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迟发性重症肌无力的疗效指标与治疗效果

Outcome measures and treatment effectiveness in late onset myasthenia gravis.

作者信息

Pasqualin Francesca, Guidoni Silvia V, Ermani Mario, Pegoraro Elena, Bonifati Domenico M

机构信息

Ospedale regionale Ca' Foncello, Unit of Neurology, 31100 Treviso, Italy.

Department of Neuroscience, University of Padova, 35128 Padova, Italy.

出版信息

Neurol Res Pract. 2020 Oct 30;2:45. doi: 10.1186/s42466-020-00091-z. eCollection 2020.

Abstract

BACKGROUND

Recently different subtypes of myasthenia gravis (MG) have been described. They differ for clinical features and pathogenesis but the prognosis and response to treatment is less clear. The aim of the study was to evaluate outcome and treatment effectiveness including side effects in late onset MG (LOMG) compared with early onset MG (EOMG).

METHODS

We analysed retrospectively 208 MG patients. Clinical features were recorded as well as treatment and side effects. Outcome at the last follow-up was evaluated with MGSTI and MGPIS scales.

RESULTS

The 208 patients included were classified as follow: 36 ocular MG, 40 EOMG, 72 LOMG, 25 thymoma-associated, 14 anti-MuSK and 21 double seronegative. Similar positive outcome was achieved in either early and late onset subgroup. We found pharmacological remission and minimal manifestations at the MGFA-PIS in the 95% and 94,4% of EOMG and LOMG respectively but in LOMG a lower dose of immunosuppressors (MGSTI< 2) was required compared to EOMG ( = 0,048). Severe side effects were present in a small percentage of patients in both group but diabetes was more frequent in LOMG vs EOMG (2,2% vs 5%,  = 0.017).

CONCLUSIONS

Despite LOMG has more comorbidities that might interfere with treatment and outcome, therapeutic management does not seem to differ between EOMG and LOMG. A similar positive outcome was seen in both subgroups but LOMG group seems to require lower doses of medication to control symptoms.

摘要

背景

最近对重症肌无力(MG)的不同亚型进行了描述。它们在临床特征和发病机制上有所不同,但预后和对治疗的反应尚不清楚。本研究的目的是评估晚发型重症肌无力(LOMG)与早发型重症肌无力(EOMG)相比的治疗结果和有效性,包括副作用。

方法

我们回顾性分析了208例MG患者。记录了临床特征以及治疗情况和副作用。使用MGSTI和MGPIS量表评估最后一次随访时的治疗结果。

结果

纳入的208例患者分类如下:36例眼肌型MG、40例EOMG、72例LOMG、25例胸腺瘤相关型、14例抗MuSK型和21例双血清阴性型。早发型和晚发型亚组均取得了相似的良好治疗结果。我们发现,EOMG和LOMG分别有95%和94.4%的患者在MGFA-PIS中达到药理学缓解和最小表现,但与EOMG相比,LOMG所需的免疫抑制剂剂量更低(MGSTI<2)(P = 0.048)。两组中均有一小部分患者出现严重副作用,但LOMG组糖尿病的发生率高于EOMG组(2.2%对5%,P = 0.017)。

结论

尽管LOMG有更多可能干扰治疗和结果的合并症,但EOMG和LOMG之间的治疗管理似乎并无差异。两个亚组均出现了相似的良好治疗结果,但LOMG组似乎需要更低剂量的药物来控制症状。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1270/7650071/86cf7cc9a50e/42466_2020_91_Fig1_HTML.jpg

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