Prognosis of Ocular Myasthenia Gravis in an Argentinian Population

Aguirre, Florencia; Villa, Andrés

doi:10.1159/000487132

Cited by 22 publications

(28 citation statements)

References 23 publications

(41 reference statements)

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“…Seropositivity for AChR-Ab as a predictor of generalization is well-documented [ 5 , 7 , 9 – 11 , 13 , 20 , 28 – 30 ]. Galassi et al surveyed a cohort of 175 patients with OMG and compared the outcome of those patients with antibodies to AChR or to MuSK (muscle-specific tyrosine kinase) with those seronegative for both of these antibodies.…”

Section: Discussionmentioning

confidence: 99%

“…Several retrospective studies have reported risk factors associated with generalization, however, these studies included patients on immunosuppression therapy or those undergoing thymectomy, which may reduce the generalization risk [2,[5][6][7][8][9][10][11][12][13][14]. Thus, a study exploring risk factors in non-immunosuppressed and nonthymectomized patients is required to adequately control for these confounding factors.…”

Section: Introductionmentioning

confidence: 99%

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Risk Factors for Generalization in Patients with Ocular Myasthenia Gravis: A Multicenter Retrospective Cohort Study

et al. 2021

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Introduction Many patients with ocular myasthenia gravis (OMG) progress to generalized disease within the first 2 years of the onset of ocular symptoms. Several retrospective studies have identified risk factors associated with generalization, however these studies included patients on immunosuppression therapy or those undergoing thymectomy, which may reduce the generalization risk. In this study we explored the risk factors for generalization in non-immunosuppressed and non-thymectomized patients with OMG. Methods Data from patients with OMG treated at seven tertiary hospitals in China were retrospectively reviewed. Clinical characteristics, including sex, age at onset, symptoms at onset, comorbid autoimmune diseases, neostigmine test response, repetitive nerve stimulation (RNS) findings, presence of serum anti-acetylcholine receptor antibody (AChR-Ab), and thymic status based on radiological and pathological studies, were collected. The main outcome measure was disease generalization. The follow-up period was defined as the date of ocular symptom onset to the date of confirmation of generalization or immunotherapy initiation, or last follow-up (defined as 60 months). The Cox proportional hazards model was used to assess the risk factors for generalization. Results Overall, 572 patients (269 women) were eligible for inclusion in the analysis, of whom 144 developed generalization. The mean (standard deviation) onset age was 45.5 (19.8) years, and the median (interquartile range) follow-up period was 14.5 (7.0–47.3) months. Multivariable Cox regression analysis demonstrated that both early-onset (adjusted hazard ratio [aHR] 5.34; 95% confidence interval [CI] 1.64–17.36; p = 0.005) and late-onset (aHR 7.18; 95% CI 2.22–23.27; p = 0.001) in adulthood, abnormal RNS findings (aHR 3.01; 95% CI 1.97–4.61; p < 0.001), seropositivity for AChR-Ab (aHR 2.58; 95% CI 1.26–5.26; p = 0.01), and thymoma (aHR 1.62; 95% CI 1.05–2.49; p = 0.03) were independently associated with increased risk of generalization. Conclusion The risk of generalization increased significantly in patients with adult-onset OMG, abnormal RNS findings, seropositivity for AChR-Ab, and thymoma, suggesting that these risk factors may predict OMG generalization. Supplementary Information The online version contains supplementary material available at 10.1007/s40120-021-00292-x.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Risk Factors for Generalization in Patients with Ocular Myasthenia Gravis: A Multicenter Retrospective Cohort Study

et al. 2021

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“…Several studies showed that early immunosuppressive treatment of MG reduces the probability of MC occurrence and recurrence ( 30 ). To date, several retrospective studies showed that the use of oral steroids in ocular MG could reduce the probability of progression to generalized MG ( 31 , 32 ). These results suggest that the use of immunosuppressive therapy in the patients with MG may not only improve symptoms, but also act as a disease modifier to prevent the progression of the disease course in the patients with MG.…”

Section: Discussionmentioning

confidence: 99%

Patients With Myasthenia Gravis With Acute Onset of Dyspnea: Predictors of Progression to Myasthenic Crisis and Prognosis

et al. 2021

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Background: Life-threatening myasthenic crisis (MC) occurs in 10–20% of the patients with myasthenia gravis (MG). It is important to identify the predictors of progression to MC and prognosis in the patients with MG with acute exacerbations.Objective: This study aimed to explore the predictors of progression to MC in the patients with MG with acute onset of dyspnea and their short-term and long-term prognosis.Methods: This study is a retrospective cohort study. We collected and analyzed data on all the patients with MG with acute dyspnea over a 10-year period in a single center using the univariate and multivariate analysis.Results: Eighty-six patients with MG were included. In their first acute dyspnea episodes, 36 (41.9%) episodes eventually progressed to MC. A multivariate analysis showed that the early-onset MG (adjusted OR: 3.079, 95% CI 1.052–9.012) and respiratory infection as a trigger (adjusted OR: 3.926, 95% CI 1.141–13.510) were independent risk factors for the progression to MC, while intravenous immunoglobulin (IVIg) treatment prior to the mechanical ventilation (adjusted OR: 0.253, 95% CI 0.087–0.732) was a protective factor. The prognosis did not significantly differ between the patients with and without MC during the MG course, with a total of 45 (52.3%) patients reaching post-intervention status better than minimal manifestations at the last follow-up.Conclusion: When treating the patients with MG with acute dyspnea, the clinicians should be aware of the risk factors of progression to MC, such as early-onset MG and respiratory infection. IVIg is an effective treatment. With proper immunosuppressive therapy, this group of patients had an overall good long-term prognosis.

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“…In light of this, several studies have tried to identify risk factors of secondary generalization to avoid unnecessary adverse effects caused by immunosuppressive medications (9,10,14,15). Thus far, however, it has been challenging to stratify patients with OMG into subgroups with different risk levels of secondary generalization.…”

Section: Discussionmentioning

confidence: 99%

Prognostic predictors of remission in ocular myasthenia after thymectomy

Xiang¹,

Zhou

Hu³

et al. 2020

J Thorac Dis

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Background: Identifying prognostic predictors can assist in making clinical decisions. This study aimed to identify the potential predictors of remission in patients with ocular myasthenia gravis (OMG) after thymectomy. Methods: OMG patients who had thymectomy between 2011 and 2017 were reviewed retrospectively.Clinical outcomes were assessed according to the Myasthenia Gravis Foundation of America Post-Intervention Status (MGFA-PIS). Kaplan-Meier analysis was used to estimate the cumulative probability of complete stable remission (CSR). Univariate and multivariable analyses with Cox proportional hazards regression were used to identify predictors of CSR.Results: Fifty-one patients (23 male, 28 female) with a median age at OMG onset of 40 (range, 5-79) years were eligible for inclusion. Patients with thymomatous OMG (n=9) had a statistically older median age at disease onset [61 (range, 32-78) vs. 33.5 (range, 5-79) years, P=0.001], shorter duration from disease onset to thymectomy [3 (range, 2-24) vs. 10 (range, 1-132) months, P=0.004], and a higher rate of postoperative complication (44.4% vs. 9.5%, P=0.025), compared with non-thymomatous OMG (n=42). The estimated cumulative probability of CSR in the whole cohort was 41.8% (95% CI, 28.6-58.2%) 5 years after surgery.Age at onset of 40 years or younger (P=0.00016), female sex (P=0.069), and thymic hyperplasia (P=0.0061) were potential predictors under univariate analysis. However, only age at onset of 40 years or younger (HR: 4.117, 95% CI, 1.177-14.399, P=0.027) remained significant after multivariable analysis.Conclusions: CSR could be achieved in about 40% of OMG patients 5 years after thymectomy and is likely to be predicted by age at onset of 40 years or younger.

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Prognosis of Ocular Myasthenia Gravis in an Argentinian Population

Cited by 22 publications

References 23 publications

Risk Factors for Generalization in Patients with Ocular Myasthenia Gravis: A Multicenter Retrospective Cohort Study

Risk Factors for Generalization in Patients with Ocular Myasthenia Gravis: A Multicenter Retrospective Cohort Study

Patients With Myasthenia Gravis With Acute Onset of Dyspnea: Predictors of Progression to Myasthenic Crisis and Prognosis

Prognostic predictors of remission in ocular myasthenia after thymectomy

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