Race, sex, and puberty influence onset, severity, and outcome in juvenile myasthenia gravis

Abstract

We assessed the influence of race, sex, and puberty upon clinical features and outcome in 115 patients with autoimmune juvenile myasthenia gravis (JMG). These demographic variables influenced not only disease incidence but also disease severity, response to therapy, and outcome, despite comparable therapeutic strategies. Among white patients, those with prepubertal onset had low incidence and equal sex ratio; the incidence in females increased during and after puberty; males had lesser disease severity than females during and after puberty (p < 0.05); spontaneous remissions were most frequent (44%, p = 0.001) and persistence of active JMG for more than 10 years was least frequent (p = 0.05) in patients with prepubertal onset; remissions were more frequent after early than late thymectomy (p = 0.03); and final disease severity was less after early than late thymectomy. Black patients had similar incidence, disease severity, and sex ratio (F:M = 2:1) with pre-, peri-, or postpubertal disease onset; infrequent spontaneous or treatment-induced remissions; and the same final disease severity after early or late thymectomy. These observations imply that race and sex hormones modify the clinical features and outcome of JMG; spontaneous remissions are common in white patients with prepubertal disease onset; early thymectomy may be more beneficial than late thymectomy in white patients; and the role of thymectomy in the youngest patients is uncertain. We suggest that demographic factors should be considered when evaluating past and future therapeutic strategies for JMG.

The characteristics of juvenile myasthenia gravis among South Africans

“…The MGFA grades at presentation and frequency of AChR-positive generalised MG among this cohort were similar to other juvenile cohorts. 3,6,10 Remission rates in children are generally said to be high (11 -37%). 2,6,7,10 Our overall remission rate of 45%, of whom approximately 10% went into spontaneous remission, is comparable.…”

“…3,6,10 Remission rates in children are generally said to be high (11 -37%). 2,6,7,10 Our overall remission rate of 45%, of whom approximately 10% went into spontaneous remission, is comparable. Compared with generalised disease, ocular MG showed more frequent remissions and more often without the use of immunosuppressive therapies.…”

Multivariate determinants of the need for postoperative ventilation in myasthenia gravis

“…The influence of sex hormones on myasthenia gravis is evidenced by their effects on the incidence of myasthenia gravis, ~9,2~ the variation of disease severity with the menstrual cycle in one third of women with myasthenia gravis, 2~ and the increased number of oestrogen receptors on lymphocytes and thymocytes in patients with myasthenia gravis. 2= Recently, Andrews et al 22 reported that sex influenced not only disease incidence but also disease severity, response to therapy, and outcome in myasthemia gravis patients.…”

Miastenia grave: avaliação clínica de 153 pacientes

“…A etiopatogênese é diferente nas formas neonatal e congênita. As adquiridas têm caráter auto-imune, com produção de auto-anticorpos anti-receptores de acetilcolina (AAChR) que bloqueiam os receptores de acetilcolina (AchR) na placa motora pós-sináptica, comprometendo sua função , 2,3,7,8,[13][14][15][16][17][18][19][20] . Com base etiopatogênica classifica-se a doença em dois grupos: miastenia grave não timomatosa e timomatosa.…”

“…A doença pode ocorrer em qualquer faixa de idade, do nascimento aos 80 anos, tendo maior comprometimento aos 26 anos nas mulheres e 3l anos nos homens. Nos vários estudos, as mulheres são mais acometidas do que os homens até quarta década, variando de 2 a 4:1 1,6,7,9,10,12,13,18,20,27 .…”