Treatment of Neuromyelitis Optica With Mycophenolate Mofetil

Abstract

Background: Neuromyelitis optica (NMO) is the first inflammatory autoimmune demyelinating disease of the central nervous system for which a specific antigenic target has been identified; the marker autoantibody NMO-IgG specifically recognizes the astrocytic water channel aquaporin 4. Current evidence strongly suggests that NMO-IgG may be pathogenic. Since disability accrues incrementally related to attacks, attack prevention with immunosuppressive therapy is the mainstay of preventing disability. Objective: To evaluate the efficacy and safety of mycophenolate mofetil therapy in NMO spectrum disorders. Design: Retrospective case series with prospective telephone follow-up.

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

“…MMF treatment for a median of 20-27 months resulted in a reduction of the ARR by 80-93 % and improvement of the median EDSS in 3/ 4 studies (not reported in 1). More than 90 % of patients had stable or improved disability [83,84], and 46-65 % were relapse-free [79,81,83,84]. Discontinuation rates due to disease activity or side effects were 15-25 %.…”

“…It inhibits lymphocyte proliferation by suppression of guanosine nucleotide biosynthesis and is used for psoriasis, proliferative lupus nephritis, and renal transplant rejection. Four retrospective cohorts evaluating 24-58 patients with mostly AQP4-IgG-positive NMOSD have reported beneficial outcomes of MMF therapy with or without concomitant corticosteroids [79,81,83,84]. MMF treatment for a median of 20-27 months resulted in a reduction of the ARR by 80-93 % and improvement of the median EDSS in 3/ 4 studies (not reported in 1).…”

“…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.…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…The utility of IVIG in the treatment of acute NMO has not been adequately studied. A variety of immunosuppressive drugs, including rituximab, mitoxantrone, mycophenolate mofetil (MMF), and azathioprine have been used to prevent relapses of NMO, but their effectiveness in the treatment of acute exacerbations is unknown [48][49][50][51][52].…”