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Abstract: PO1413

Focal Segmental Glomerulosclerosis: A Rare Cause of Nephrotic Syndrome in Graft vs. Host Disease

Session Information

Category: Glomerular Diseases

  • 1201 Glomerular Diseases: Fibrosis and Extracellular Matrix

Authors

  • Da Silva Lugo, Ian J., Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
  • Santiago-Gonzalez, Juan Carlos, Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
  • Ocasio Feliciano, Edilberto Jose, Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
  • Pico-Ramirez, Alexandra C., Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
  • Rodriguez, Yamiris, Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
  • Andujar, Krystahl Z., Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
  • Garcia, Deniz E., Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
  • Ocasio Melendez, Ileana E., Universidad de Puerto Rico Escuela de Medicina, San Juan, Puerto Rico
Introduction

Nephrotic syndrome (NS) is a very rare complication of allogeneic hematopoietic cell transplantation (HCT) and is usually associated with chronic graft versus host disease (GVHD). In such patients, membranous nephropathy and minimal change disease are the most frequently observed renal pathology. However, focal segmental glomerulosclerosis (FSGS) is an extremely uncommon etiology of NS in patients with HCT and GVHD. We herein describe a case of a patient with HCT and chronic GVHD who developed NS secondary to FSGS.

Case Description

A 24-year-old man with medical history of Sickle Cell Disease status post splenectomy and HCT, epilepsy, arterial hypertension and chronic GVHD with cutaneous and esophageal manifestations who presented to the emergency department with a one month of evolution progressive lower extremity edema and intermittent hematuria. Vital signs were remarkable for uncontrolled blood pressure. Home medications were lisinopril 20mg daily, levetiracetam 500mg twice daily and prednisone 10mg daily. Physical examination was remarkable for edema of the lower extremities, ascites, and cachexia. Laboratories revealed: BUN 35 mg/dL, serum creatinine 1.15 mg/dL, total protein 3.00 g/dL, serum albumin 1 g/dL, glucose 110mg/dL, total cholesterol 425 mg/dL, triglycerides 335 mg/dL, VLDL 67 mg/dL and LDL 316 mg/dL. Urine protein/creatinine ratio resulted in 30,000mg/g. Laboratory findings of hypoalbuminemia, hyperlipidemia, and nephrotic range proteinuria were consistent with NS. A renal biopsy was performed and revealed findings consistent with a diagnosis of FSGS, not otherwise specified. Partial remission of NS was achieved at 3 months of treatment with mycophenolate mofetil 500mg twice daily and prednisone 15mg twice daily.

Discussion

GVHD is a significant cause of morbidity and mortality in patients after HCT. Renal involvement can be a serious manifestation and prompt recognition is essential for adequate management and prevention of renal disease. FSGS is an extremely rare complication of HCT and very few cases have been reported in the literature linking FSGS to HCT. Further documentation of this phenomenon is important to further characterize the clinical and pathological features of this complication.