Lymphomatoid granulomatosis in a patient with rheumatoid arthritis receiving methotrexate: successful treatment with the anti-CD20 antibody mabthera.

摘要

The risk for malignant lymphoproliferative diseases (LPD), particularly aggressive lymphoma, is increased in patients with rheumatoid arthritis (RA). Chronic B cell activation due to chronic inflammation, long-term use of immunosuppressive drugs such as methotrexate (MTX) as well as Epstein-Barr virus (EBV) infection have been proposed as the etiologic cause [1, 2]. Lymphomatoid granulomatosis (LYG) is a rare, angio-centric and angiodestmctive, EBV-driven LPD involving ex-tranodal sites. Patients with underlying immunodeficiency are at increased risk. Predisposing conditions include allogenic organ transplantation, Wiskott-Aldrich syndrome, human immunodeficiency virus infection, and X-linked lymphoproliferative syndrome. The clinical course is extremely variable, ranging from an indolent form to a disease that is similar to aggressive lymphoma.