A case of idiopathic portal hypertension accompanying multiple hepatic nodular regenerative hyperplasia in a patient with systemic sclerosis

被引:5
作者
Yamamoto, Arisa [1 ]
Matsuda, Hidetaka [1 ]
Hiramatsu, Katsushi [1 ]
Tsuji, Arisa [1 ]
Midori, Yohei [1 ]
Murata, Yosuke [1 ]
Tanaka, Tomoko [1 ]
Tohda, Gen [1 ]
Nosaka, Takuto [1 ]
Takahashi, Kazuto [1 ]
Naito, Tatsushi [1 ]
Ofuji, Kazuya [1 ]
Ohtani, Masahiro [1 ]
Imamura, Yoshiaki [2 ]
Nakamoto, Yasunari [1 ]
机构
[1] Univ Fukui, Fac Med Sci, Dept Internal Med 2, 23-3 Matsuoka Shimoaizuki, Eiheiji, Fukui 9101193, Japan
[2] Univ Fukui Hosp, Dept Pathol, 23-3 Matsuoka Shimoaizuki, Eiheiji, Fukui 9101193, Japan
关键词
Idiopathic portal hypertension; Nodular regenerative hyperplasia; Systemic sclerosis;
D O I
10.1007/s12328-021-01348-z
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
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.
引用
收藏
页码:820 / 826
页数:7
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