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Abstract: PO1436

Treatment of Crescentic Lupus Nephritis with Voclosporin

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Hussain, Irshad, State University of New York Upstate Medical University Division of Nephrology, Syracuse, New York, United States
  • Bukhari, Syed HR, State University of New York Upstate Medical University Division of Nephrology, Syracuse, New York, United States
  • Shanley, Paul F., State University of New York Upstate Medical University Department of Pathology, Syracuse, New York, United States
  • Bhargava, Ramya, State University of New York Upstate Medical University Division of Nephrology, Syracuse, New York, United States
Introduction

Crescentic glomerulonephritis (CGN) is a rare complication of Lupus nephritis (LN) and carries a worse prognosis. There is paucity of data regarding effective treatment options for CGN. We present a case of crescentic ANCA negative LN treated with voclosporin (VSN).

Case Description

19-year-old African American female with 2-year history of Class II LN, treated with hydroxychloroquine 200 mg/day, prednisone 10 mg/day, mycophenolate mofetil 1 gm twice daily and Belimumab, presented with a 2-week history of anasarca and generalized bullous skin rash. On exam BP 126/78 mm Hg, HR 96/min, afebrile, RR 18/min, O2 saturation 97% on room air. Investigations revealed hemoglobin 9.2 g/dL, serum creatinine (SCR) 1.2 mg/dl (baseline 0.6), albumin 1.9 g/dL, hypocomplementemia, microscopic hematuria and proteinuria of 4.3 gm. A kidney biopsy showed diffuse crescentic immune complex LN and membranous LN (Figure 1). The patient received IV methylprednisolone 1gm for 3 days, however, became anuric, SCR peaked at 5 mg/dl and was commenced on hemodialysis and 7 sessions of plasma exchange. She was started on VSN 15.8 mg BID and after 10 days of therapy, SCR improved and dialysis was discontinued. On discharge, SCR was 2.0 mg/dl and proteinuria 0.9 gm. C4 normalized and C3 improved.

Discussion

There has not been any published case report of Crescentic LN being treated successfully with VSN. Given poor prognosis of CGN, early diagnosis and treatment is imperative. Our patient had rapid recovery of renal function and resolution of proteinuria following treatment with VSN. VSN may be effective in combination with plasma exchange in ANCA negative Crescentic LN. Larger studies with longer follow-up are needed to assess the efficacy of VSN in CGN.

A. H&E stain shows diffuse segmental endocapillary hypercellularity and glomeruli with cellular crescents (60%), segmental necrosis and subendothelial deposits. B and C. PAS and Silver stains show no glomerular basement membrane remodeling. D. Trichome stain with moderate interstitial fibrosis and tubular atrophy (35%).