Liver Unit, Azienda Ospedaliera San Camillo, Forlanini, Rome, Italy.
Angiology. 2010 Nov;61(8):802-6. doi: 10.1177/0003319710369101. Epub 2010 May 24.
Portopulmonary hypertension (PPHTN) is a rare complication in patients with portal hypertension. A role of endothelin 1 (ET-1) and other cytokines was demonstrated in primary pulmonary hypertension but not in PPHTN. We evaluated the possible role of ET-1, interleukin 6 (IL-6), interleukin 1β (IL-1β), and tumor necrosis factor alpha (TNF-α) in the pathogenesis of PPHTN. Plasmatic concentrations of ET-1, IL-6, IL-1β, and TNF-α were measured in patients with pulmonary systolic arterial pressure (PAPs) >30 mm Hg and in patients with cirrhosis. In all, Six out of 11 patients with PAPs >30 mm Hg had PPHTN on right heart catheterization. The remaining 10 patients had an hyperdynamic circulation (HC). In PPHTN patients, ET-1 and IL-6 were significantly higher compared with HC and patients with cirrhosis. Endothelin 1 and IL-6 could be implicated in the pathogenesis of PPHTN. On the basis of these results, ET-1 receptor antagonists or anti-IL-6 could have a rationale in the treatment of PPHTN.
门脉高压性肺高血压（PPHTN）是门脉高压患者的一种罕见并发症。内皮素 1（ET-1）和其他细胞因子在原发性肺动脉高压中起作用，但在 PPHTN 中不起作用。我们评估了 ET-1、白细胞介素 6（IL-6）、白细胞介素 1β（IL-1β）和肿瘤坏死因子 α（TNF-α）在 PPHTN 发病机制中的可能作用。在肺动脉收缩压（PAPs）>30mmHg 的患者和肝硬化患者中测量了血浆 ET-1、IL-6、IL-1β 和 TNF-α的浓度。在 PAPs>30mmHg 的 11 名患者中，有 6 名患者经右心导管检查确诊为 PPHTN。其余 10 名患者存在高动力循环（HC）。在 PPHTN 患者中，ET-1 和 IL-6 明显高于 HC 和肝硬化患者。内皮素 1 和白细胞介素 6 可能参与了 PPHTN 的发病机制。基于这些结果，ET-1 受体拮抗剂或抗 IL-6 可能有理由用于治疗 PPHTN。
