The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Mod Rheumatol Case Rep. 2021 Jul;5(2):285-291. doi: 10.1080/24725625.2021.1904607. Epub 2021 Apr 15.
A 43-year-old-woman admitted to our department because of abdominal pain, abdominal distension, pain on both inner thighs and blurred vision lasting for 3 months. Pancytopenia and positive anti-double stranded DNA (dsDNA) antibodies were noted 5 years prior to her hospitalisation. On admission, the patient was diagnosed with systemic lupus erythematosus (SLE) with retinal vasculitis, panniculitis, cholecystitis and enteritis. The ultrasound test revealed a large amount of ascites, splenomegaly, a hypoechoic band in the liver, and portal hypertension with mildly elevated hepatic venous wedge pressure (15 mmHg). Liver biopsy showed no evidence of hepatitis, cholangitis or liver cirrhosis, leading to the diagnosis of idiopathic non-cirrhotic portal hypertension (INCPH). Prednisolone (PSL) at a daily dose of 50 mg and intravenous cyclophosphamide pulse therapy (IVCY) were initiated for SLE, while diuretics were administered for transudative ascites associated with INCPH. Although these symptoms temporarily improved, 2 months later, SLE and ascites effusion aggravated again, and portal vein thrombosis was confirmed by computed tomography. After increasing the dose of IVCY and adding an anticoagulant agent, all symptoms improved, allowing a reduction of the PSL dose. In the present case, the exacerbation of INCPH was associated with the exacerbation of SLE and the occurrence of portal thrombosis, suggesting an autoimmune and thrombotic mechanism of INCPH. On the other hand, splenomegaly, oesophageal varices, the hypoechoic band remained unchanged, suggesting the established organised INCPH was refractory to immunosuppressive agents.
一位 43 岁女性因腹痛、腹胀、双大腿内侧疼痛和视力模糊 3 个月就诊于我科。5 年前她入院时发现全血细胞减少和抗双链 DNA（dsDNA）抗体阳性。入院时，患者被诊断为系统性红斑狼疮（SLE）合并视网膜血管炎、脂膜炎、胆囊炎和肠炎。超声检查显示大量腹水、脾肿大、肝脏低回声带和门静脉高压症，肝静脉楔压轻度升高（15mmHg）。肝脏活检未发现肝炎、胆管炎或肝硬化证据，因此诊断为特发性非肝硬化性门静脉高压症（INCPH）。给予患者泼尼松龙（PSL）50mg/d 治疗 SLE，同时给予利尿剂治疗与 INCPH 相关的渗出性腹水。尽管这些症状暂时改善，但 2 个月后，SLE 和腹水再次加重，并通过计算机断层扫描确认门静脉血栓形成。增加 IVCY 剂量并添加抗凝剂后，所有症状均改善，PSL 剂量得以减少。在本病例中，INCPH 加重与 SLE 加重和门静脉血栓形成有关，提示 INCPH 的自身免疫和血栓形成机制。另一方面，脾肿大、食管静脉曲张、低回声带保持不变，提示已建立的特发性非肝硬化性门静脉高压症对免疫抑制剂有抵抗性。
