ABSTRACT Patients with systemic lupus erythematosus (SLE) are predisposed to Epstein–Barr virus (EBV) reactivation and the subsequent development of lymphoproliferative disorders (LPDs) due to immune dysregulation and prolonged immunosuppressive therapy. We report a case of a 42-year-old woman with a 26-year history of SLE who had been clinically stable for several years on low-dose prednisolone, azathioprine, cyclosporine, and hydroxychloroquine. She presented for evaluation of persistent fevers and abdominal pain. Imaging studies revealed segmental small bowel thickening with mesenteric hyperemia, suggestive of lupus enteritis. Laboratory findings included hypoalbuminemia and elevated inflammatory markers, but no serologic evidence of active lupus. Histologic examination of small intestinal biopsies revealed a polymorphic lymphoid infiltrate with atypical B cells positive for EBV, leading to a diagnosis of EBV-associated LPD. Following a reduction in immunosuppressive therapy and initiation of rituximab, the patient achieved clinical and radiologic improvement. This case highlights the importance of considering EBV-LPD in the differential diagnosis of gastrointestinal manifestations in SLE patients, particularly when serologic markers of active lupus are absent. The overlapping clinical and imaging features of lupus enteritis and EBV-LPD can lead to diagnostic delays, making histopathologic assessment crucial for accurate identification. The case also underscores the potential risk of EBV-LPD associated with long-term immunosuppressive treatment in SLE. Careful monitoring for lymphoproliferative complications is warranted in patients undergoing sustained immunosuppression.
Oikawa et al. (Thu,) studied this question.