Clinical features and treatment status of adult myasthenia gravis in Japan

Hiroyuki Murai, Masayuki Masuda, Kimiaki Utsugisawa, Yuriko Nagane, Shigeaki Suzuki, Tomihiro Imai, Masakatsu Motomura, Shingo Konno, Jun Ichi Kira

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Abstract

Objective Myasthenia gravis (MG) is classified as early-onset MG (EOMG; age at onset ≤49 years), late-onset MG (LOMG; age at onset ≥50 years) or thymoma-associated MG (TAMG) (E-L-T classification). To clarify the characteristics of each group in the E-L-T classification in Japan, we carried out multicenter analyses of MG. Methods A total of 640 adult patients from 11 MG centers participated in the study. Age at onset, sex, clinical symptoms, frequency of crisis, thymic pathology, positivity of autoantibodies against acetylcholine receptor (AChR) and muscle-specific receptor tyrosine kinase (MuSK), selected treatment, Cushingoid appearance and post-intervention status were evaluated in each group. Results EOMG, LOMG and TAMG accounted for 44%, 33%, and 23% of the patients, respectively. Females predominated in the EOMG group (77%), whereas there was no sex difference in the LOMG group. The frequency of ocular MG was the highest in the LOMG group (EOMG 15%, LOMG 38%, TAMG 12%). Bulbar symptoms and crisis were most frequent in the TAMG group. Anti-AChR antibody was always positive in patients with TAMG (EOMG 70%, LOMG 78%, TAMG 99%), whereas anti-MuSK antibody was never positive in TAMG patients, and more frequently detected in EOMG patients than in LOMG patients. Thymectomy was carried out in 51% of EOMG patients, 26% of LOMG patients and 97% of TAMG patients. Immunotherapy was carried out most aggressively in TAMG patients, and least aggressively in LOMG patients. Minimal manifestations or better with prednisolone ≤5 mg were achieved only in one-third of EOMG and TAMG patients. Conclusion Thymoma-associated MG required the most aggressive immunotherapy, followed by early-onset MG.

Original languageEnglish
Pages (from-to)84-91
Number of pages8
JournalClinical and Experimental Neuroimmunology
Volume5
Issue number1
DOIs
Publication statusPublished - 2014

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Myasthenia Gravis
Thymoma
Japan
Therapeutics
Age of Onset
Receptor Protein-Tyrosine Kinases
Cholinergic Receptors
Immunotherapy
Muscles
Thymectomy
Antibodies
Prednisolone
Sex Characteristics
Autoantibodies
Pathology

Keywords

  • classification
  • corticosteroids
  • immunotherapy
  • myasthenia gravis
  • treatment

ASJC Scopus subject areas

  • Clinical Neurology
  • Immunology and Microbiology (miscellaneous)
  • Neuroscience (miscellaneous)
  • Immunology

Cite this

Clinical features and treatment status of adult myasthenia gravis in Japan. / Murai, Hiroyuki; Masuda, Masayuki; Utsugisawa, Kimiaki; Nagane, Yuriko; Suzuki, Shigeaki; Imai, Tomihiro; Motomura, Masakatsu; Konno, Shingo; Kira, Jun Ichi.

In: Clinical and Experimental Neuroimmunology, Vol. 5, No. 1, 2014, p. 84-91.

Research output: Contribution to journalArticle

Murai, H, Masuda, M, Utsugisawa, K, Nagane, Y, Suzuki, S, Imai, T, Motomura, M, Konno, S & Kira, JI 2014, 'Clinical features and treatment status of adult myasthenia gravis in Japan', Clinical and Experimental Neuroimmunology, vol. 5, no. 1, pp. 84-91. https://doi.org/10.1111/cen3.12091
Murai, Hiroyuki ; Masuda, Masayuki ; Utsugisawa, Kimiaki ; Nagane, Yuriko ; Suzuki, Shigeaki ; Imai, Tomihiro ; Motomura, Masakatsu ; Konno, Shingo ; Kira, Jun Ichi. / Clinical features and treatment status of adult myasthenia gravis in Japan. In: Clinical and Experimental Neuroimmunology. 2014 ; Vol. 5, No. 1. pp. 84-91.
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abstract = "Objective Myasthenia gravis (MG) is classified as early-onset MG (EOMG; age at onset ≤49 years), late-onset MG (LOMG; age at onset ≥50 years) or thymoma-associated MG (TAMG) (E-L-T classification). To clarify the characteristics of each group in the E-L-T classification in Japan, we carried out multicenter analyses of MG. Methods A total of 640 adult patients from 11 MG centers participated in the study. Age at onset, sex, clinical symptoms, frequency of crisis, thymic pathology, positivity of autoantibodies against acetylcholine receptor (AChR) and muscle-specific receptor tyrosine kinase (MuSK), selected treatment, Cushingoid appearance and post-intervention status were evaluated in each group. Results EOMG, LOMG and TAMG accounted for 44{\%}, 33{\%}, and 23{\%} of the patients, respectively. Females predominated in the EOMG group (77{\%}), whereas there was no sex difference in the LOMG group. The frequency of ocular MG was the highest in the LOMG group (EOMG 15{\%}, LOMG 38{\%}, TAMG 12{\%}). Bulbar symptoms and crisis were most frequent in the TAMG group. Anti-AChR antibody was always positive in patients with TAMG (EOMG 70{\%}, LOMG 78{\%}, TAMG 99{\%}), whereas anti-MuSK antibody was never positive in TAMG patients, and more frequently detected in EOMG patients than in LOMG patients. Thymectomy was carried out in 51{\%} of EOMG patients, 26{\%} of LOMG patients and 97{\%} of TAMG patients. Immunotherapy was carried out most aggressively in TAMG patients, and least aggressively in LOMG patients. Minimal manifestations or better with prednisolone ≤5 mg were achieved only in one-third of EOMG and TAMG patients. Conclusion Thymoma-associated MG required the most aggressive immunotherapy, followed by early-onset MG.",
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AU - Murai, Hiroyuki

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AU - Suzuki, Shigeaki

AU - Imai, Tomihiro

AU - Motomura, Masakatsu

AU - Konno, Shingo

AU - Kira, Jun Ichi

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N2 - Objective Myasthenia gravis (MG) is classified as early-onset MG (EOMG; age at onset ≤49 years), late-onset MG (LOMG; age at onset ≥50 years) or thymoma-associated MG (TAMG) (E-L-T classification). To clarify the characteristics of each group in the E-L-T classification in Japan, we carried out multicenter analyses of MG. Methods A total of 640 adult patients from 11 MG centers participated in the study. Age at onset, sex, clinical symptoms, frequency of crisis, thymic pathology, positivity of autoantibodies against acetylcholine receptor (AChR) and muscle-specific receptor tyrosine kinase (MuSK), selected treatment, Cushingoid appearance and post-intervention status were evaluated in each group. Results EOMG, LOMG and TAMG accounted for 44%, 33%, and 23% of the patients, respectively. Females predominated in the EOMG group (77%), whereas there was no sex difference in the LOMG group. The frequency of ocular MG was the highest in the LOMG group (EOMG 15%, LOMG 38%, TAMG 12%). Bulbar symptoms and crisis were most frequent in the TAMG group. Anti-AChR antibody was always positive in patients with TAMG (EOMG 70%, LOMG 78%, TAMG 99%), whereas anti-MuSK antibody was never positive in TAMG patients, and more frequently detected in EOMG patients than in LOMG patients. Thymectomy was carried out in 51% of EOMG patients, 26% of LOMG patients and 97% of TAMG patients. Immunotherapy was carried out most aggressively in TAMG patients, and least aggressively in LOMG patients. Minimal manifestations or better with prednisolone ≤5 mg were achieved only in one-third of EOMG and TAMG patients. Conclusion Thymoma-associated MG required the most aggressive immunotherapy, followed by early-onset MG.

AB - Objective Myasthenia gravis (MG) is classified as early-onset MG (EOMG; age at onset ≤49 years), late-onset MG (LOMG; age at onset ≥50 years) or thymoma-associated MG (TAMG) (E-L-T classification). To clarify the characteristics of each group in the E-L-T classification in Japan, we carried out multicenter analyses of MG. Methods A total of 640 adult patients from 11 MG centers participated in the study. Age at onset, sex, clinical symptoms, frequency of crisis, thymic pathology, positivity of autoantibodies against acetylcholine receptor (AChR) and muscle-specific receptor tyrosine kinase (MuSK), selected treatment, Cushingoid appearance and post-intervention status were evaluated in each group. Results EOMG, LOMG and TAMG accounted for 44%, 33%, and 23% of the patients, respectively. Females predominated in the EOMG group (77%), whereas there was no sex difference in the LOMG group. The frequency of ocular MG was the highest in the LOMG group (EOMG 15%, LOMG 38%, TAMG 12%). Bulbar symptoms and crisis were most frequent in the TAMG group. Anti-AChR antibody was always positive in patients with TAMG (EOMG 70%, LOMG 78%, TAMG 99%), whereas anti-MuSK antibody was never positive in TAMG patients, and more frequently detected in EOMG patients than in LOMG patients. Thymectomy was carried out in 51% of EOMG patients, 26% of LOMG patients and 97% of TAMG patients. Immunotherapy was carried out most aggressively in TAMG patients, and least aggressively in LOMG patients. Minimal manifestations or better with prednisolone ≤5 mg were achieved only in one-third of EOMG and TAMG patients. Conclusion Thymoma-associated MG required the most aggressive immunotherapy, followed by early-onset MG.

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