Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort: results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature
Prevalence of pulmonary hypertension in an unselected, mixed connective tissue disease cohort: results of a nationwide, Norwegian cross-sectional multicentre study and review of current literature
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DOI：
10.1093/rheumatology/kes430
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发表时间：
2013-07-01
期刊：
影响因子：
5.5
通讯作者：
Gran, Jan Tore
中科院分区：
文献类型：
--
作者：
Gunnarsson, Ragnar;Andreassen, Arne K.;Gran, Jan Tore
目标。本研究的目的是评估未选择的 MCTD 队列中肺动脉高压 (PH) 的总体患病率，并通过系统数据库搜索回顾当前知识。方法。由 147 名成年 MCTD 患者组成的全国多中心队列最初通过超声心动图、高分辨率计算机断层扫描 (HRCT)、肺功能测试和 N 末端脑钠肽前体 (NT-proBNP) 筛查 PH，然后平均随访 5.6 年。当超声心动图估计肺动脉收缩压 > 40 mmHg 时，进行右侧心导管插入术。根据2009年欧洲心脏病学会和欧洲呼吸学会指南诊断PH。结果。纳入时，2.0% (3/147) 已建立 PH。随访期间发现了另外两名 PH 患者，队列中总 PH 频率为 3.4% (5/147)。所有五人的血清 NT-proBNP 均升高。两人患有单纯性肺动脉高压（PAH），三人患有与间质性肺病相关的肺动脉高压（PH-ILD）。 3 名 PH 患者在随访期间死亡。该队列中的其他 9 名患者也死亡，但他们在死亡前均未出现超声心动图 PH 体征。结论。当前未选择的 MCTD 队列的数据表明，PH 的患病率远低于先前研究的预期，但证实了该疾病并发症的严重性。
Objectives. The aim of this study was to assess the overall prevalence of pulmonary hypertension (PH) in an unselected MCTD cohort and review the current knowledge with a systematic database search.Methods. A nationwide multicentre cohort of 147 adult MCTD patients were initially screened for PH by echocardiography, high-resolution computed tomography (HRCT), pulmonary function tests and N-terminal pro-brain natriuretic peptide (NT-proBNP) and then followed up for a mean of 5.6 years. Right-sided heart catheterization was performed when estimated pulmonary artery systolic pressure was > 40 mmHg on echocardiography. PH was diagnosed according to the 2009 European Society of Cardiology and European Respiratory Society guidelines.Results. At inclusion, 2.0% (3/147) had established PH. Two additional PH patients were identified during follow-up, giving a total PH frequency in the cohort of 3.4% (5/147). All five had elevated serum NT-proBNP. Two had isolated pulmonary arterial hypertension (PAH) and three PH associated with interstitial lung disease (PH-ILD). Three PH patients died during follow-up. Nine other patients in the cohort also died, but none of them had echocardiographic signs of PH prior to death.Conclusion. The data from the current unselected MCTD cohort suggest that the prevalence of PH is much lower than expected from previous studies but confirm the seriousness of the disease complication.