PULMONARY HYPERTENSION IN IDIOPATHIC PULMONARY FIBROSIS: PREVALENCE AND CLINICAL PROGRESS

PULMONARY HYPERTENSION IN IDIOPATHIC PULMONARY FIBROSIS: PREVALENCE AND CLINICAL PROGRESS
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DOI:
10.1177/039463201202500314
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发表时间:
2012-07-01
影响因子:
3.5
通讯作者:
Rottoli, P.
Rottoli, P.
中科院分区:
医学4区
文献类型:
--
作者:
Castria, D.;Refini, R. M.;Rottoli, P.

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本研究的目的是确定一组特发性肺纤维化(IPF)患者中肺动脉高压(PH)的患病率,调查收缩压(PAPs)和功能数据之间的任何相关性,评估临床进展并比较IPF患者有无PH的长期生存。共招募了126名IPF患者。超声心动图根据PAPS>36毫米汞柱评价PH的高患病率(39.7%,50/126),主要见于吸烟者和女性患者。回归分析显示PAPS和GT;50毫米汞柱与DLCO/VA显著相关(p=0.0294)。平均PAPs在PH发病一年后显著增加(p=0.01)。11/21例FVC和Lt;50%的患者在PH发病1年后PAPs显著增加(p=0.02)。有无PH的IPF患者的存活率有非常显著差异(p=0.0001;危险比=3.56)。这项研究表明,肺高压在IPF患者中有很高的患病率,并与死亡风险增加有关。对合并肺动脉高压的IPF患者的早期诊断非常重要,这样他们才能尽快进入肺移植的等待名单。
The aims of the present study are to define the prevalence of Pulmonary Hypertension (PH) in a cohort of Idiopathic Pulmonary Fibrosis (IPF) patients, to investigate any correlations between systolic pulmonary artery pressure (PAPs) and functional data, to evaluate clinical progress and to compare long-term survival in IPF patients with and without PH. A population of 126 IPF patients was recruited. A high prevalence of PH (39.7%, 50/126), evaluated by echocardiography on the basis of PAPs > 36 mmHg, was mainly observed in smokers and female patients. Regression analysis revealed a significant correlation between PAPs > 50 mmHg and DLCO/VA (p=0.0294). Mean PAPs was significantly greater one year after onset of PH (p=0.01). 11/21 patients with FVC < 50% had a significant increase in PAPs one year after onset of PH (p=0.02). There was a highly significant difference between survival of IPF patients with and without PH (p=0.0001; hazard ratio=3.56). This study revealed that PH has a high prevalence in patients with IPF and is associated with increased risk of mortality. Early diagnosis of IPF patients with pulmonary hypertension is important, so that they can be enrolled in waiting lists for lung transplant as soon as possible.