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Abstract: PO1910

Glomerular Tip Lesion FSGS: A Rare Case of Nephrotic Syndrome in African Americans

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Moses, Andrew A., Columbia University Irving Medical Center, New York, New York, United States
  • Husain, Syed Ali, Columbia University Irving Medical Center, New York, New York, United States
  • Ahn, Wooin, Columbia University Irving Medical Center, New York, New York, United States
Introduction

Nephrotic syndrome caused by focal segmental glomerular sclerosis (FSGS) in African Americans is known to have a poor prognosis and frequent treatment failure. Tip variant FSGS connotes good prognosis, usually responsive to calcineurin inhibitor (CNI) and steroid. We present a case of CNI resistant tip lesion FSGS requiring plasma exchange (PLEX) and rituximab

Case Description

A 25-year-old African-American male, initially presenting to outpatient clinic for edema associated with proteinuria. He had a past medical history of seizure disorder controlled with zonisamide. He trialed hydrochlorothiazide and furosemide without success. His urine showed nephrotic range proteinuria with 6g/g creatinine on spot urine. Outpatient workup was unrevealing, and was managed with enalapril and torsemide. He eventually had worsening edema and shortness of breath, prompting ED visit. Biopsy was performed which showed FSGS, tip variant. He was discharged on cyclosporine and increased diuretic. Cyclosporine caused gastrointestinal upset, and so patient switched to tacrolimus. He again had increasing swelling, and re-presented to hospital. He was found to have acute kidney injury, and 10g/g creatinine despite therapeutic tacrolimus levels. Patient trialed stress dose steroids but serum creatinine rose to 4.44. He started PLEX for 10 treatments, and then transitioned to rituximab. During treatment with PLEX patient creatinine quickly downtrended, and after second dose of rituximab as an outpatient he was back to baseline. He lost 30 kg with resolution of his edema. Patient then tapered off steroids, and will continue on rituximab alone.

Discussion

Glomerular tip lesion is more common in European Americans and associated with a favorable outcome compared to other subtypes of FSGS. Resistance to one immunosuppressive treatment is not always associated with resistance to other treatment modalities. PLEX and rituximab should be considered in glomerular tip lesion in African Americans