Treatment-resistant PLA2R-negative membranous nephropathy responsive to low-density lipoprotein apheresis

J Clin Apher. 2019 Aug;34(4):495-498. doi: 10.1002/jca.21677. Epub 2018 Dec 10.

Abstract

Idiopathic membranous nephropathy is the most common cause of nephrotic syndrome in nondiabetic adults. The antibody most often implicated is the M-type phospholipase A2 receptor (PLA2R) antibody, found in >70% of primary membranous nephropathy cases. First-line therapy is immunosuppressive in nature, but for patients who are treatment-resistant there is a significant risk of end-stage renal disease and mortality. Hypercholesterolemia is not only a side effect of nephrotic syndrome, but also its presence may worsen renal function. A recent single-arm observational study in Japan found that low-density lipoprotein apheresis (LDL-A) was able to ameliorate nephrotic syndrome in half of patients who were resistant to medication. We present a case of treatment resistant PLA2R negative membranous nephropathy who had significant improvement following two courses of LDL-A. To our knowledge, this is the first such reported case in the United States.

Keywords: lipoprotein apheresis; membranous nephropathy; phospholipase A2.

Publication types

  • Case Reports

MeSH terms

  • Blood Component Removal*
  • Glomerulonephritis, Membranous / therapy*
  • Humans
  • Lipoproteins, LDL / isolation & purification*
  • Nephrotic Syndrome / prevention & control
  • Receptors, Phospholipase A2 / deficiency
  • Receptors, Phospholipase A2 / immunology
  • Salvage Therapy / methods
  • Treatment Outcome
  • United States

Substances

  • Lipoproteins, LDL
  • Receptors, Phospholipase A2