A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome

被引：9

作者：

Ono, Masafumi ^{[1
]}

Ohashi, Naro ^{[2
]}

Namikawa, Akio ^{[2
]}

Katahashi, Naoko ^{[2
]}

Ishigaki, Sayaka ^{[2
]}

Tsuji, Naoko ^{[2
]}

Isobe, Shinsuke ^{[2
]}

Iwakura, Takamasa ^{[2
]}

Sakao, Yukitoshi ^{[3
]}

Tsuji, Takayuki ^{[2
]}

Kato, Akihiko ^{[1
]}

Fujigaki, Yoshihide ^{[4
]}

Shimizu, Akira ^{[5
]}

Yasuda, Hideo ^{[2
]}

机构：

[1] Hamamatsu Univ Sch Med, Blood Purificat Unit, Hamamatsu, Shizuoka, Japan

[2] Hamamatsu Univ Sch Med, Dept Med 1, Div Nephrol, Hamamatsu, Shizuoka, Japan

[3] Hamana Clin, Hamamatsu, Shizuoka, Japan

[4] Teikyo Univ, Sch Med, Dept Internal Med, Tokyo, Japan

[5] Nihon Univ, Sch Med, Dept Pathol, Tokyo, Japan

来源：

INTERNAL MEDICINE | 2018年 / 57卷 / 11期

关键词：

thrombotic microangiopathy; systemic lupus erythematosus; atypical hemolytic uremic syndrome; eculizumab; pseudotubulization; COMPLEMENT INHIBITOR ECULIZUMAB; CRESCENTIC GLOMERULONEPHRITIS; EFFICACY;

D O I：

10.2169/internalmedicine.0228-17

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：1617 / 1623

页数：7

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