Sakamaki Yusuke, Konishi Konosuke, Hashiguchi Akinori, Tomita Shigeki, Kubota Eiji, Itoh Hiroshi, Hayashi Koichi
Nihon Jinzo Gakkai Shi. 2016;58(1):45-54.
The patient was a 48-year-old Japanese woman diagnosed as having systemic lupus erythematosus at the age of 21 years when she presented with fever and an erythematous skin rash on her face and extremities. Prednisolone was initiated at that time. Thirteen days before admission to our hospital, she was referred to us by her family physician. Upon admission, blood tests showed pancytopenia, hypocomplementemia, and renal dysfunction, as well as the presence of lupus anticoagulant. Urinalysis showed abundant proteinuria and heavy microscopic hematuria. After performing a renal biopsy, we initiated immunosuppressive therapy and an anticoagulant. On the 22nd hospital day, microangiopathic hemolytic anemia appeared with the progression of thrombocytopenia and renal failure, and the patient subsequently underwent ten sessions of plasma exchange. After the commencement of the plasma exchange, her general condition improved. Her renal dysfunction, however, continued to progress, and hemodialysis was started on the 36th hospital day. The light microscopy showed severe endo- and extra-capillary proliferative glomerulonephritis with abundant crescents, and massive thrombi in the capillary lumen of the glomeruli. The arterioles contained occlusive hyaline materials. An immunofluorescence study showed granular staining of immunoglobulins and complements along the glomerular capillary wall. An electron microscopy examination revealed the presence of electron-dense deposits in the subepithelial and intramembranous areas of the glomeruli, but subendothelial deposits were absent. For cases with lupus nephritis (LN), immunosuppressive therapy based on corticosteroid remains the mainstay of treatment. However, immunosuppression alone may be insufficient when antiphospholipid antibody syndrome and thrombotic microangiopathy (TMA) are also present, and other treatment modalities including antiplatelet therapy, anticoagulation, and plasma exchange are likely to be necessary, as illustrated by the present case. Although the mechanism responsible for LN remains uncertain, we report a case of LN suggesting that TMA is associated with renal dysfunction.
该患者为一名48岁的日本女性，21岁时因发热、面部及四肢出现红斑性皮疹而被诊断为系统性红斑狼疮，当时开始使用泼尼松龙治疗。入院前13天，她被家庭医生转诊至我院。入院时，血液检查显示全血细胞减少、补体减少和肾功能不全，同时存在狼疮抗凝物。尿液分析显示大量蛋白尿和严重镜下血尿。进行肾活检后，我们开始了免疫抑制治疗和抗凝治疗。在住院第22天，随着血小板减少和肾衰竭的进展出现了微血管病性溶血性贫血，患者随后接受了10次血浆置换。血浆置换开始后，她的一般状况有所改善。然而，她的肾功能仍继续恶化，在住院第36天开始进行血液透析。光镜检查显示严重的毛细血管内和毛细血管外增生性肾小球肾炎，伴有大量新月体形成，肾小球毛细血管腔内有大量血栓。小动脉含有闭塞性透明物质。免疫荧光研究显示免疫球蛋白和补体沿肾小球毛细血管壁呈颗粒状染色。电子显微镜检查显示肾小球上皮下和膜内区域存在电子致密沉积物，但无内皮下沉积物。对于狼疮性肾炎（LN）病例，基于皮质类固醇的免疫抑制治疗仍然是主要的治疗方法。然而，当同时存在抗磷脂抗体综合征和血栓性微血管病（TMA）时，仅免疫抑制可能不足，其他治疗方式包括抗血小板治疗、抗凝治疗和血浆置换可能是必要的，本病例即说明了这一点。尽管LN的发病机制尚不确定，但我们报告了一例LN病例，提示TMA与肾功能不全有关。
