Kadoh Yoichi, Yoshino Jun, Oka Tomohiro, Itoga Kenichi, Hanada Maki, Niino Daisuke, Nagai Atsushi, Ichinose Kunihiro, Kanda Takeshi
Division of Nephrology, Department of Internal Medicine, Faculty of Medicine, Shimane University, 89-1 Enya-Cho, Izumo, Shimane, 693-8501, Japan.
Department of Neurology, Faculty of Medicine, Shimane University, Izumo , Shimane, Japan.
CEN Case Rep. 2025 Feb 7. doi: 10.1007/s13730-025-00973-8.
Posterior reversible encephalopathy syndrome (PRES) is a rare clinico-neuroradiologic disease associated with various conditions, such as hypertension, eclampsia, chronic kidney disease, and autoimmune diseases. Here, we present the case of the unusual occurrence of PRES with hypertensive emergency and renal insufficiency in a 37-year-old woman previously undiagnosed with systemic lupus erythematosus (SLE) and lupus nephritis. The patient was emergently admitted to our hospital with sudden onset of visual impairment, headache, and high blood pressure, and she was eventually diagnosed with PRES by brain magnetic resonance imaging (MRI). Her PRES-associated clinical symptoms and MRI abnormalities were improved following anti-hypertensive treatment with calcium channel blocker. A kidney biopsy revealed diffuse proliferative glomerulonephritis with a full-house immunofluorescence pattern and fibrinoid necrosis in small blood vessels, suggesting a class IV-G (A) lupus nephritis with vasculitis. The immunosuppressive therapy with intravenous methylprednisolone pulse followed by oral prednisolone, mycophenolate mofetil, and intravenous belimumab, attenuated SLE-associated clinical manifestations including butterfly rush, edema, renal dysfunction, and proteinuria. Our case highlights the need to consider PRES as an initial clinical presentation of lupus nephritis and provide the early diagnosis and timely treatment to achieve a favorable outcome.
后部可逆性脑病综合征（PRES）是一种罕见的临床神经放射学疾病，与多种病症相关，如高血压、子痫、慢性肾病和自身免疫性疾病。在此，我们报告一例37岁女性的病例，该患者既往未被诊断为系统性红斑狼疮（SLE）和狼疮性肾炎，却出现了伴有高血压急症和肾功能不全的PRES异常情况。患者因突发视力障碍、头痛和高血压紧急入院，最终通过脑部磁共振成像（MRI）被诊断为PRES。使用钙通道阻滞剂进行抗高血压治疗后，她与PRES相关的临床症状和MRI异常情况得到改善。肾脏活检显示弥漫性增殖性肾小球肾炎，免疫荧光呈满堂亮模式，小血管有纤维蛋白样坏死，提示为伴有血管炎的IV - G（A）级狼疮性肾炎。静脉注射甲泼尼龙脉冲治疗，随后口服泼尼松龙、霉酚酸酯和静脉注射贝利尤单抗的免疫抑制疗法，减轻了包括蝶形红斑、水肿、肾功能不全和蛋白尿在内的与SLE相关的临床表现。我们的病例强调了需要将PRES视为狼疮性肾炎的初始临床表现，并提供早期诊断和及时治疗以获得良好预后。
