Clinical Characteristics and Outcomes of Generalized Myasthenia Gravis in Malaysia: A Single-Center Experience
0
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Background and Purpose Myasthenia gravis (MG) is clinically heterogeneous and can be classified into subgroups according to the clinical presentation, antibody status, age at onset, and thymic abnormalities. This study aimed to determine the clinical characteristics and outcomes of generalized MG (GMG) patients based on these subgroups. Methods Medical records of MG patients from 1976 to 2023 were reviewed retrospectively. Patients with pure ocular MG were excluded. Data on demographic, clinical characteristics, laboratory features, and outcomes were analyzed. Results This study included 120 GMG patients. There was a slight preponderance of female patients over male patients (male:female ratio=1:1.3), with the age at onset exhibiting a bimodal distribution. Female patients peaked at a lower age (21–30 years) whereas male patients peaked at a higher age (61–70 years). Most (92%, 105 of 114) patients had positive anti-acetylcholine receptor antibodies. Five patients were also tested for anti-muscle-specific tyrosine kinase antibodies, with two showing positivity. Thymectomy was performed in 62 (52%) patients, of which 30 had thymoma, 16 had thymic hyperplasia, 7 had an involuted thymus, and 6 had a normal thymus. There were significantly more female patients (68% vs. 45%, p=0.011) with early-onset disease (<50 years old) and thymic hyperplasia (33% vs. 0%, p<0.025). Most (71%) of the patients had a good outcome based on the Myasthenia Gravis Foundation of America postintervention status. GMG patients with early-onset disease had a significantly better outcome than patients with a late onset in univariate (58% vs. 37%, p=0.041) and multivariate (odds ratio=4.68, 95% confidence interval=1.17–18.64, p=0.029) analyses. Conclusions Female patients with early-onset MG and thymic hyperplasia had significantly better outcomes, but only early-onset disease was independently associated with a good outcome. These findings are comparable with those of other studies.
Abstract
Background and Purpose Myasthenia gravis (MG) is clinically heterogeneous and can be classified into subgroups according to the clinical presentation, antibody status, age at onset, and thymic abnormalities. This study aimed to determine the clinical characteristics and outcomes of generalized MG (GMG) patients based on these subgroups. Methods Medical records of MG patients from 1976 to 2023 were reviewed retrospectively. Patients with pure ocular MG were excluded. Data on demographic, clinical characteristics, laboratory features, and outcomes were analyzed. Results This study included 120 GMG patients. There was a slight preponderance of female patients over male patients (male:female ratio=1:1.3), with the age at onset exhibiting a bimodal distribution. Female patients peaked at a lower age (21–30 years) whereas male patients peaked at a higher age (61–70 years). Most (92%, 105 of 114) patients had positive anti-acetylcholine receptor antibodies. Five patients were also tested for anti-muscle-specific tyrosine kinase antibodies, with two showing positivity. Thymectomy was performed in 62 (52%) patients, of which 30 had thymoma, 16 had thymic hyperplasia, 7 had an involuted thymus, and 6 had a normal thymus. There were significantly more female patients (68% vs. 45%, p=0.011) with early-onset disease (<50 years old) and thymic hyperplasia (33% vs. 0%, p<0.025). Most (71%) of the patients had a good outcome based on the Myasthenia Gravis Foundation of America postintervention status. GMG patients with early-onset disease had a significantly better outcome than patients with a late onset in univariate (58% vs. 37%, p=0.041) and multivariate (odds ratio=4.68, 95% confidence interval=1.17–18.64, p=0.029) analyses. Conclusions Female patients with early-onset MG and thymic hyperplasia had significantly better outcomes, but only early-onset disease was independently associated with a good outcome. These findings are comparable with those of other studies.
참고문헌 (45)
- Hehir MK, "Generalized myasthenia gravis : classification, clinical presentation, natural history, and epidemiology", Neurol Clin, 36, pp.253~260 (2018)
- Keesey JC, "Clinical evaluation and management of myasthenia gravis", Muscle Nerve, 29, pp.484~505 (2004)
- Juel VC, "Myasthenia gravis", Orphanet J Rare Dis, 2, pp.44~ (2007)
- Gilhus NE, "Myasthenia gravis", Nat Rev Dis Primers, 5, pp.30~ (2019)
- Nicolle MW, "Myasthenia gravis and Lambert-Eaton myasthenic syndrome", Continuum(Minneap Minn), 22, pp.1978~2005 (2016)
- Gilhus NE, "Myasthenia gravis", N Engl J Med, 375, pp.2570~2581 (2016)
- Drachman DB, "Myasthenia gravis", Semin Neurol, 36, pp.419~424 (2016)
- Gilhus NE, "Myasthenia gravis : subgroup classification and therapeutic strategies", Lancet Neurol, 14, pp.1023~1036 (2015)
- DeHart-McCoyle M, "New and emerging treatments for myasthenia gravis", BMJ Med, 2, pp.e000241~ (2023)
- Jaretzki A 3rd, "Myasthenia gravis : recommendations for clinical research standards", Neurology, 55, pp.16~23 (2000)
- Salins S, "Low-dose medication and long-term outcome in myasthenia gravis", J Clin Neuromuscul Dis, 18, pp.61~66 (2016)
- Lee CY, "Clinical outcome of generalized myasthenia gravis in Hong Kong Chinese", J Neuroimmunol, 289, pp.177~181 (2015)
- Yildiz Celik S, "Late-onset generalized myasthenia gravis : clinical features, treatment, and outcome", Acta Neurol Belg, 120, pp.133~140 (2020)
- Alkhawajah NM, "Late-onset myasthenia gravis : a review when incidence in older adults keeps increasing", Muscle Nerve, 48, pp.705~710 (2013)
- Kara F, "Evaluation of clinical features and prognosis of myasthenia gravis in adults based on the age of onset : a retrospective study from a single center of 30 years MG registry", Neurol Asia, 27, pp.981~989 (2022)
- Wolfe GI, "Randomized trial of thymectomy in myasthenia gravis", N Engl J Med, 375, pp.511~522 (2016)
- Witthayaweerasak J, "Prognostic factors for conversion to generalization in ocular myasthenia gravis", Medicine(Baltimore), 100, pp.e25899~ (2021)
- Ding J, "Prediction of generalization of ocular myasthenia gravis under immunosuppressive therapy in Northwest China", BMC Neurol, 20, pp.238~ (2020)
- Teo KY, "Low conversion rate of ocular to generalized myasthenia gravis in Singapore", Muscle Nerve, 57, pp.756~760 (2018)
- Hong YH, "Prognosis of ocular myasthenia in Korea : a retrospective multicenter analysis of 202 patients", J Neurol Sci, 273, pp.10~14 (2008)
- Kamarajah SK, "Ocular presentation of myasthenia gravis : a natural history cohort", Muscle Nerve, 57, pp.622~627 (2018)
- Li F, "Generalization after ocular onset in myasthenia gravis : a case series in Germany", J Neurol, 265, pp.2773~2782 (2018)
- Aguirre F, "Prognosis of ocular myasthenia gravis in an Argentinian population", Eur Neurol, 79, pp.113~117 (2018)
- Gilhus NE, "Myasthenia gravis and infectious disease", J Neurol, 265, pp.1251~1258 (2018)
- Muralidhar Reddy Y, "Nocardiosis-an uncommon infection in patients with myasthenia gravis : report of three cases and review of literature", BMJ Case Rep, 13, pp.e237208~ (2020)
- Pedersen EG, "Risk of non-melanoma skin cancer in myasthenia patients treated with azathioprine", Eur J Neurol, 21, pp.454~458 (2014)
- Na R, "High azathioprine dose and lip cancer risk in liver, heart, and lung transplant recipients : a population-based cohort study", J Am Acad Dermatol, 74, pp.1144~1152 (2016)
- Zhang Z, "Cancer occurrence following azathioprine treatment in myasthenia gravis patients : a systematic review and meta-analysis", J Clin Neurosci, 88, pp.70~74 (2021)
- Wendell LC, "Myasthenic crisis", Neurohospitalist, 1, pp.16~22 (2011)
- Mao ZF, "Course and prognosis of myasthenia gravis : a systematic review", Eur J Neurol, 17, pp.913~921 (2010)
- Donaldson DH, "The relationship of age to outcome in myasthenia gravis", Neurology, 40, pp.786~790 (1990)
- Andersen JB, "Factors affecting outcome in myasthenia gravis", Muscle Nerve, 54, pp.1041~1049 (2016)
- Aarli JA, "Late-onset myasthenia gravis : a changing scene", Arch Neurol, 56, pp.25~27 (1999)
- Suzuki S, "Clinical and immunological differences between early and late-onset myasthenia gravis in Japan", J Neuroimmunol, 230, pp.148~152 (2011)
- Barnett C, "New insights into very-late-onset myasthenia gravis", Nat Rev Neurol, 16, pp.299~300 (2020)
- Vijayan J, "Clinical profile and impact of comorbidities in patients with very-late-onset myasthenia gravis", Muscle Nerve, 64, pp.462~466 (2021)
- Cortés-Vicente E, "Clinical and therapeutic features of myasthenia gravis in adults based on age at onset", Neurology, 94, pp.e1171~e1180 (2020)
- Evoli A, "Clinical characteristics and prognosis of myasthenia gravis in older people", J Am Geriatr Soc, 48, pp.1442~1448 (2000)
- Fan L, "Clinical differences of early and late-onset myasthenia gravis in 985 patients", Neurol Res, 41, pp.45~51 (2019)
- Tang YL, "Clinical characteristics and prognosis of very late-onset myasthenia gravis in China", Neuromuscul Disord, 33, pp.358~366 (2023)
- Meriggioli MN, "Autoimmune myasthenia gravis : emerging clinical and biological heterogeneity", Lancet Neurol, 8, pp.475~490 (2009)
- Hellmann MA, "Myasthenia gravis in the elderly", J Neurol Sci, 325, pp.1~5 (2013)
- Skeie GO, "Guidelines for treatment of autoimmune neuromuscular transmission disorders", Eur J Neurol, 17, pp.893~902 (2010)
- Farrugia ME, "A practical approach to managing patients with myasthenia gravis-opinions and a review of the literature", Front Neurol, 11, pp.604~ (2020)
- Ozawa Y, "Longterm outcomes and prognostic factors in generalized myasthenia gravis", J Neurol, 268, pp.3781~3788 (2021)
