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

Intern Med. 2018 Jun 1;57(11):1617-1623. doi: 10.2169/internalmedicine.0228-17. Epub 2018 Feb 9.

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.

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

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Atypical Hemolytic Uremic Syndrome / complications*
  • Atypical Hemolytic Uremic Syndrome / diagnosis
  • Atypical Hemolytic Uremic Syndrome / drug therapy
  • Female
  • Humans
  • Lupus Erythematosus, Systemic / complications
  • Lupus Erythematosus, Systemic / diagnosis
  • Lupus Nephritis / complications*
  • Lupus Nephritis / diagnosis
  • Plasma Exchange
  • Plasmapheresis
  • Thrombotic Microangiopathies / complications
  • Thrombotic Microangiopathies / diagnosis

Substances

  • Antibodies, Monoclonal, Humanized
  • eculizumab