Abstract: TH-PO205

Response to Intravenous Immunoglobulin in Acute Parvovirus B19-Associated Nephrotic Syndrome from Collapsing Focal Segmental Glomerulosclerosis

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Uppal, Nupur N., Hofstra Northwell School of Medicine, Great Neck, New York, United States
  • Parikh, Nishita, Hofstra Northwell School of Medicine, Great Neck, New York, United States
  • Shah, Hitesh H., Hofstra Northwell School of Medicine, Great Neck, New York, United States
Background

Human parvovirus B19 (HPV B19) has been associated with collapsing focal segmental glomerulosclerosis (c-FSGS). However, the optimal therapy for HPV B19-associated c-FSGS is currently unknown. While intravenous immunoglobulin (IVIG) therapy has been used for treatment of c-FSGS in immunocompromised patients, its role in immunocompetent patients remains unclear. We report the response to IVIG treatment in 2 immunocompetent patients with HPV B19-associated c-FSGS.

Methods

Case 1: 37-year-old African American (AA) female with sickle cell disease was hospitalized and treated for transient aplastic crisis secondary to acute HPV B19 infection. Five days later, patient presented with worsening lower extremity (LE) edema. She was found to have massive proteinuria (spot urine TP/CR: 56), hypoalbuminemia (serum albumin: 1.8 g/dL) and serum creatinine (Scr) of 1.8 mg/dL. HPV B19 DNA by PCR was markedly elevated (1.8 X 108 IU/ml). Other serological work up for nephrotic syndrome including HIV infection was negative. Kidney biopsy subsequently revealed c-FSGS. In addition to diuretics, patient received trial of 2 doses of IVIG therapy. Despite IVIG treatment, HPV B19 viral load remained elevated and our patient progressed to ESRD within 11 months of initial presentation.
Case 2: 18-year-old AA male presented for evaluation of AKI (Scr: 2.74 mg/dL) and acute onset nephrotic syndrome. Patient was found to have significant proteinuria (spot urine TP/CR: 14.9) and hypoalbuminemia (serum albumin: 1.9g/dL). HPV B19 DNA by PCR was elevated (95,600 IU/ml). Other serological work up for nephrotic syndrome including HIV infection was negative. Kidney biopsy showed c-FSGS. Patient subsequently received trial of 2 doses of IVIG treatment. HPV B19 viral load remained elevated despite IVIG treatment. Patient did not respond to IVIG treatment and continues to have significant nephrotic syndrome and progressive renal failure.

Conclusion

Optimal therapy for HPV B19-associated c-FSGS is currently unknown. Role of IVIG treatment in immunocompetent patients with acute HPV B19-associated c-FSGS remains unclear. Our patients continued to have elevated HPV B19 viral load and progressive renal failure despite IVIG treatment. Well-designed studies are needed to understand the mechanisms and treatment of this devastating medical condition.