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Abstract: PO2314

Rescue Therapy with Eculizumab for Catastrophic Antiphospholipid Syndrome in Juvenile Systemic Lupus Erythematosus

Session Information

Category: Trainee Case Report

  • 1700 Pediatric Nephrology

Authors

  • Arroyo Parejo Drayer, Patricia Alejandra, University of Miami School of Medicine, Miami, Florida, United States
  • Al Barbandi, Malek, University of Miami School of Medicine, Miami, Florida, United States
  • Defreitas, Marissa J., University of Miami School of Medicine, Miami, Florida, United States
  • Katsoufis, Chryso P., University of Miami School of Medicine, Miami, Florida, United States
  • Chandar, Jayanthi, University of Miami School of Medicine, Miami, Florida, United States
  • Abitbol, Carolyn L., University of Miami School of Medicine, Miami, Florida, United States
  • Seeherunvong, Wacharee, University of Miami School of Medicine, Miami, Florida, United States
Introduction

Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition, and is associated with acute multiorgan failure, small vessels thromboembolism, and elevated markers of antiphospholipid syndrome (APL). Early recognition and promptly treatment can improve the outcome. We present a case of juvenile systemic lupus erythematosus (SLE), who presented with CAPS, refractory to conventional treatment, but rescue with Eculizumab

Case Description

The patient is a 10yo female, previously diagnosis with systemic lupus erythematosus (SLE) without renal involvement and treated with hydroxychloroquine and low dose steroid, who presented with acute kidney injury and hypertensive crisis. On exam, her weight was 31.3 Kg, height 133 cm. Her BP was 185/127 mmHg and pulse 118/min. Initial investigation showed hemoglobin of 7.2 g/dL, platelet count of 182/uL, BUN 99 mg/dL, Cr 7.86 mg/dL, haptoglobin < 10 mg/dL. Urine protein to creatinine ratio was 5 mg/mg. Serum complements were low and had positive serology for Anti-DSDNA, Lupus anticoagulant, Anti-beta2 glycoprotein, and anticardiolipin. The presumptive diagnosis was CAPS associated with SLE and thrombotic microangiopathy (TMA) which was confirmed by kindney biopsy. She underwent Methylprednisone, therapeutic plasma exchange (TPE), renal replacement therapy amd Nicardipine infusion. After 7 sessions of TPE, Methylprednisone, Rituximab, and Mycophenolate Mofetil, her renal function improved and was taken off hemodialysis. Blood pressure was still uncontrolled. She had evidence of on-going hemolysis with undetectable haptoglobin and elevated LDH. Repeat TPE did not control her BP or hemolysis, thus Eculizumab was administered as a rescue therapy for TMA associated with CAPS. After 2 weekly doses Eculizumab, her renal function, blood pressure and hemolytic markers were much improved. Currently, her serum creatinine was 1 mg/dL, without significant proteinuria. She remained on 3 antihypertensive medications with good BP control

Discussion

This is a rare but challenging case of juvenile SLE, complicated with CAPS and TMA, who responded partially to conventional treatment. Eculizumab served as a rescue therapy with good result. Our case supports the use of Eculizumab for refractory CAPS in SLE.