Familial occurrence of two patients with malignant rheumatoid arthritis

In Japan, patients with rheumatoid arthritis associated with severe extra-articular manifestations due to vasculitis are diagnosed as having malignant rheumatoid arthritis. We report the occurrence of two cases of malignant rheumatoid arthritis in a Japanese family. Both patients, a father and son, expressed HLA-DR4 (Dw15), and were infected with Epstein-Barr virus. Moreover, the father developed malignant rheumatoid arthritis during reactivation of the Epstein-Barr virus. An unaffected male family member with the same HLA haplotypes was not infected by the virus. The possible role of the virus infection in the pathogenesis of malignant rheumatoid arthritis in a genetically susceptible family is discussed.