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

Masafumi Ono; Naro Ohashi; Akio Namikawa; Naoko Katahashi; Sayaka Ishigaki; Naoko Tsuji; Shinsuke Isobe; Takamasa Iwakura; Yukitoshi Sakao; Takayuki Tsuji; Akihiko Kato; Yoshihide Fujigaki; Akira Shimizu; Hideo Yasuda

doi:10.2169/internalmedicine.0228-17

CASE REPORTS

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

Masafumi Ono, Naro Ohashi, Akio Namikawa, Naoko Katahashi, Sayaka Ishigaki, Naoko Tsuji, Shinsuke Isobe, Takamasa Iwakura, Yukitoshi Sakao, Takayuki Tsuji, Akihiko Kato, Yoshihide Fujigaki, Akira Shimizu, Hideo Yasuda

Author information

Masafumi Ono
Blood Purification Unit, Hamamatsu University School of Medicine, Japan
Naro Ohashi
Division of Nephrology, First Department of Medicine, Hamamatsu University School of Medicine, Japan
Akio Namikawa
Division of Nephrology, First Department of Medicine, Hamamatsu University School of Medicine, Japan
Naoko Katahashi
Division of Nephrology, First Department of Medicine, Hamamatsu University School of Medicine, Japan
Sayaka Ishigaki
Division of Nephrology, First Department of Medicine, Hamamatsu University School of Medicine, Japan
Naoko Tsuji
Division of Nephrology, First Department of Medicine, Hamamatsu University School of Medicine, Japan
Shinsuke Isobe
Division of Nephrology, First Department of Medicine, Hamamatsu University School of Medicine, Japan
Takamasa Iwakura
Division of Nephrology, First Department of Medicine, Hamamatsu University School of Medicine, Japan
Yukitoshi Sakao
Hamana Clinic, Japan
Takayuki Tsuji
Division of Nephrology, First Department of Medicine, Hamamatsu University School of Medicine, Japan
Akihiko Kato
Blood Purification Unit, Hamamatsu University School of Medicine, Japan
Yoshihide Fujigaki
Department of Internal Medicine, Teikyo University School of Medicine, Japan
Akira Shimizu
Department of Pathology, Nihon University School of Medicine, Japan
Hideo Yasuda
Division of Nephrology, First Department of Medicine, Hamamatsu University School of Medicine, Japan

Keywords: thrombotic microangiopathy, systemic lupus erythematosus, atypical hemolytic uremic syndrome, eculizumab, pseudotubulization

JOURNAL OPEN ACCESS

2018 Volume 57 Issue 11 Pages 1617-1623

DOI https://doi.org/10.2169/internalmedicine.0228-17

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Abstract

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.

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