Devic's neuromyelitis optica: A prospective study of seven patients treated with prednisone and azathioprine

Abstract

Seven newly diagnosed patients with Devic's neuromyelitis optica were treated with long-term prednisone and azathioprine, and were followed every 2 months for at least 18 months. Their Expanded Disability Status Scale score improved significantly (mean at baseline, 9; mean at 18 months, 3; p < 0.005), and no relapses occurred for more than 18 months. Multicenter controlled studies are needed to prove the efficacy of this therapeutic regimen.

Neuromyelitis optica spectrum disorder in patients with connective tissue disease and myelitis

“…In those with relapsing disease, lifelong therapy should be considered (2). Prednisone, azathioprine, and mycophenolate mofetil have all been effective in disease stabilization and relapse reduction in uncontrolled trials of NMO (41)(42)(43). The use of rituximab has been successful in a case series of patients with resistant disease (44).…”

Present and Future Therapies in Neuromyelitis Optica Spectrum Disorders

“…Several studies including a total of almost 400 patients have shown that AZA reduces the ARR, sometimes associated with improvement of neurological disability [17,[74][75][76][77][78][79][80]. A retrospective study evaluating classical NMO and patients with AQP4-IgG-positive NMOSD reported a decrease in the mean ARR from 2.18 to 0.64 in 70 patients treated with AZA for >1 year, with or without concomitant corticosteroid therapy [77].…”

“…Adjunctive therapy of AZA [17,75,77,79], MMF [79,83], MTX [85][86][87], ciclosporine A [88], and tacrolimus [89] with oral corticosteroids have all been reported to be (partly) effective and safe in NMOSD. In breakthrough disease intermittent TPE might be added to immunosuppressive treatment [8,98], but usually escalation to a biological is preferable.…”

“…Azathioprine (AZA) was among the first immunosuppressants reported to be effective for NMOSD [17]. Subsequently, the new diseasemodifying drugs approved for MS were given for NMOSD, with variable success [18][19][20], while more promising results came from B-cell depletion therapy [21].…”

Efficacy of tacrolimus in Sjögren’s syndrome-associated CNS disease with aquaporin-4 autoantibodies

“…Combination therapy with a corticosteroid and azathioprine is the current standard treatment for preventing NMO relapse [5]; however, some patients are refractory to it.…”