Chronic Active Epstein-Barr Virus Infection in a Patient With Sjogren's Syndrome

Abstract

BACKGROUNDInfectious conditions in patients with autoimmune diseases are common, because both the pathological processes and the treatment can increase the risk of infections. However, despite being frequent, diagnosis in these cases can be challenging. CASE REPORTA 63-year-old white woman presents to the emergency department with cervical, axillary, and inguinal lymphadenopathy for 1 month, associated with daily fever, night sweats and weight loss. The patient had a previous diagnosis of rheumatoid arthritis and Sjögren's syndrome for 20 years, using methotrexate 20 mg/week and folic acid 5 mg/week since the diagnosis, without follow-up with a rheumatologist. The initial laboratory results were significant for hemoglobin levels of 9.4 (12.5-15.5), with leukocytes of 16,770 (3,500-10,500), with increased neutrophils and lymphocytes and lymphocytic atypia. The results also showed a non-PTH-mediated hypercalcemia, creatinine of 1.63 (0.5-0.9), hyperkalemia, hyperphosphatemia and increased levels of uric acid. Immunoglobulin levels were reduced and serologies for cytomegalovirus and Epstein-Barr virus (EBV) were negative. Protein electrophoresis had no monoclonal peak, tuberculin skin test was negative, serologies for HIV, syphilis and hepatitis C were negatives and serology for hepatitis B showed anti-HBc IgG and anti-HBs positives. Computed tomography (CT) scan revealed cervical, thoracic, and abdominal lymphadenopathy, hepatosplenomegaly and pulmonary nodules and opacities, which were suggestive of interstitial lymphocytic granulomatosis. Lymph node biopsy was performed, and dexamethasone 20 mg/day for 4 days and supportive treatment for tumor lysis syndrome were started. Biopsy showed atypical lymphoid proliferation, with focal expression of CD30 and EBV, without criteria for hematologic malignancy. Prednisone was started with clinical improvement and lymph node involution. Months after discontinuing the corticosteroid, the patient started new episodes of fever associated with dyspnea. CT scan showed an increase in pulmonary nodules, the appearance of new nodules and areas of consolidation. Laboratory tests showed EBV DNA quantification of 110,855 IU/mL (5.04 log IU/mL). Antibiotics and ganciclovir were started, but the patient did not respond and eventually died 18 days after admission. CONCLUSIONChronic active EBV infection is a rare, but serious condition. The only curative treatment regimen is hematopoietic stem cell transplantation, but other treatments like high dose corticosteroids or antiviral therapy have been used. Patients with rheumatic diseases who developed a persistent mononucleosis-like syndrome and hypogammaglobulinemia must be promptly evaluated, since delay in diagnosis can be life-threatening.