Ocular myasthenia gravis and risk factors for developing a secondary generalisation: description of a Spanish series

Díaz-Maroto, Inmaculada; García-García, J.; Sánchez-Ayaso, P.A.; Alcahut-Rodríguez, C.; González-Villar, E.; Pardal-Fernández, José Manuel; Segura, Tomás

doi:10.1016/j.nrleng.2020.09.004

Cited by 6 publications

(16 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A 2020 retrospective study in 62 adults with myasthenia gravis showed a high rate of conversion during the first 2 years of progression, and a strong association with female sex and with the presence of acetylcholine receptor antibodies. 43 Corticosteroid treatment is generally accepted as firstline immunosuppressive treatment in all autoimmune neuromuscular junction disorders. The conventional policy of aiming for remission by using high daily doses of 60 mg prednisolone, and then reducing to a low maintenance dose, is still the most frequently used schedule globally.…”

Section: Immunosuppressive Treatmentmentioning

confidence: 99%

Advances and ongoing research in the treatment of autoimmune neuromuscular junction disorders

Verschuuren¹,

Palace²,

Murai³

et al. 2022

The Lancet Neurology

View full text Add to dashboard Cite

Section: Immunosuppressive Treatmentmentioning

confidence: 99%

Advances and ongoing research in the treatment of autoimmune neuromuscular junction disorders

Verschuuren¹,

Palace²,

Murai³

et al. 2022

The Lancet Neurology

View full text Add to dashboard Cite

“…Of the 31 studies, only 5 studies found an association of female sex with risk of generalization. 7,22-25 Only 1 study demonstrated male sex to have an association with generalization. 26 Martinka et al reported a significant risk ratio of 1.23 of male sex with generalization.…”

Section: Resultsmentioning

confidence: 99%

“…7,21,22,28 Fifteen of 31 studies found an association with positive AChR as a risk factor of generalization. 9,[19][20][21][22]24,25,28,29,34,[36][37][38][39][40] Eight studies reported no association with AChR antibodies and secondary generalization. 7,9,32,33,35,[41][42][43][44] The pooled risk ratio of AChR positivity to SGMG was 1.30 (95% CI 1.05-1.56, I 2 = 0% p = 0.455, 16 studies included) (Figure 4).…”

Section: Figure 3 Rr Of Female Sex With Secondary Generalizationmentioning

confidence: 99%

“…7,9,22,29,31,32,[42][43][44] Two studies reported the results of sfEMG testing, but did not analyze for association with SGMG. 20,24 Neurophysiology: RNS RNS was reported in 16 of 31 studies. [20][21][22][23][24]29,32,33,35,37,38,41,43,[45][46][47] Of these, 8 studies reported on both sfEMG and RNS data (eTable 3, links.lww.com/WNL/D67).…”

Section: Figure 3 Rr Of Female Sex With Secondary Generalizationmentioning

confidence: 99%

“…42 Seven of 29 studies (24%, pooled n = 754) showed no effect of immunosuppression on SGMG risk. 20,21,24,27,28,37,39 Risk of Bias and Publication Bias Ottawa Newcastle Score was assessed for all studies included (eTable 6, links.lww.com/WNL/D67). Few studies reported on loss of follow-up (attrition bias).…”

Section: Treatments/interventionmentioning

confidence: 99%

See 2 more Smart Citations

Clinical Characteristics Associated With Secondary Generalization in Patients With Ocular Myasthenia Gravis

Fang,

Bokre,

Wong

2023

Neurology

View full text Add to dashboard Cite

Background and Objectives 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. Methods 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. Results 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). Discussion 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.

show abstract

Effect of Immunosuppression in Risk of Developing Generalized Symptoms in Ocular Myasthenia Gravis

Menon,

Alharbi,

Katzberg

et al. 2024

Neurology

View full text Add to dashboard Cite

Backgrounds and ObjectivesEarly use of immunosuppression has been suggested to prevent generalization of ocular myasthenia gravis (OMG), but high-quality evidence is limited in this regard. We examined whether treatment with prednisone and other immunosuppressants reduce the risk of generalization in OMG. MethodsThis is a retrospective study of consecutive adults with pure OMG who had a minimum 6 months of follow-up. The main outcome was the time to developing generalized symptoms. We used propensity scores to create matched data sets of patients treated with prednisone or any immunosuppressant vs controls. We also used unmatched models with inverse probability of treatment weights (IPTW) and variable exposure times. We used Cox proportional hazards model to estimate hazard ratio (HR) for generalization, comparing treated patients vs controls. ResultsA total of 154 patients were included, with a mean follow-up of 87.4 ± 73 months since onset. Forty-three (28%) were generalized, and mean time to generalization from diagnosis was 24.2 ± 24.1 months. Patients who received prednisone had lower risk of generalization than controls, with pooled HR 0.43 (95% CI 0.19-1.06) for the matched model, HR 0.46 (95% CI 0.21-0.89) for the IPTW model, and for HR 0.44 (95% CI 0.23-0.81) for the time-dependent exposure model. Patients who received any immunosuppressant had lower risk of generalization, with HR 0.30 (95% CI 0.11-0.77), 0.32 (95% CI 0.14-0.70), and 0.35 (95% CI 0.15-0.80) for the matched, IPTW, and IPTW-varying exposure models, respectively. DiscussionOur study provides evidence that steroidal and nonsteroidal immunosuppression in patients with OMG is associated with a reduced risk of developing generalized symptoms over time. This supports the early use of immunosuppression in this population. Classification of EvidenceThis study provides Class III evidence that treatment of OMG with corticosteroids or nonsteroidal immunosuppressants reduces the risk of generalization.

show abstract

Ocular myasthenia gravis and risk factors for developing a secondary generalisation: description of a Spanish series

Cited by 6 publications

References 29 publications

Advances and ongoing research in the treatment of autoimmune neuromuscular junction disorders

Advances and ongoing research in the treatment of autoimmune neuromuscular junction disorders

Clinical Characteristics Associated With Secondary Generalization in Patients With Ocular Myasthenia Gravis

Effect of Immunosuppression in Risk of Developing Generalized Symptoms in Ocular Myasthenia Gravis

Contact Info

Product

Resources

About