PDF(Pubmed)
Abstract：
Intrapulmonary vasodilation leads to impaired arterial oxygenation, a hallmark of hepatopulmonary syndrome (HPS), a common pulmonary complication in end-stage liver disease. We present a case of HPS primarily diagnosed due to orthodeoxia in a 62-year-old ex-smoker with autoimmune hepatitis, under immunosuppressive treatment, but without liver cirrhosis. The patient reported dyspnea at rest that improved when supine. A recent chest CT scan showed no pulmonary embolism but indicated small nodules, bronchiectasis, and emphysema lesions. Functional breath monitoring revealed a low diffusing capacity for carbon monoxide (48% predicted). Blood gas analysis showed an increased alveolar-arterial difference, and contrast-enhanced echocardiography confirmed HPS with bubbles in the left heart chambers after the fourth cardiac cycle. Lung perfusion scintigraphy was negative for thromboembolic disease, but kidney imaging reinforced the HPS diagnosis. Our case is, to the best of our knowledge, the first presentation of HPS in a patient with autoimmune hepatitis without evidence of liver cirrhosis. This case highlights a rare instance of HPS in a patient with autoimmune hepatitis without liver cirrhosis, where orthodeoxia was the first clinical manifestation.
摘要：
肺内血管舒张导致动脉氧合受损，肝肺综合征（HPS）的标志，终末期肝病中常见的肺部并发症。我们介绍了一例HPS的病例，该病例主要是由于62岁的自身免疫性肝炎的前吸烟者的直立缺氧而诊断的。在免疫抑制治疗下，但没有肝硬化.患者报告休息时呼吸困难，仰卧时有所改善。最近的胸部CT扫描显示无肺栓塞，但显示小结节，支气管扩张,和肺气肿病变.功能性呼吸监测显示一氧化碳的扩散能力较低（预计为48％）。血气分析显示肺泡动脉差异增加,和对比增强超声心动图证实HPS在第4个心动周期后左心腔有气泡。肺灌注显像对血栓栓塞性疾病阴性，但是肾脏成像增强了HPS的诊断。我们的案子是,据我们所知,在无肝硬化证据的自身免疫性肝炎患者中首次出现HPS。这个病例突出了一个罕见的HPS的患者自身免疫性肝炎无肝硬化，其中直立缺氧是第一个临床表现。
