Abstract
A case of malignant rheumatoid arthritis (MRA) associated with fulminating sensorimotor neuropathy was reported. A 53-year-old female had been suffered from RA since 1969 and received long-term corticosteroid treatment. The motor and sensory nerve disturbance of upper and lower extremities was suddenly appeared on February 1, 1988, and she was transferred to Senami Hospital. Since she had severe sensorimotor neuropathy of forearms and legs, leukocytosis, thrombocytosis and increase of rheumatoid factor and acute phase reactants, the diagnosis of MRA was made. Because gangrene of both fingers and leg ulcers developed rapidly within a few days, she was treated with high dose corticosteroid combined with immunosuppressants and plasmapheresis. Despite of improvement of laboratory examinations the ulcers of both legs had been infected frequently and become worse, followed by bilateral below knee amputation. Vasa nervosum of the sural nerve and small arteries around the ulcers showed vasculitis.
This case was classified as Bywaters' type of MRA because of little internal organ damages. In addition to the conventional steroid and immunosuppressant treatment, more extensive therapy including intravenous cyclophosphamide therapy and long-term plasmapheresis should be considered in MRA with fulminating sensorimotor neuropathy.