Nineteen Episodes of recurrent myelitis in a woman with neuromyelitis optica and systemic lupus erythematosus

Objectives: To describe the case of a patient with systemic lupus erythematosus (SLE) and neuromyelitis optica (NMO) who experienced 19 recurrent attacks of myelitis. Design: Case report. Setting: An outpatient neurorheumatology clinic at the Johns Hopkins Hospital devoted to care of patients with neurological manifestation of rheumatic diseases. Patient: A woman with NMO and SLE. Intervention: Rituximab therapy. Main Outcome Measures: Clinical and neuroimaging features of relapsing disease. Results: Recurrent and increasingly severe myelitis attacks still occurred after treatment with rituximab. Conclusions: It may be progressively more difficult to prevent relapses and commensurate disability in patients with later stages of relapsing NMO. Recognition of NMO as a distinct diagnostic entity in patients with SLE and other rheumatic diseases is crucial, in that institution of earlier targeted immunosuppressant treatment may be more effective than later targeted immunosuppression. The cellular arm of the immune system may be recruited by pathogenic B cells and may explain why relapses may occur after treatment with B cell - depleting therapy.