Iatrogenic immunodeficiency-associated latent Epstein-Barr virus-related lymphomatoid granulomatosis initially presenting as unusual gynecological manifestations in a case of rheumatoid arthritis - a case report and review

摘要

It is known that lymphoproliferative disorders (LPD) could develop in patients with autoimmune diseases during immunosuppressive therapy, including methotrexate (MTX), known as iatrogenic immunodeficiency-associated LPD. Lymphomatoid granulomatosis (LYG) is an Epstein-Barr virus (EBV) driven LPD primarily involving the lungs, and occurs as very rare complications in rheumatoid arthritis (RA) patients during MTX treatment. A 46-year-old Japanese woman with RA and Sjogren’s syndrome who had been treated with MTX and prednisolone noticed metrorrhagia. A haemorrhagic ulcer and necrotic tissue were found in the cervix. Lung computerized tomography revealed multiple nodular lesions in both lung fields. Pathological examination revealed that angiodestructive lesions consist of atypical EBER-positive/CD20-positive cell infiltrations surrounded by T-cells. She was diagnosed with grade 3 LYG. The EBV latency profile was classified as type III. After MTX discontinuation, uterocervical ulcer lesions as well as multiple nodules in both lung fields rapidly regressed. This case is the first description of a RA patient with LYG during MTX treatment presenting uterocervical ulcer lesions mimicking uterocervical carcinoma.