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Abstract: FR-PO629

Bamboozled: Concomitant Lupus Nephritis and Systemic Thrombotic Microangiopathy

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation

Authors

  • Spolnik, Margaret, University of Virginia, Charlottesville, Virginia, United States
  • Cavanaugh, Corey J., University of Virginia, Charlottesville, Virginia, United States
  • Cathro, Helen P., University of Virginia, Charlottesville, Virginia, United States
Introduction

Lupus nephritis (LN) and thrombotic microangiopathy (TMA) are rare diseases rarely occurring together. Both can have devastating outcomes of kidney failure, neurological demise, and even death.

Case Description

A 31-year-old African-American woman with history of lupus and idiopathic thrombotic thrombocytopenic purpura (TTP) presented with malaise, swelling, emesis and diarrhea for 1 week. Initial testing- Cr 8.7 mg/dL (baseline 1.1), plts 30 k/uL, haptoglobin <8, LDH 938 U/L, Hgb 6.7 g/dL, uPr/Cr 2.89 g/g, low C3/C4, ANA >1:640, positive dsDNA, Smith, and SSA antibodies. Shiga toxin neg. Peripheral smear showed schistocytes. Urine microscopy had granular casts, but no RBC casts and no dysmorphic RBCs. Given concern for possible TTP, plasmapheresis(PLEX) occurred for 5 sesssions until ADAMTS-13 returned with 57% activity. She was also given eculizumab and solumedrol for concern of complement-mediated TMA. Kidney biopsy showed membranous pattern glomerulopathy with 64% cellular crescents and 63% globally sclerotic glomeruli, acute tubular damage, mild interstitial fibrosis and mild tubular atrophy. IF staining was positive for IgG 1+, IgM trace, kappa 2+, lambda 2+, but negative for C1q and IgA and without evidence of TMA. She was treated as proliferative LN given high clinical suspicion with lupus history and active cellular crescents. She started IV cyclophosphamide at 500 mg every 2 weeks for a total of 6 doses per Euro-lupus protocol with corticosteroids. Repeat labs with Cr 1.3, uPr/Cr decreased to 0.14 g/g, and C3/C4 normalized. Peripheral smear without schistocytes and improved LDH/haptoglobin levels. With a negative atypical HUS genetic panel, eculizumab was terminated after two months. She is currently on low-dose steroids and mycophenolate mofetil 1000mg BID.

Discussion

Two rare and severe diseases presented concomitantly in a patient with acute kidney injury. She was treated with multiple agents for immunosuppression including cyclophosphamide, steroids and eculizumab as well as five plasmapheresis sessions with no apparent adverse events. Definitive data for cessation of eculizumab is not available, but cessation has not resulted in a recurrent flare. Despite a biopsy without typical characteristic full-house staining for LN, the patient responded well to the Euro-lupus protocol and remains with stable kidney function and minimal proteinuria.