Early fast-acting treatment strategy against generalized myasthenia gravis

Utsugisawa, Kimiaki; Nagane, Yuriko; Akaishi, Tetsuya; Suzuki, Yasushi; Imai, Tomihiro; Tsuda, Etsuko; Minami, Naoya; Uzawa, Akiyuki; Kawaguchi, Naoki; Masuda, Masayuki; Konno, Shingo; Suzuki, Hidekazu; Murai, Hiroyuki; Akiyama, Masashi

doi:10.1016/j.jns.2017.08.3054

Cited by 19 publications

(81 citation statements)

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“…This survey was carried out at 13 neurological centers in Japan. [22][23][24] We evaluated consecutive MG patients between April and July 2015 (4 months). During this period, we were able to collect full detailed clinical data from 923 patients.…”

Section: Survey and Data Analyses In 2015mentioning

confidence: 99%

“…Using this database, we attempted to clarify what type of treatment strategies would offer a better effect, and whether dosing regimens of oral steroid would show any effects on the frequency and time course of achieving MM-or-better-5 mg >6 months among generalized MG (gMG) patients. 22,23 Among these 923 patients, we also administered a questionnaire survey to obtain information on social disadvantages experienced by MG patients. We obtained 917 complete responses to our questionnaire.…”

Section: Survey and Data Analyses In 2015mentioning

confidence: 99%

“…5,[14][15][16][17] During assessments of associations between MG-QOL15 and detailed clinical information from our survey in 2010 to validate the scale, we noticed several important points warranting clarification in order to improve the QOL of MG patients. 5 In addition to a 2010 survey, 5,18 we carried out further multicenter surveys in 2012 6,[19][20][21] and 2015, [22][23][24] and obtained detailed quantitative data and some longitudinal clinical information from a total of 1890 patients with established MG. By analyzing such data, we clarified the actual condition of MG patients, 5,6,[18][19][20][21] determined major factors that negatively affect patient QOL, 5,6,18 recommended an appropriate treatment target to achieve good QOL, 5,6 and proposed treatment strategies to enable MG patients to achieve the target early and live a normal lifestyle. 22 Herein, we introduce our findings and current thinking about MG treatment.…”

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confidence: 99%

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Health‐related quality of life and treatment strategies in myasthenia gravis

Utsugisawa

Nagane

Uzawa

et al. 2020

Clinical & Exp Neuroim

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The spread of immune treatments based on oral steroids since the 1970s has led to a non-lethal disease course and decreased severity of illness for many patients with myasthenia gravis (MG). 1-6 However, the rate of long-term complete stable remission (CSR) has not been increased and remains quite low. 1-6 Even today, many patients experience difficulties with daily activities as a result of residual fatigability and long-term side-effects of medications, such as oral steroids, and they are unable to live a normal lifestyle. 1,2,5,6 Such conditions negatively influence health-related quality of life (QOL). 5-8 In Japan until the 2000s, a considerable proportion of neurologists were content with the better clinical symptoms after immune treatment and the preferable condition compared with the period when MG had been a severe disease with a high mortality rate. MG symptoms fluctuate and limit the utility of measuring disease status based solely on simple examination during clinical visits. 9,10 Given the delay in the adoption of quantitative estimation of fatigability

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Section: Survey and Data Analyses In 2015mentioning

confidence: 99%

Section: Survey and Data Analyses In 2015mentioning

confidence: 99%

mentioning

confidence: 99%

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Health‐related quality of life and treatment strategies in myasthenia gravis

Utsugisawa

Nagane

Uzawa

et al. 2020

Clinical & Exp Neuroim

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“…2). 18 Multivariate Cox regression analysis calculated a hazard ratio of 1.98 (P < 0.0001) for achieving MM-5 mg with utilization of EFT. Indeed, in the JAMG-R study group, the percentage of patients achieving this treatment goal increased from 37.4% in 2012 to 44.9% in 2015 as the EFT strategy was increasingly adopted (used in 36% of patients with generalized MG at the time of the study).…”

Section: Early Fast-acting Treatment Strategymentioning

confidence: 99%

Rationale for the clinical guidelines for myasthenia gravis in Japan

Murai

Utsugisawa

Nagane

et al. 2018

Annals of the New York Academy of Sciences

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According to the 2014 Japanese clinical guidelines for myasthenia gravis, the most important priority in treatment is maintaining patients’ health‐related quality of life. Therefore, the initial treatment goal is defined as maintaining a postintervention status of minimal manifestations or better (according to the Myasthenia Gravis Foundation of America classification) with an oral prednisolone dose of 5 mg/day or less. Every effort should be made to attain this level as rapidly as possible. To achieve this goal, the guidelines recommend minimizing the oral prednisolone dose, starting calcineurin inhibitors early in the course of treatment, using intravenous methylprednisolone infusion judiciously (often combined with plasma exchange/plasmapheresis or intravenous immunoglobulin), and effectively treating patients with an early, fast‐acting treatment strategy. The early, fast‐acting treatment strategy enables more frequent and earlier attainment of the initial goal than other strategies. Thymectomy is considered an option for treating nonthymomatous early‐onset myasthenia gravis in patients with antiacetylcholine receptor antibodies and thymic hyperplasia in the early stages of the disease.

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“…11 However, Japanese studies also showed a considerable population (40%-50%) of generalized MG patients who had difficulty controlling disease progression even with the new treatment strategy. 10,12 These patients failed to respond to adequate doses and durations of conventional immunotherapies and required excessive fast-acting treatments such as PE/PP and IVIg.…”

Section: Introductionmentioning

confidence: 99%

Why is development of new treatments necessary for myasthenia gravis? Recent advances in clinical trials

Imai

2019

Neurology & Clinical Neurosc

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Despite the availability of an increasing variety of disease‐modifying therapies, the proportion of patients with generalized myasthenia gravis (MG) who achieved the practical treatment goal remained low at approximately 50%‐60% in our latest survey. Therefore, there is a need for further development of new treatment options based on novel mechanisms of action for MG treatment. Eculizumab and ravulizumab are monoclonal antibodies used to prevent complement‐mediated damage at the endplate of neuromuscular junction in MG with acetylcholine receptor (AChR) antibody. Neonatal Fc receptor (FcRn) antibodies including efgartigimod and rozanolixizumab are currently in different stages of clinical trial. The FcRn antibodies would be rational therapeutic agents for decreasing the levels of pathogenic autoantibodies with IgG isotypes, resulting in reduction in muscle‐specific kinase (MuSK) antibody as well as AChR antibody. Rituximab, a CD20+ B‐cell depleting monoclonal antibody, also results in a significant decrease in autoantibody titers in serum. Several studies indicate that rituximab may have the potential to treat MuSK‐MG. Monarsen, an antisense oligonucleotide, may reduce the production of acetylcholinesterase. A phase 2 study showed improvement in quantitative MG scores while on the drug. Several case reports showed the efficacy of autologous hemopoietic stem cell transplantation in patients with severe MG refractory to conventional therapies. This article introduces the new drugs and their latest development and discusses the evidence for thymectomy in relation to the Japanese clinical guidelines for MG.

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Early fast-acting treatment strategy against generalized myasthenia gravis

Cited by 19 publications

References 0 publications

Health‐related quality of life and treatment strategies in myasthenia gravis

Health‐related quality of life and treatment strategies in myasthenia gravis

Rationale for the clinical guidelines for myasthenia gravis in Japan

Why is development of new treatments necessary for myasthenia gravis? Recent advances in clinical trials

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