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

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 exc...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2018
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6028688/
https://www.ncbi.nlm.nih.gov/pubmed/29434134
http://dx.doi.org/10.2169/internalmedicine.0228-17
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author 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
author_facet 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
author_sort Ono, Masafumi
collection PubMed
description 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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spelling pubmed-60286882018-07-03 A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome 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 Intern Med Case Report 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. The Japanese Society of Internal Medicine 2018-02-09 2018-06-01 /pmc/articles/PMC6028688/ /pubmed/29434134 http://dx.doi.org/10.2169/internalmedicine.0228-17 Text en Copyright © 2018 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/ The Internal Medicine is an Open Access article distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
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
A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome
title A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome
title_full A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome
title_fullStr A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome
title_full_unstemmed A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome
title_short A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome
title_sort rare case of lupus nephritis presenting as thrombotic microangiopathy with diffuse pseudotubulization possibly caused by atypical hemolytic uremic syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6028688/
https://www.ncbi.nlm.nih.gov/pubmed/29434134
http://dx.doi.org/10.2169/internalmedicine.0228-17
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