Yamamoto Motohisa, Taniguchi Hiroaki, Ohara Mikiko, Suzuki Chisako, Naishiro Yasuyoshi, Ozeki Itaru, Yamamoto Hiroyuki, Takahashi Hiroki, Imai Kohzoh
First Department of Internal Medicine, School of Medicine, Sapporo Medical University.
Nihon Rinsho Meneki Gakkai Kaishi. 2004 Feb;27(1):40-7. doi: 10.2177/jsci.27.40.
A 54-year-old female experienced morning stiffness and arthralgia of the hands from November 2001 and consulted her local doctor in January 2002. Hematological data showed liver dysfunction and antinuclear antibody was positive. In February 2002, the patient was admitted to our hospital to investigate these abnormalities of liver function further. The features of arthritis, thrombocytopenia with elevated platelet-associated IgG, positive antinuclear antibody, and anti-DNA antibody lead to a diagnosis of systemic lupus erythematosus. Liver biopsy revealed infiltration of Glisson's capsule by plasma cells, compatible with autoimmune hepatitis. We performed hepatic venography to investigate severe pancytopenia, remarkable splenomegaly and esophageal varices. Wedged hepatic venous pressure was mildly elevated, and hepatic veins displayed sharp angles, smooth walls and development of anastomosis with each other. These findings suggested idiopathic portal hypertension. Cytopenia and liver dysfunction gradually improved on treatment with 40 mg/day prednisolone. Esophageal varices were disappeared, and splenomegaly had improved after 6 months. As autoimmune factors are considered to underlie the development of idiopathic portal hypertension with systemic lupus erythematosus, steroid administration represents a therapeutic option in this condition.
一名54岁女性自2001年11月起出现手部晨僵和关节痛，并于2002年1月咨询当地医生。血液学检查显示肝功能异常，抗核抗体呈阳性。2002年2月，患者因进一步调查肝功能异常情况入住我院。关节炎、血小板减少伴血小板相关IgG升高、抗核抗体阳性及抗DNA抗体阳性等特征导致系统性红斑狼疮的诊断。肝活检显示汇管区有浆细胞浸润，符合自身免疫性肝炎。我们进行了肝静脉造影以调查严重全血细胞减少、显著脾肿大和食管静脉曲张情况。肝楔压轻度升高，肝静脉呈锐角、壁光滑且相互吻合。这些发现提示为特发性门静脉高压症。使用泼尼松龙40mg/天治疗后，血细胞减少和肝功能障碍逐渐改善。6个月后食管静脉曲张消失，脾肿大有所改善。由于自身免疫因素被认为是系统性红斑狼疮伴发特发性门静脉高压症的发病基础，因此类固醇给药是这种情况下的一种治疗选择。
