TREATMENT-RESISTANT PLA(2)R-NEGATIVE MEMBRANOUS NEPHROPATHY RESPONSIVE TO LDL APHERESIS

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 (PLA(2)R) 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 (ESRD) and mortality. Hypercholesterolemia is not only a side effect of nephrotic syndrome, but 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 PLA(2)R negative membranous nephropathy who had significant improvement following two courses of LDL apheresis. To our knowledge, this is the first such reported case in the United States.