1. A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome
- Author
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Yukitoshi Sakao, Hideo Yasuda, Sayaka Ishigaki, Akira Shimizu, Naro Ohashi, Naoko Tsuji, Shinsuke Isobe, Yoshihide Fujigaki, Naoko Katahashi, Takayuki Tsuji, Takamasa Iwakura, Akio Namikawa, Masafumi Ono, and Akihiko Kato
- Subjects
Adult ,Pathology ,medicine.medical_specialty ,Thrombotic microangiopathy ,medicine.medical_treatment ,Lupus nephritis ,Case Report ,030204 cardiovascular system & hematology ,Antibodies, Monoclonal, Humanized ,urologic and male genital diseases ,03 medical and health sciences ,0302 clinical medicine ,systemic lupus erythematosus ,immune system diseases ,hemic and lymphatic diseases ,Atypical hemolytic uremic syndrome ,Biopsy ,Internal Medicine ,medicine ,Humans ,Lupus Erythematosus, Systemic ,030212 general & internal medicine ,skin and connective tissue diseases ,Kidney ,Plasma Exchange ,medicine.diagnostic_test ,Thrombotic Microangiopathies ,atypical hemolytic uremic syndrome ,business.industry ,pseudotubulization ,Plasmapheresis ,General Medicine ,Eculizumab ,medicine.disease ,Lupus Nephritis ,female genital diseases and pregnancy complications ,ADAMTS13 ,thrombotic microangiopathy ,medicine.anatomical_structure ,Female ,eculizumab ,business ,medicine.drug - 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.
- Published
- 2018