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Abstract: PO1979

Cytomegalovirus Viremia-Associated Collapsing FSGS in an Immunosuppressed Systemic Lupus Erythematosus Patient

Session Information

  • Podocyte Biology
    October 22, 2020 | Location: On-Demand
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Report

  • 1204 Podocyte Biology

Authors

  • Daniel, Emily, Columbia University Irving Medical Center, New York, New York, United States
  • Santoriello, Dominick, Columbia University Irving Medical Center, New York, New York, United States
  • Bomback, Andrew S., Columbia University Irving Medical Center, New York, New York, United States
  • Fernandez, Hilda E., Columbia University Irving Medical Center, New York, New York, United States
Introduction

Maintaining a broad differential in evaluating AKI in SLE patients with a history of lupus nephritis (LN) is important. We describe a patient with SLE and AKI progressing to dialysis dependence due to collapsing FSGS in the setting of CMV infection.

Case Description

A 23 yo African American F with a history of LN class IV + V (maintained on MMF 1g BID) and APLS presented with one week history of nausea, vomiting and diarrhea. Admission labs were notable for a Cr of 10.5 mg/dl (baseline Cr 0.7), pancytopenia, with albumin of 1.7 mg/dl. Spot urine protein to creatinine ratio was >27 g/g. Serologies showed low C3 and C4, positive ANA, LDH 1437 and negative HIV test. Patient became anuric and required hemodialysis. Stress dose steroids were administered for presumed RPGN from LN. Renal biopsy on hospital day 8 demonstrated collapsing FSGS with 100% podocyte foot process effacement without significant IFTA and 4/9 globally sclerotic glomeruli. Testing revealed CMV viremia with viral load of >700,000 IU/mL. MMF was held. Ganciclovir was initiated with a subsequent decrease in viral load and sufficient renal recovery to stop hemodialysis. Patient was discharged off MMF on oral valganciclovir with a steroid taper. Three months after discharge, patient's viral load was undetectable and renal function had returned to baseline.

Discussion

Our patient with SLE had an atypical presentation. The appearance of significant proteinuria and severe AKI led to initial empiric treatment for LN. Rarely, collapsing FSGS has been described as the primary lesion in lupus podocytopathies. The high CMV viral load and robust response to antiviral therapy argue that the lesion was mediated by CMV infection rather than SLE.

The development of collapsing FSGS is commonly due to a "second hit" on a high risk APOL1 genetic background, in our case from elevated interferon levels in the setting of CMV viremia. APOL1 has been implicated in collapsing FSGS due to CMV viremia in African American DDRT recipients, demonstrating the role of genetic testing in African American patients.

Our case demonstrates an infectious trigger, rather than autoimmune cause of renal failure in an immunosuppressed patient with SLE.