PDF(Pubmed)
Abstract：
Ocular myasthenia gravis (OMG) is an autoimmune disorder resulting in ocular symptoms such as diplopia and ptosis. The proportion of patients who convert to secondary generalized myasthenia gravis (SGMG) reported in the literature has been varied. The aim of this systematic review was to determine the clinical characteristics of patients with OMG and the proportion of SGMG conversion.
We conducted an electronic database search for randomized controlled trials, prospective nonrandomized studies, observational studies, and retrospective studies in EMBASE, CENTRAL, MEDLINE, and Web of Science. We included studies with patients with OMG who initially presented with ocular symptoms and signs only and were seen in clinical practice, reporting on the characteristics and outcomes of SGMG. We excluded studies with pediatric and congenital myasthenia gravis populations. Eligible studies included articles written in any language and containing data on patients with OMG. The main outcome measured was the proportion of patients with OMG who converted to SGMG and risk factors associated with secondary generalization of OMG. Two independent reviewers screened titles and abstracts and extracted data from full texts, reporting findings according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The methodology was evaluated using the Joanna Briggs Institute critical appraisal forms. PROSPERO registration number: CRD2021285257.
Thirty-one studies were included in the quantitative and qualitative analysis. The proportion of generalization ranged from 11% to 84%. The pooled proportion was 39% (95% CI 32%-47%, I 2 = 95.86%, p < 0.001 unweighted, low certainty). The pooled risk ratio of female sex for conversion to SGMG was 1.06 (95% CI 0.96-1.17, I 2 = 0% p = 0.614, 21 studies included, very low certainty), and the pooled risk ratio of acetylcholine receptor (AChR) positivity was 1.30 (95% CI 1.05-1.56, I 2 = 0% p = 0.455, 16 studies included, very low certainty).
Risk factors such as female sex and anti-AChR positivity have been identified to have possible associations with SGMG, but there are not enough quality observational studies. There is a need for a prospective global database of patients with OMG, including all countries with different populations.
We conducted an electronic database search for randomized controlled trials, prospective nonrandomized studies, observational studies, and retrospective studies in EMBASE, CENTRAL, MEDLINE, and Web of Science. We included studies with patients with OMG who initially presented with ocular symptoms and signs only and were seen in clinical practice, reporting on the characteristics and outcomes of SGMG. We excluded studies with pediatric and congenital myasthenia gravis populations. Eligible studies included articles written in any language and containing data on patients with OMG. The main outcome measured was the proportion of patients with OMG who converted to SGMG and risk factors associated with secondary generalization of OMG. Two independent reviewers screened titles and abstracts and extracted data from full texts, reporting findings according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The methodology was evaluated using the Joanna Briggs Institute critical appraisal forms. PROSPERO registration number: CRD2021285257.
Thirty-one studies were included in the quantitative and qualitative analysis. The proportion of generalization ranged from 11% to 84%. The pooled proportion was 39% (95% CI 32%-47%, I 2 = 95.86%, p < 0.001 unweighted, low certainty). The pooled risk ratio of female sex for conversion to SGMG was 1.06 (95% CI 0.96-1.17, I 2 = 0% p = 0.614, 21 studies included, very low certainty), and the pooled risk ratio of acetylcholine receptor (AChR) positivity was 1.30 (95% CI 1.05-1.56, I 2 = 0% p = 0.455, 16 studies included, very low certainty).
Risk factors such as female sex and anti-AChR positivity have been identified to have possible associations with SGMG, but there are not enough quality observational studies. There is a need for a prospective global database of patients with OMG, including all countries with different populations.
摘要：
背景：重症肌无力（OMG）是一种自身免疫性疾病，可导致眼部症状，例如复视和上睑下垂。文献中报道的转换为继发性广泛性重症肌无力（SGMG）的患者比例各不相同。本系统综述的目的是确定OMG患者的临床特征并确定SGMG转换的比例。
方法：我们对随机对照试验进行了电子数据库搜索，前瞻性非随机研究，EMBASE的观察性研究和回顾性研究，中部,MEDLINE和WebofScience。我们纳入了OMG患者的研究，这些患者最初仅在临床实践中出现眼部症状和体征。报告SGMG的特征和结果。我们排除了儿科和先天性重症肌无力人群的研究。Eligibile研究包括用任何语言撰写的文章，其中包含OMG患者的数据。测量的主要结果是转换为SGMG的OMG患者的比例以及与OMG的二次推广相关的危险因素。两名独立审稿人筛选标题和摘要，并从全文中提取数据，根据系统评价和荟萃分析（PRISMA）指南的首选报告项目报告结果。使用JoannaBriggs研究所的关键评估表格对方法进行了评估。PROSPERO注册号：CRD2021285257结果：31项研究纳入了定量和定性分析。泛化的比例从11％到84％不等。合并比例为39%(95%CI32%，47%,I2=95·86%，p<0·001未加权，低确定性)。转换为SGMG的女性合并风险比为1.06（95CI0·96,1·17，I2=0％p=0·614，包括21项研究，非常低的确定性)和AChR阳性的合并风险比为1·30(95CI1·05,1·56，I2=0%p=0·455，包括16项研究，非常低的确定性)。
结论：已确定女性和抗AChR阳性等危险因素可能与SGMG相关，但没有足够的高质量观察性研究。需要一个前瞻性的全球OMG患者数据库，包括所有不同人口的国家。
方法：我们对随机对照试验进行了电子数据库搜索，前瞻性非随机研究，EMBASE的观察性研究和回顾性研究，中部,MEDLINE和WebofScience。我们纳入了OMG患者的研究，这些患者最初仅在临床实践中出现眼部症状和体征。报告SGMG的特征和结果。我们排除了儿科和先天性重症肌无力人群的研究。Eligibile研究包括用任何语言撰写的文章，其中包含OMG患者的数据。测量的主要结果是转换为SGMG的OMG患者的比例以及与OMG的二次推广相关的危险因素。两名独立审稿人筛选标题和摘要，并从全文中提取数据，根据系统评价和荟萃分析（PRISMA）指南的首选报告项目报告结果。使用JoannaBriggs研究所的关键评估表格对方法进行了评估。PROSPERO注册号：CRD2021285257结果：31项研究纳入了定量和定性分析。泛化的比例从11％到84％不等。合并比例为39%(95%CI32%，47%,I2=95·86%，p<0·001未加权，低确定性)。转换为SGMG的女性合并风险比为1.06（95CI0·96,1·17，I2=0％p=0·614，包括21项研究，非常低的确定性)和AChR阳性的合并风险比为1·30(95CI1·05,1·56，I2=0%p=0·455，包括16项研究，非常低的确定性)。
结论：已确定女性和抗AChR阳性等危险因素可能与SGMG相关，但没有足够的高质量观察性研究。需要一个前瞻性的全球OMG患者数据库，包括所有不同人口的国家。
