Clinical features, treatment and prognosis of MuSK antibody-associated myasthenia gravis in Northwest China: a single-centre retrospective cohort study

Abstract Background and purpose To summarize the clinical characteristics of patients with muscle-specific kinase antibody-associated myasthenia gravis (MuSK-MG) and to evaluate the therapeutic responses to different treatment regimes. Methods Eighteen MuSK-MG patients admitted in our department bet...

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Autores principales:	Sijia Zhao, Kai Zhang, Kaixi Ren, Jiarui Lu, Chao Ma, Cong Zhao, Zhuyi Li, Jun Guo
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Muscle-specific kinase Myasthenia gravis Rituximab Tacrolimus Azathioprine Neurology. Diseases of the nervous system RC346-429
Acceso en línea:	https://doaj.org/article/06a2091176234cacbe2f0df117658544
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spelling	oai:doaj.org-article:06a2091176234cacbe2f0df1176585442021-11-07T12:23:16ZClinical features, treatment and prognosis of MuSK antibody-associated myasthenia gravis in Northwest China: a single-centre retrospective cohort study10.1186/s12883-021-02439-71471-2377https://doaj.org/article/06a2091176234cacbe2f0df1176585442021-11-01T00:00:00Zhttps://doi.org/10.1186/s12883-021-02439-7https://doaj.org/toc/1471-2377Abstract Background and purpose To summarize the clinical characteristics of patients with muscle-specific kinase antibody-associated myasthenia gravis (MuSK-MG) and to evaluate the therapeutic responses to different treatment regimes. Methods Eighteen MuSK-MG patients admitted in our department between October 2017 and September 2020 were included. Clinical parameters were collected and the responses to different immunosuppressive drugs were assessed by MGFA Postintervention Status (MGFA-PIS). Meanwhile, the correlation between QMG scores and MuSK antibody titers were analyzed and MuSK antibody (MuSK-ab) titers were compared before and after therapy based on different immunosuppressive treatment regimes. Results Female predominance (ratio of females to males, 15:3) was evident in the study population, with the average onset age of (40.28 ± 18.57) years and the median disease course of 30.50 months (interquartile range [IQR], 17.50–44.75 months). Ocular manifestation was the most common onset symptom (11/18; 61.11%), and mild symmetrical ptosis was most frequent. Bulbar symptoms had the highest incidence of 88.89% over the entire disease course. Abnormal responses to RNS test were recorded most frequently on the musculus deltoideus (83.33%). All patients were treated with prednisone (Pred) alone or plus azathioprine (AZA), tacrolimus (TAC) or low-dose rituximab (RTX), and 17 (94.44%) of them achieved a favorable outcome defined as minimal manifestation (MM) or better. In general, an obvious positive correlation between QMG score and MuSK-ab titer (r = 0.710, P < 0.001) were found in all patients. A more significant reduction of MuSK-ab titers was observed in patients receiving TAC or RTX plus Pred than those receiving AZA plus Pred. Conclusions The prominent clinical manifestations of ocular and bulbar muscles involvements, together with abnormal RNS response mostly recorded on the musculus deltoideus and better efficacy associated with TAC or low-dose RTX plus Pred, provide a more exhaustive picture of MuSK-MG, particularly in Northwest China.Sijia ZhaoKai ZhangKaixi RenJiarui LuChao MaCong ZhaoZhuyi LiJun GuoBMCarticleMuscle-specific kinaseMyasthenia gravisRituximabTacrolimusAzathioprineNeurology. Diseases of the nervous systemRC346-429ENBMC Neurology, Vol 21, Iss 1, Pp 1-9 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Muscle-specific kinase Myasthenia gravis Rituximab Tacrolimus Azathioprine Neurology. Diseases of the nervous system RC346-429
spellingShingle	Muscle-specific kinase Myasthenia gravis Rituximab Tacrolimus Azathioprine Neurology. Diseases of the nervous system RC346-429 Sijia Zhao Kai Zhang Kaixi Ren Jiarui Lu Chao Ma Cong Zhao Zhuyi Li Jun Guo Clinical features, treatment and prognosis of MuSK antibody-associated myasthenia gravis in Northwest China: a single-centre retrospective cohort study
description	Abstract Background and purpose To summarize the clinical characteristics of patients with muscle-specific kinase antibody-associated myasthenia gravis (MuSK-MG) and to evaluate the therapeutic responses to different treatment regimes. Methods Eighteen MuSK-MG patients admitted in our department between October 2017 and September 2020 were included. Clinical parameters were collected and the responses to different immunosuppressive drugs were assessed by MGFA Postintervention Status (MGFA-PIS). Meanwhile, the correlation between QMG scores and MuSK antibody titers were analyzed and MuSK antibody (MuSK-ab) titers were compared before and after therapy based on different immunosuppressive treatment regimes. Results Female predominance (ratio of females to males, 15:3) was evident in the study population, with the average onset age of (40.28 ± 18.57) years and the median disease course of 30.50 months (interquartile range [IQR], 17.50–44.75 months). Ocular manifestation was the most common onset symptom (11/18; 61.11%), and mild symmetrical ptosis was most frequent. Bulbar symptoms had the highest incidence of 88.89% over the entire disease course. Abnormal responses to RNS test were recorded most frequently on the musculus deltoideus (83.33%). All patients were treated with prednisone (Pred) alone or plus azathioprine (AZA), tacrolimus (TAC) or low-dose rituximab (RTX), and 17 (94.44%) of them achieved a favorable outcome defined as minimal manifestation (MM) or better. In general, an obvious positive correlation between QMG score and MuSK-ab titer (r = 0.710, P < 0.001) were found in all patients. A more significant reduction of MuSK-ab titers was observed in patients receiving TAC or RTX plus Pred than those receiving AZA plus Pred. Conclusions The prominent clinical manifestations of ocular and bulbar muscles involvements, together with abnormal RNS response mostly recorded on the musculus deltoideus and better efficacy associated with TAC or low-dose RTX plus Pred, provide a more exhaustive picture of MuSK-MG, particularly in Northwest China.
format	article
author	Sijia Zhao Kai Zhang Kaixi Ren Jiarui Lu Chao Ma Cong Zhao Zhuyi Li Jun Guo
author_facet	Sijia Zhao Kai Zhang Kaixi Ren Jiarui Lu Chao Ma Cong Zhao Zhuyi Li Jun Guo
author_sort	Sijia Zhao
title	Clinical features, treatment and prognosis of MuSK antibody-associated myasthenia gravis in Northwest China: a single-centre retrospective cohort study
title_short	Clinical features, treatment and prognosis of MuSK antibody-associated myasthenia gravis in Northwest China: a single-centre retrospective cohort study
title_full	Clinical features, treatment and prognosis of MuSK antibody-associated myasthenia gravis in Northwest China: a single-centre retrospective cohort study
title_fullStr	Clinical features, treatment and prognosis of MuSK antibody-associated myasthenia gravis in Northwest China: a single-centre retrospective cohort study
title_full_unstemmed	Clinical features, treatment and prognosis of MuSK antibody-associated myasthenia gravis in Northwest China: a single-centre retrospective cohort study
title_sort	clinical features, treatment and prognosis of musk antibody-associated myasthenia gravis in northwest china: a single-centre retrospective cohort study
publisher	BMC
publishDate	2021
url	https://doaj.org/article/06a2091176234cacbe2f0df117658544
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Clinical features, treatment and prognosis of MuSK antibody-associated myasthenia gravis in Northwest China: a single-centre retrospective cohort study

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