A case of idiopathic portal hypertension accompanying multiple hepatic nodular regenerative hyperplasia in a patient with systemic sclerosis
A case of idiopathic portal hypertension accompanying multiple hepatic nodular regenerative hyperplasia in a patient with systemic sclerosis
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DOI：
10.1007/s12328-021-01348-z
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发表时间：
2021-04-22
影响因子：
1
通讯作者：
Nakamoto, Yasunari
中科院分区：
文献类型：
--
作者：
Yamamoto, Arisa;Matsuda, Hidetaka;Nakamoto, Yasunari
特发性门静脉高压症（IPH）是引起结节性再生性增生（NRH）的背景性疾病之一。此外，IPH患者伴有自身免疫性疾病，如系统性红斑狼疮（SLE）和系统性硬化症（SSc），更容易在肝脏中形成NRH。一位76岁的女性在过去的30年里一直意识到雷诺氏现象和硬皮病。在这种情况下，她提出了腹部饱胀，她的影像学分析显示腹水和多个肝结节。在Gd-EOB-DTPA增强磁共振成像（EOB-MRI）上，在肝胆期的结节中观察到甜甜圈样摄取。肝活检显示结节由增生的肝细胞组成，无纤维间隔，结节旁可见扩张的血窦。相反，背景肝脏显示外周门静脉出现狭窄，门静脉区出现致密纤维化。最终诊断为SSc患者合并IPH时出现肝脏多发性NRH。此病例提示，NRH可能意外地在伴有IPH的自身免疫性疾病患者中被诊断出来。
Idiopathic portal hypertension (IPH) is one of the background diseases causing nodular regenerative hyperplasia (NRH). Furthermore, IPH patients accompanied with autoimmune diseases, such as systemic lupus erythematosus (SLE) and systemic sclerosis (SSc), are more likely to form NRH in the liver. A 76-year-old woman had been aware of the Raynaud's phenomenon and scleroderma for the past 30 years. In this case, she presented with abdominal fullness, and her imaging analysis revealed ascites and multiple liver nodules. On Gd-EOB-DTPA enhanced magnetic resonance imaging (EOB-MRI), donut-like uptake was observed in the nodules in the hepatobiliary phase. Liver biopsy of a nodule demonstrated that it was composed of hyperplastic hepatocytes without fibrous septa, and dilated sinusoids were observed beside the nodule. Conversely, background liver showed that peripheral portal veins appeared stenotic with dense fibrosis in the portal area. The final diagnosis was that multiple NRH of the liver developed in SSc patient accompanying IPH. This case suggests that NRH may be unexpectedly diagnosed in patients with autoimmune diseases accompanying IPH.