A case of lupus vasculopathy presenting favorable renal outcome

CEN Case Rep. 2020 Feb;9(1):74-80. doi: 10.1007/s13730-019-00431-2. Epub 2019 Dec 20.

Abstract

Noninflammatory necrotizing vasculopathy, also referred to as lupus vasculopathy, is not infrequently observed in the pathology of lupus nephritis. It affects vessels causing them to become severely narrowed and occluded by a mechanism involving immune complexes. We experienced a 51-year-old woman with lupus nephritis class IV + V, which was accompanied by lupus vasculopathy. Renal biopsy and light microscopy showed eosinophilic hyaline-like material in the afferent and/or efferent arterioles, which narrowed the lumen, and which were positive for IgG by immunofluorescent analysis. Electron microscopy indicated that amorphous material and endothelial detachment occluded the arterioles. These findings were consistent with those of lupus vasculopathy. We treated the patient with steroids and cyclophosphamide. By the day of discharge, her levels of creatinine and proteinuria had undergone partial remission. Although lupus vasculopathy was implied as a lesion with unfavorable renal prognosis, some recent reports suggest its true renal prognosis is not unfavorable necessarily. Nevertheless, lupus vasculopathy is an important finding in diagnosis in contradiction to other vascular legions in systemic lupus erythematosus. In addition, a standard therapy has also not been established. Therefore, it is important to accumulate cases of lupus vasculopathy to determine its prognosis and develop standard treatments.

Keywords: Lupus nephritis; Lupus vasculopathy; Noninflammatory necrotizing vasculopathy.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Hormones / administration & dosage
  • Adrenal Cortex Hormones / therapeutic use
  • Arterioles / injuries*
  • Arterioles / pathology
  • Biopsy
  • Creatinine / blood
  • Cyclophosphamide / administration & dosage
  • Cyclophosphamide / therapeutic use
  • Drug Therapy, Combination
  • Fluorescent Antibody Technique / methods
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / therapeutic use
  • Kidney / blood supply*
  • Kidney / pathology
  • Lupus Nephritis / complications*
  • Lupus Nephritis / diagnosis
  • Lupus Nephritis / immunology
  • Lupus Nephritis / pathology
  • Microscopy, Electron / methods
  • Middle Aged
  • Prognosis
  • Proteinuria / urine
  • Vascular Diseases / drug therapy
  • Vascular Diseases / etiology*
  • Vascular Diseases / pathology

Substances

  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Creatinine