Oral corticosteroid dosing regimen and long-term prognosis in generalised myasthenia gravis: a multicentre cross-sectional study in Japan

Objective We examined the correlation between the dosing regimen of oral prednisolone (PSL) and the achievement of minimal manifestation status or better on PSL ≤5 mg/day lasting >6 months (the treatment target) in patients with generalised myasthenia gravis (MG). Methods We classified 590 patients with generalised MG into high-dose (n=237), intermediate-dose (n=187) and low-dose (n=166) groups based on the oral PSL dosing regimen, and compared the clinical characteristics, previous treatments other than PSL and prognosis between three groups. The effect of oral PSL dosing regimen on the achievement of the treatment target was followed for 3 years of treatment. Results To achieve the treatment target, ORs for low-dose versus high-dose regimen were 10.4 (P<0.0001) after 1 year of treatment, 2.75 (P=0.007) after 2 years and 1.86 (P=0.15) after 3 years; and those for low-dose versus intermediate-dose regimen were 13.4 (P<0.0001) after 1 year, 3.99 (P=0.0003) after 2 years and 4.92 (P=0.0004) after 3 years. Early combined use of fast-acting treatment (OR: 2.19 after 2 years, P=0.02; OR: 2.11 after 3 years, P=0.04) or calcineurin inhibitors (OR: 2.09 after 2 years, P=0.03; OR: 2.36 after 3 years, P=0.02) was associated positively with achievement of treatment target. Conclusion A low-dose PSL regimen with early combination of other treatment options may ensure earlier achievement of the treatment target in generalised MG.

[1]  M. Freimer Faculty Opinions recommendation of International consensus guidance for management of myasthenia gravis: Executive summary. , 2018, Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature.

[2]  Hidekazu Suzuki,et al.  Early fast‐acting treatment strategy against generalized myasthenia gravis , 2017, Muscle & nerve.

[3]  G. Cutter,et al.  Randomized trial of thymectomy in myasthenia gravis. , 2016, Journal of thoracic disease.

[4]  M. Dalakas Treating myasthenia on consensus guide: Helpful and challenging but still unfinished business , 2016, Neurology.

[5]  K. Fujihara,et al.  Oral corticosteroid therapy and present disease status in myasthenia gravis , 2015, Muscle & nerve.

[6]  Shigeaki Suzuki,et al.  Treatment of myasthenia gravis patients with calcineurin inhibitors in Japan: A retrospective analysis of outcomes , 2015 .

[7]  H. Murai Japanese clinical guidelines for myasthenia gravis: Putting into practice , 2015 .

[8]  K. Fujihara,et al.  Health‐related quality‐of‐life and treatment targets in myasthenia gravis , 2014, Muscle & nerve.

[9]  K. Fujihara,et al.  The MG‐QOL15 Japanese version: Validation and associations with clinical factors , 2012, Muscle & nerve.

[10]  T. Komori,et al.  Reference values for voluntary and stimulated single-fibre EMG using concentric needle electrodes: A multicentre prospective study , 2012, Clinical Neurophysiology.

[11]  K. Fujihara,et al.  [The MG-QOL15 Japanese version: validation and associations with clinical factors]. , 2012, Rinsho shinkeigaku = Clinical neurology.

[12]  T. Kiuchi,et al.  Randomised, double-blind, placebo-controlled study of tacrolimus in myasthenia gravis , 2011, Journal of Neurology, Neurosurgery & Psychiatry.

[13]  V. Bril,et al.  Comparison of IVIg and PLEX in patients with myasthenia gravis , 2011, Neurology.

[14]  Shigeaki Suzuki,et al.  Early Aggressive Treatment Strategy against Myasthenia Gravis , 2010, European Neurology.

[15]  D. Sanders,et al.  Immunosuppressive therapies in myasthenia gravis , 2010, Autoimmunity.

[16]  Shigeaki Suzuki,et al.  Factors associated with response to calcineurin inhibitors in myasthenia gravis , 2010, Muscle & nerve.

[17]  D. Sanders,et al.  Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity , 2009, The Lancet Neurology.

[18]  N. Gilhus Autoimmune myasthenia gravis , 2009, Expert review of neurotherapeutics.

[19]  A trial of mycophenolate mofetil with prednisone as initial immunotherapy in myasthenia gravis , 2008, Neurology.

[20]  M. Pagala,et al.  Lifetime course of myasthenia gravis , 2008, Muscle & nerve.

[21]  L. Zinman,et al.  IV immunoglobulin in patients with myasthenia gravis , 2007, Neurology.

[22]  D B Sanders,et al.  Myasthenia gravis: recommendations for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. , 2000, Neurology.

[23]  R. Barohn,et al.  Myasthenia gravis , 2000, Neurology.

[24]  A. Lefvert,et al.  Treatment of myasthenia gravis with methylprednisolone pulse: a double blind study , 1998, Acta neurologica Scandinavica.

[25]  S. Chevret,et al.  Clinical trial of plasma exchange and high‐dose intravenous immunoglobulin in myasthenia gravis , 1997 .

[26]  A. Pestronk,et al.  Autoimmune myasthenia gravis. , 1993, Hospital practice.

[27]  H. Coslett,et al.  Long‐term corticosteriod treatment of myasthenia gravis: Report of 116 Patients , 1984, Annals of neurology.

[28]  L. Rowland Controversies about the treatment of myasthenia gravis. , 1980, Journal of neurology, neurosurgery, and psychiatry.