A Rare Complication of Prolonged Immunosuppression in Lupus Nephritis
- Glomerular Diseases: Epidemiology and Case Reports
November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Trials
- Broka, Andrea, UC Davis Health, Sacramento, California, United States
- Terry, Merryl, UCSF Medical Center, San Francisco, California, United States
- Wiegley, Nasim, UC Davis Health, Sacramento, California, United States
Epstein-Barr virus (EBV) is a well-recognized virus associated with lymphoproliferative disease (LD). Immunodeficiency and immunosuppression can lead to the reactivation and replication of the virus, increasing the likelihood of malignancy. We present a case of a patient with Lupus nephritis (LN) patient on long-term immunosuppression presenting with non-transplant-associated central nervous system (CNS) LD.
A 35-year-old woman with frequently relapsing LN Class IV on mycophenolate mofetil (MMF), Plaquenil and prednisone for 15 years presented with numbness and weakness in the left side of her face and arm. MRI head showed bilateral supratentorial enhancing lesions (Fig 1). Positive dsDNA Ab, reduced C3, normal C4, EBV positive in cerebrospinal fluid, blood viral load <750 units/ml. CNS lesion biopsy confirmed iatrogenic EBV-associated LD (Fig 2). MMF was discontinued. Rituximab was started for the management of LD with resolution of the symptomatology. To date, lupus nephritis has remained quiescent on Rituximab.
EBV-associated LD is influenced by the level of immune suppression and reduced surveillance by T cells. Certain medications can impede the proliferation of lymphoblastoid cell lines, and others affect T-cell function. In vitro and animal model studies have shown that prolonged use of MMF was associated with diminished recovery of Vδ2+ T cells and increased occurrence of EBV reactivation.
MMF, commonly used in autoimmune diseases, may unmask an inherent susceptibility of the CNS for immunosuppression-related LD. While LD is frequently considered after organ transplantation, it is less often considered in non-transplant immunosuppressed patients. A high index of suspicion is needed when caring for non-transplant immunosuppressed patients presenting with primary CNS symptoms. Prompt evaluation is necessary to distinguish between lupus cerebritis and LD to individualize care and improve patient outcomes.