Vannozzi Giancarlo, Tozzi Alessandro, Chibbaro Guido, Mello Giorgio, Ponzalli Massimo
Department of Gastroenterology, AOU Careggi, Florence, Italy.
Eur J Gastroenterol Hepatol. 2008 Oct;20(10):1032-5. doi: 10.1097/MEG.0b013e3282f4488d.
Here we report a case of a 25-year-old woman with mesenteric and hepatic sarcoidosis without lung involvement complicated by severe noncirrhotic portal hypertension. In 1992, at the clinical presentation, she had abdominal pain, asthenia, and weight loss. Splenomegaly, signs of flogosis, sideropenic anemia, and cholestasis were observed. Laparoscopic abdominal exploration and histological analysis demonstrated noncaseating granulomas of the liver, abdominal lymph nodes, and mesenteric connective tissue. The clinical course was severe with episodic remissions and recrudescences characterized by ascites (mild or moderate), elevation of bilirubin levels (mean: 1.1 mg/dl; range: 0.9-3.5 mg/dl), reduction of albumin levels (mean: 4 g/dl; range: 3.4-4.2 g/dl), and prolongation of elevated international normalized ratio (mean: 1; range: 0.9-1.4). In 1997, the patient had variceal bleeding. Complete hemostasis was obtained with band ligation. Liver function was preserved, and until 2000 the disease remained stable. In 2001, the patient became pregnant. At the 36th week of gestation, the patient delivered a healthy female infant and afterwards remained in clinical remission. This report stresses that sarcoidosis can have a hepatic and mesenteric involvement in absence of thoracic lymphadenopathy. Portal hypertension may be severe, and in absence of cirrhosis it may be associated with portal thrombosis. Finally, portal hypertension in patients with hepatic sarcoidosis and preserved liver function should not be considered as an absolute contraindication to pregnancy.
在此，我们报告一例25岁女性，患有肠系膜和肝结节病，无肺部受累，并发严重的非肝硬化门静脉高压症。1992年，临床表现为腹痛、乏力和体重减轻。观察到脾肿大、炎症体征、缺铁性贫血和胆汁淤积。腹腔镜腹部探查和组织学分析显示肝脏、腹部淋巴结和肠系膜结缔组织有非干酪样肉芽肿。临床病程严重，有间歇性缓解和复发，表现为腹水（轻度或中度）、胆红素水平升高（平均：1.1mg/dl；范围：0.9 - 3.5mg/dl）、白蛋白水平降低（平均：4g/dl；范围：3.4 - 4.2g/dl）以及国际标准化比值升高（平均：1；范围：0.9 - 1.4）。1997年，患者发生静脉曲张出血。通过套扎实现了完全止血。肝功能得以保留，直到2000年病情保持稳定。2001年，患者怀孕。在妊娠第36周时，患者产下一名健康女婴，之后病情处于临床缓解状态。本报告强调，结节病可在无胸内淋巴结肿大的情况下累及肝脏和肠系膜。门静脉高压可能很严重，在无肝硬化的情况下可能与门静脉血栓形成有关。最后，肝功能保留的肝结节病患者的门静脉高压不应被视为妊娠的绝对禁忌证。
