre in Israel.

BACKGROUND

Pulmonary hypertension (PH) is a predictor of poor outcome in patients with sarcoidosis. Early diagnosis may improve outcome.

OBJECTIVE

To determine factors that might contribute to the early diagnosis of PH in sarcoidosis patients with near normal lung function tests.

DESIGN

Retrospective patient review.

METHODS

Data from 127 patients with sarcoidosis and near normal lung function tests (forced vital capacity > 70%, forced expiratory volume in 1 second > 70% and diffusion capacity of carbon monoxide [D(LCO)] > 60%), who underwent high resolution computed tomography (HRCT) scan, the 6-minute walk distance (6MWD) test and echocardiogram were analysed. Demographic, clinical and HRCT findings were compared between patients with and those without PH.

RESULTS

Thirty-six patients (28.3%) had PH. Patients with PH tended to have lower D(LCO) (68% ± 8 vs. 75% ± 17, P = 0.038), 6MWD (308 m ± 106 vs. 486 m ± 99, P = 0.009) and exercise saturation (91 ± 4 vs. 95 ± 3, P = 0.0001) compared to those without PH. HRCT patterns in PH showed higher frequencies of interstitial thickening (P = 0.004), ground glass appearance (P = 0.01) and fibrosis (P = 0.032). In logistic regression, only 6MWD was predictive of PH (P = 0.005, 95%CI 0.970-0.995).

CONCLUSION

Physiological and radiographic characteristics appeared to differentiate patients with PH from those without. Physicians should be aware of PH in patients with sarcoidosis, even in those with near normal lung function.

摘要

背景

肺动脉高压（PH）是结节病患者预后不良的预测因素。早期诊断可能改善预后。

目的

确定有助于早期诊断肺功能正常结节病患者 PH 的因素。

设计

回顾性患者分析。

方法

对 127 例肺功能正常（用力肺活量 > 70%，1 秒用力呼气量 > 70%，一氧化碳弥散量 [D（LCO）]> 60%）结节病患者的临床资料进行回顾性分析，这些患者均接受了高分辨率计算机断层扫描（HRCT）、6 分钟步行距离（6MWD）试验和超声心动图检查。比较 PH 患者与无 PH 患者的临床资料和 HRCT 结果。

结果

36 例患者（28.3%）存在 PH。PH 患者的 D（LCO）水平（68%±8%比 75%±17%，P=0.038）、6MWD（308m±106m 比 486m±99m，P=0.009）和运动饱和度（91%±4%比 95%±3%，P=0.0001）均较低。PH 患者的 HRCT 表现为间质性增厚（P=0.004）、磨玻璃样外观（P=0.01）和纤维化（P=0.032）的发生率较高。在 logistic 回归中，只有 6MWD 可预测 PH（P=0.005，95%CI 0.970-0.995）。