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Abstract: TH-PO512

Hemophagocytic Lymphohistiocytosis (HLH) and Collapsing Focal Segmental Glomerulosclerosis (cFSGS) in an Immunocompetent Patient With Cytomegalovirus and Epstein-Barr Virus Viremia

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • He, Mingyue, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, United States
  • Noel, Edva, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, United States
  • Koul, Sheetal, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, United States
  • Gillespie, Avrum, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, United States
  • Constantinescu, Serban, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, United States
  • Lee, Iris J., Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, United States
Introduction

cFSGS is an aggressive variant of FSGS characterized by collapse and sclerosis of the glomerular tuft, nephrotic syndrome, and rapidly progressive renal failure often resistant to treatment. Patients of African ancestry are disproportionately affected. To our knowledge, this is the first reported case of cFSGS and HLH in an immunocompetent host with CMV and EBV viremia successfully treated with steroids and ganciclovir.

Case Description

A 56-year-old African American female was admitted with fever, dry cough, nausea/vomiting, and 30 lb weight loss over the past 6 months. Her persistent fevers led to an infectious workup and testing for HLH. Studies showed EBV viremia (9300 copies/ml), CMV viremia (15,567 IU/ml), hypertriglyceridemia, elevated soluble CD25, hyperferritinemia, and evidence of hemophagocytosis on bone marrow biopsy. 1 week later, she developed oliguric AKI with a peak creatinine of 8.3 mg/dl and 8.8 gm proteinuria. Serologies were all negative and complements were normal. A kidney biopsy showed cFSGS, acute tubulointerstitial nephritis. Immunofluorescence was negative. She was treated with dexamethasone for HLH and ganciclovir for CMV. 1.5 months later, her viremia resolved, abnormal markers improved, and her renal function normalized.

Discussion

The onset of HLH-related markers coinciding with CMV and EBV viremia suggests secondary HLH, likely triggered by viral infection. Rapid response to steroids and ganciclovir supports CMV infection as a driver of HLH and cFSGS development.
HIV-associated cFSGS is well established, however, association with CMV and EBV are rare. Increased interferon production in response to viral infection may provide the “second hit” for podocyte damage in AA patients with high-risk APOL1 alleles. HLH-related inflammation and cytokine dysregulation with markedly elevated Interferon levels may also contribute to cFSGS.
cFSGS is a rare complication of CMV, EBV infection and HLH. Testing for these is not routinely performed for immunocompetent patients, therefore these potential causes of cFSGS may be under-recognized. Cases of idiopathic cFSGS should be screened for viral infection and HLH, as anti-viral therapy combined with steroids may result in the resolution of renal disease.