Azathioprine plus corticosteroid treatment in Chinese patients with neuromyelitis optica

Qiu, Wei; Kermode, Allan G.; Li, Rui; Dai, Yongqiang; Wang, Yuge; Wang, Jingqi; Zhong, Xiaonan; Li, Caixia; Lu, Zhengqi

doi:10.1016/j.jocn.2015.01.028

Cited by 41 publications

(24 citation statements)

References 22 publications

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“…AZA, which has been previously reported to be partially effective in NMO [57–59], including in AQP4-IgG-positive NMOSD [60], was the most commonly applied IS therapy in our cohort. However, more than 80 % of all AZA-treated patients experienced at least one attack while under therapy.…”

Section: Discussionmentioning

confidence: 96%

MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome

Jarius

Ruprecht

Kleiter

et al. 2016

J Neuroinflammation

755

959

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Background: A subset of patients with neuromyelitis optica spectrum disorders (NMOSD) has been shown to be seropositive for myelin oligodendrocyte glycoprotein antibodies (MOG-IgG).Objective: To describe the epidemiological, clinical, radiological, cerebrospinal fluid (CSF), and electrophysiological features of a large cohort of MOG-IgG-positive patients with optic neuritis (ON) and/or myelitis (n = 50) as well as attack and long-term treatment outcomes.Methods: Retrospective multicenter study. Results: The sex ratio was 1:2.8 (m:f). Median age at onset was 31 years (range 6-70). The disease followed a multiphasic course in 80% (median time-to-first-relapse 5 months; annualized relapse rate 0.92) and resulted in significant disability in 40% (mean follow-up 75 ± 46.5 months), with severe visual impairment or functional blindness (36%) and markedly impaired ambulation due to paresis or ataxia (25%) as the most common long-term sequelae. Functional blindness in one or both eyes was noted during at least one ON attack in around 70%. Perioptic enhancement was present in several patients. Besides acute tetra-/paraparesis, dysesthesia and pain were common in acute myelitis (70%). Longitudinally extensive spinal cord lesions were frequent, but short lesions occurred at least once in 44%. Fourty-one percent had a history of simultaneous ON and myelitis. Clinical or radiological involvement of the brain, brainstem, or cerebellum was present in 50%; extra-opticospinal symptoms included intractable nausea and vomiting and respiratory insufficiency (fatal in one). CSF pleocytosis (partly neutrophilic) was present in 70%, oligoclonal bands in only 13%, and blood-CSF-barrier dysfunction in 32%. Intravenous methylprednisolone (IVMP) and long-term immunosuppression were often effective; however, treatment failure leading to rapid accumulation of disability was noted in many patients as well as flare-ups after steroid withdrawal. Full recovery was achieved by plasma exchange in some cases, including after IVMP failure. Breakthrough attacks under azathioprine were linked to the drug-specific latency period and a lack of cotreatment with oral steroids. Methotrexate was effective in 5/6 patients. Interferon-beta was associated with ongoing or increasing disease activity. Rituximab and ofatumumab were effective in some patients. However, treatment with rituximab was followed by early relapses in several cases; end-of-dose relapses occurred 9-12 months after the first infusion. Coexisting autoimmunity was rare (9%). Wingerchuk's 2006 and 2015 criteria for NMO(SD) and Barkhof and McDonald criteria for multiple sclerosis (MS) were met by 28%, 32%, 15%, 33%, respectively; MS had been suspected in 36%. Disease onset or relapses were preceded by infection, vaccination, or pregnancy/delivery in several cases. Conclusion: Our findings from a predominantly Caucasian cohort strongly argue against the concept of MOG-IgG denoting a mild and usually monophasic variant of NMOSD. The predominantly relapsing and often severe disease course ...

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Section: Discussionmentioning

confidence: 96%

MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome

Jarius

Ruprecht

Kleiter

et al. 2016

J Neuroinflammation

755

959

View full text Add to dashboard Cite

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“…Larger series have since supported the initial observations in both children and adults with doses of azathioprine at the higher range of 3 mg/kg/day plus concurrent prednisone showing more benefit than azathioprine monotherapy. In three large NMOSD cohorts of 99 (Mayo), 103 (UK), and 77 (China) subjects treated with azathioprine for at least 1 year, treatment with azathioprine was successful in preventing relapses in 37–57 % of subjects [24–26]. The annualized relapse rate (ARR) in the Mayo cohort dropped from 2.20 to 0.52 in those taking more than 2 mg/kg/day and dropped from 2.09 to 0.82 in those taking less than 2 mg/kg/day, suggesting a dose-response effect [24].…”

Section: Preventive Treatmentmentioning

confidence: 99%

Treatment of Neuromyelitis Optica Spectrum Disorder: Acute, Preventive, and Symptomatic

Kessler

Mealy

Lévy

2015

Curr Treat Options Neurol

128

105

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Opinion statement Neuromyelitis optica spectrum disorder (NMOSD) is a rare, autoimmune disease of the central nervous system that primarily attacks the optic nerves and spinal cord leading to blindness and paralysis. The spectrum of the disease has expanded based on the specificity of the autoimmune response to the aquaporin-4 water channel on astrocytes. With wider recognition of NMOSD, a standard of care for treatment of this condition has condition based on a growing series of retrospective and prospective studies. This review covers the present state of the field in the treatment of acute relapses, preventive approaches, and therapies for symptoms of NMOSD.

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“…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].…”

Section: Azamentioning

confidence: 99%

“…A third retrospective study of 31 patients with AQP4-IgG-positive or AQP4-IgG-negative NMOSD described a reduction in the mean ARR from 2.26 to 0.63 with a failure rate in 53 % of patients, despite concomitant prednisone therapy [79]. Another retrospective study of AZA plus long-term low-dose corticosteroid therapy in 77 Chinese patients found a relapse-free status at median follow-up of 19 months in 44 patients (57.1 %) and significant improvements of the ARR, EDSS, and modified Rankin Scale [80].…”

Section: Azamentioning

confidence: 99%

Present and Future Therapies in Neuromyelitis Optica Spectrum Disorders

Kleiter

Gold

2016

Neurotherapeutics

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Neuromyelitis optica spectrum disorders (NMOSD) are important evolving entities, which have reached much attention in the recent years. NMOSD are characterized by inflammatory lesions in the optic nerves, spinal cord, and central parts of the brain, as well as an autoimmune process directed against aquaporin-4. As disability in NMOSD accumulates by inflammatory damage from attacks, both the treatment and prevention of attacks are decisive for the long-term outcome. NMOSD attacks are treated with highdose intravenous corticosteroids and apheresis therapies, in particular therapeutic plasma exchange. In cases of incomplete remission, escalation of attack treatment is recommended. Preventive therapy is immunosuppressive and should by commenced as early as possible. Apart from classical immunosuppressants such as azathioprine and mycophenolate mofetil, repurposed biologicals are increasingly used. B-cell depletion with rituximab and other agents, inhibition of the interleukin-6 receptor with tocilizumab, and blockade of complement-mediated damage by eculizumab all are promising therapeutic strategies evaluated in randomized controlled trials. In this review, we will discuss present and future immunotherapies for NMOSD and also consider combination of treatments, plasma, cellular and other therapies. Current advances in immunopathological knowledge are translated into innovative concepts and begin a new era of NMOSD therapy.

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Azathioprine plus corticosteroid treatment in Chinese patients with neuromyelitis optica

Cited by 41 publications

References 22 publications

MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome

MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome

Treatment of Neuromyelitis Optica Spectrum Disorder: Acute, Preventive, and Symptomatic

Present and Future Therapies in Neuromyelitis Optica Spectrum Disorders

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