一例罕见的狼疮性肾炎表现为血栓性微血管病伴弥漫性假肾小管形成，可能由非典型溶血尿毒症综合征引起。
A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome.
作者信息
Ono Masafumi, Ohashi Naro, Namikawa Akio, Katahashi Naoko, Ishigaki Sayaka, Tsuji Naoko, Isobe Shinsuke, Iwakura Takamasa, Sakao Yukitoshi, Tsuji Takayuki, Kato Akihiko, Fujigaki Yoshihide, Shimizu Akira, Yasuda Hideo
机构信息
Blood Purification Unit, Hamamatsu University School of Medicine, Japan.
Division of Nephrology, First Department of Medicine, Hamamatsu University School of Medicine, Japan.
出版信息
Intern Med. 2018 Jun 1;57(11):1617-1623. doi: 10.2169/internalmedicine.0228-17. Epub 2018 Feb 9.
A 31-year-old woman was admitted to our hospital for thrombotic microangiopathy (TMA). She was diagnosed with systemic lupus erythematosus (SLE) and class V lupus nephritis. She had no aggravated SLE activity, Shiga toxin positivity, ADAMTS13 abnormality, or other causes of secondary TMA. Plasma exchange partially improved TMA, and eculizumab was introduced for suspected atypical hemolytic uremic syndrome (aHUS), as eculizumab was effective in suppressing the TMA activity. A kidney biopsy revealed diffusely organized crescents (pseudotubulization) with glomerular and arteriolar endothelial injury and subepithelial immune deposits. Thus, this was a rare case of lupus nephritis presenting as TMA with pseudotubulization possibly caused by aHUS.
一名31岁女性因血栓性微血管病（TMA）入住我院。她被诊断为系统性红斑狼疮（SLE）和Ⅴ型狼疮性肾炎。她没有SLE活动加重、志贺毒素阳性、ADAMTS13异常或其他继发性TMA的病因。血浆置换使TMA部分改善，鉴于依库珠单抗对抑制TMA活动有效，遂因疑似非典型溶血性尿毒症综合征（aHUS）而使用依库珠单抗。肾脏活检显示弥漫性组织化的新月体（假肾小管形成），伴有肾小球和小动脉内皮损伤以及上皮下免疫沉积物。因此，这是一例罕见的狼疮性肾炎表现为TMA并伴有可能由aHUS引起的假肾小管形成的病例。
Zotero 插件