Moschos John, Leontiadis Grigoris I, Kelly Clive, Henry James, Kadis Savvas
Department of Gastroenterology, Queen Elizabeth Hospital, Sheriff Hill, Gateshead NE6 9SX, UK.
BMC Gastroenterol. 2005 May 26;5:16. doi: 10.1186/1471-230X-5-16.
Patients with systemic sclerosis may develop mild abnormalities of liver function tests. More serious hepatic involvement has been well documented but is rare. Idiopathic portal hypertension had been reported only in a few female patients with systemic sclerosis.
An 82-year-old man with known systemic sclerosis presented with melaena. Urgent gastroscopy revealed oesophageal varices, which re-started bleeding during the procedure and were treated ensocopically, with Sengstaken tube and glypressin. Liver function tests and coagulation were normal. Non-invasive liver screen (including hepatitis viral serology and autoantibodies) was negative. Ultrasound scan of the abdomen revealed a small liver with coarse texture and no focal lesion. Hepato-portal flow was demonstrated in the portal vein. The spleen was enlarged. A moderate amount of free peritoneal fluid was present. A CT scan confirmed the absence of portal vein thrombosis. One month following discharge the patient had a liver biopsy. Histological examination showed essentially normal liver tissue; there was no evidence of any excess inflammation and no features to suggest cirrhosis or drug-induced liver disease. Taking into account the above evaluation we concluded that the patient had idiopathic portal hypertension.
Both male and female patients with systemic sclerosis may -- rarely -- develop idiopathic portal hypertension.
系统性硬化症患者可能会出现肝功能检查的轻度异常。更严重的肝脏受累情况已有充分记录，但较为罕见。特发性门静脉高压仅在少数患有系统性硬化症的女性患者中被报道过。
一名82岁的已知患有系统性硬化症的男性出现黑便。紧急胃镜检查发现食管静脉曲张，在检查过程中再次出血，并通过使用三腔二囊管和垂体后叶素进行内镜治疗。肝功能检查和凝血功能正常。非侵入性肝脏筛查（包括肝炎病毒血清学和自身抗体）呈阴性。腹部超声扫描显示肝脏较小，质地粗糙，无局灶性病变。门静脉显示肝门静脉血流。脾脏肿大。有中等量的游离腹腔积液。CT扫描证实无门静脉血栓形成。出院后一个月，患者进行了肝脏活检。组织学检查显示肝脏组织基本正常；没有任何过度炎症的证据，也没有提示肝硬化或药物性肝病的特征。综合上述评估，我们得出结论，该患者患有特发性门静脉高压。
患有系统性硬化症的男性和女性患者都可能——很少见——发生特发性门静脉高压。
