Computed Tomography Findings of Pulmonary Venoocclusive Disease in Scleroderma Patients Presenting With Precapillary Pulmonary Hypertension
Computed Tomography Findings of Pulmonary Venoocclusive Disease in Scleroderma Patients Presenting With Precapillary Pulmonary Hypertension
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DOI：
10.1002/art.34501
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发表时间：
2012-09-01
影响因子：
--
通讯作者：
Montani, D.
中科院分区：
文献类型：
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作者：
Guenther, S.;Jais, X.;Montani, D.
Objective.肺静脉闭塞性疾病（PVOD）是一种罕见的肺动脉高压（PH），其特征是小肺静脉阻塞。系统性硬化症（SSc）患者出现毛细血管前PH的病理评估中已报告肺静脉受累。胸部高分辨率计算机断层扫描（HRCT）是一种用于筛选PVOD的非侵入性诊断工具。没有关于SSc伴毛细血管前PH患者的HRCT数据。我们进行了这项研究，以评估SSc伴毛细血管前PH患者PVOD HRCT征象的频率和对预后的影响。我们回顾了26例有毛细血管前PH的SSc患者和28例无肺动脉高压（PAH）或间质性肺疾病（ILD）的SSc患者的胸部HRCT资料。PVOD的HRCT影像学三联征（淋巴结肿大[57.7% vs 3.6%]、小叶中心磨玻璃影[46.2% vs 10.7%]和间隔线[88.5% vs 7.1%]）在伴有毛细血管前PH的SSc患者中的发生率显著高于不伴PAH或ILD的SSc患者（均P < 0.005）。事实上，61.5%的毛细血管前PH的SSc患者有>= 2个这些体征。心脏扩大（P < 0.0001）、肺动脉扩大（P < 0.0001）和心包积液（P < 0.0005）在伴有毛细血管前PH的SSc患者中也明显更常见。存在>= 2个PVOD放射学体征与开始PAH特异性治疗后肺水肿的发生相关（16例患者中的8例），并且从PH诊断到死亡的进展速度更快。结论。PVOD的HRCT征象常见于伴有毛细血管前PH的SSc患者，与组织学评估相关，并与肺水肿的高风险相关。
Objective. Pulmonary venoocclusive disease (PVOD) is an uncommon form of pulmonary hypertension (PH) characterized by obstruction of small pulmonary veins. Pulmonary venous involvement has been reported in pathologic assessment of patients with systemic sclerosis (SSc) presenting with precapillary PH. High-resolution computed tomography (HRCT) of the chest is a noninvasive diagnostic tool used to screen for PVOD. No HRCT data are available on SSc patients with precapillary PH. We undertook this study to evaluate the frequency and effect on prognosis of HRCT signs of PVOD in SSc patients with precapillary PH.Methods. We reviewed chest HRCT data from 26 SSc patients with precapillary PH and 28 SSc patients without pulmonary arterial hypertension (PAH) or interstitial lung disease (ILD).Results. The radiographic triad of HRCT signs of PVOD (lymph node enlargement [57.7% versus 3.6%], centrilobular ground-glass opacities [46.2% versus 10.7%], and septal lines [88.5% versus 7.1%]) was significantly more frequent in SSc patients with precapillary PH than in SSc patients without PAH or ILD (all P < 0.005). Indeed, 61.5% of SSc patients with precapillary PH had >= 2 of these signs. Cardiomegaly (P < 0.0001), pulmonary artery enlargement (P < 0.0001), and pericardial effusion (P < 0.0005) were also significantly more frequent in SSc patients with precapillary PH. Pulmonary venous involvement was histologically confirmed in 2 patients with radiographic signs of PVOD. The presence of >= 2 radiographic signs of PVOD was associated with the occurrence of pulmonary edema after initiation of PAH-specific therapy (in 8 of 16 patients) and with more rapid progression from diagnosis of PH to death.Conclusion. HRCT signs of PVOD are frequently observed in SSc patients with precapillary PH, correlated with histologic assessment, and were associated with a high risk of pulmonary edema.