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Kidney Week

Abstract: PO2319

Lupus Vasculopathy Successfully Treated with Eculizumab and Rituximab in an 8-Year-Old Male

Session Information

Category: Trainee Case Report

  • 1700 Pediatric Nephrology

Authors

  • Black, Elizabeth Ashley, University of California San Francisco, San Francisco, California, United States
  • Winnicki, Erica, University of California San Francisco, San Francisco, California, United States
Introduction

Lupus vasculopathy of the kidney is a rare form of vascular disease in patients with systemic lupus erythematosus characterized by non-inflammatory, necrotizing vessel wall changes. The pathogenesis may be related to immune-mediated vascular injury from accumulation of immunoglobulins and complement in the vascular wall. Lupus vasculopathy carries a poor renal prognosis, and no standardized treatment has been established.

Case Description

An 8-year-old previously healthy male presented to the emergency department with fever, fatigue, headache, diarrhea, nosebleeds, decreased urine output and lower extremity swelling. He was found to have hypertension, fluid overload, active urine sediment and severe acute kidney injury necessitating renal replacement therapy. He had thrombocytopenia and hemolytic anemia without presence of antiphospholipid antibodies or abnormal ADAMTS13 activity. He met criteria for systemic lupus erythematosus including hypocomplementemia, positive ANA and dsDNA antibodies. Renal biopsy showed class III lupus nephritis and multifocal arterial and arteriolar large intimal immune complex deposits with endothelial cell necrosis, consistent with a diagnosis of lupus vasculopathy. He received 6 sessions of plasmapheresis without improvement. Based on renal biopsy, he was treated with cyclophosphamide per Euro Lupus protocol. Eculizumab was started for treatment of lupus vasculopathy. His anemia, thrombocytopenia, and proteinuria improved after initiation of eculizumab, but he remained dialysis dependent. Eculizumab was discontinued after 6 weeks of therapy, but he again developed thrombocytopenia, hemolytic anemia, worsening proteinuria and hypertension. These improved after restarting eculizumab. Due to ongoing evidence of lupus activity, rituximab was given after completion of cyclophosphamide. Hemodialysis was discontinued one month after his first rituximab dose with B-cell depletion. He remains on eculizumab therapy and has stable chronic kidney disease stage 2 despite B-cell repopulation.

Discussion

Prior case reports have documented effective treatment of lupus vasculopathy with rituximab. To our knowledge, no data exists on eculizumab for treatment of lupus vasculopathy. Given our patient’s clinical improvement with these therapies, we conclude that more research is needed to define their role in treatment of patients with lupus vasculopathy.