Ocular presentation of myasthenia gravis: A natural history cohort

Kamarajah, Sivesh K.; Sadalage, Girija; Palmer, Jonathan; Carley, Helena; Maddison, Paul; Sivaguru, Arul

doi:10.1002/mus.25971

Cited by 24 publications

(35 citation statements)

References 21 publications

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“…Given that older age at onset has been demonstrated to correlate with higher risk of generalization [16,17], the lower conversion rate in this study than in Caucasian population further highlights the potential role of ethnicity in the prevalence of OMG. Several studies suggested that factors such as age at onset, AChR-Ab titers, positive rate of RNS, thymoma and immunosuppressive therapy were associated with generalization [6][7][8][9].…”

Section: Predictors Of Conversion To Gmgmentioning

confidence: 60%

“…Prior studies showed that the conversion rate of juvenile-onset OMG fluctuated between 23 and 43% [3,21], while that of adultonset OMG between 31 and 49% [4,22]. Kamarajah and Wang reported that late-onset was correlated with higher risk of generalization [16,17]. In another study, patients were inclined to convert to GMG with increasing age at onset [2].…”

Section: Predictors Of Conversion To Gmgmentioning

confidence: 99%

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Prediction of generalization of ocular myasthenia gravis under immunosuppressive therapy in Northwest China

et al. 2020

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Background It is well demonstrated that immunosuppressants can reduce, but not eliminate the risk of generalized development in ocular myasthenia gravis (OMG). In this study, we aimed to explore the predictive factors of generalized conversion of OMG patients who received immunosuppressive treatments. Methods OMG patients under immunosuppressive treatments in Tangdu Hospital from June 2008 to June 2012 were retrospectively reviewed. Baseline clinical characteristics were documented. Patients were followed up regularly by face-to-face interview and the main outcome measure was generalized conversion. The logistic regression analysis was performed to determine the predictive factors of generalization of OMG. Results Two hundred twenty-three eligible OMG patients completed the final follow-up visit and 38 (17.0%) progressed to generalized MG (GMG) at a median time to generalization of 0.9 year. Patients with adult onset and positive repetitive nerve stimulation (RNS) of facial or axillary nerve had higher conversion rate than those with juvenile onset and negative RNS (p = 0.001; p = 0.019; p = 0.015, respectively). Adult-onset patients converted earlier than juvenile-onset OMG patients (p = 0.014). Upon multivariate logistic regression analysis, age of onset (Odds ratio [OR] 1.023, 95% confidence interval [CI] 1.006–1.041, p = 0.007) and positive facial nerve RNS (OR 2.826, 95%CI 1.045–5.460, p = 0.038) were found to be positively associated with generalized development. Moreover, an obviously negative association was found for disease duration (OR 0.603, 95%CI 0.365–0.850, p = 0.019). Conclusions Age of onset, disease duration and facial nerve RNS test can predict generalized conversion of OMG under immunosuppressive therapy. Adult-onset, shorter disease duration and facial nerve RNS-positive OMG patients have a higher risk of generalized development.

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Section: Predictors Of Conversion To Gmgmentioning

confidence: 60%

Section: Predictors Of Conversion To Gmgmentioning

confidence: 99%

Prediction of generalization of ocular myasthenia gravis under immunosuppressive therapy in Northwest China

et al. 2020

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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%

“…Therefore, identifying the predictors of CSR after thymectomy might be beneficial and may allow us to better counsel patients about formulating a treatment plan. A l t h o u g h v e r y f e w s t u d i e s h a v e s p e c i f i c a l l y investigated the role of thymectomy in OMG, a metaanalysis of 26 studies with a median eligible sample size of 16 (range, 3-105) showed that thymectomy is beneficial in terms of inducing remission in patients with OMG (9). Of these studies, one with a sample size of 61 patients demonstrated that thymectomy could lead to an apparent cure (symptoms free without medications) in 51% of patients with OMG (16).…”

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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“…It was reported the conversion rate of juvenile-onset OMG patients fluctuated between 23% and 43% [3,17], while that of adult-onset OMG patients fluctuated between 31% and 49% [4,18]. Kamarajah and Wang suggested late-onset were positively related with generalization [19,20]. Mark et al [2] also reported patients were inclined to converted to GMG with increasing onset age.…”

Section: Discussionmentioning

confidence: 99%

Prediction of myasthenia gravis generalization under immunosuppressive therapy in Northwest China

Ding

Zhao

Ren

et al. 2020

Preprint

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Objective It is well demonstrated that immunosuppressants can reduce, but not eliminate the risk of generalized development in ocular myasthenia gravis (OMG). Herein, we explored the potential predictors of conversion of OMG patients receiving immunosuppressive treatments. Methods OMG patients under immunosuppressive treatments in Tangdu Hospital from June 2008 to June 2012 were retrospectively reviewed. Baseline clinical characteristics were documented. Patients were followed up regularly by face-to-face interview to evaluate clinic status and treatment efficacy. Main outcome measure was generalized conversion and the relative data were collected and analyzed by logistic regression. Results 223 eligible OMG patients completed the final follow-up visit and 38 (17.0%) progressed to generalized MG (GMG) at a median interval of 0.9 year. Patients with adult onset and positive repetitive nerve stimulation (RNS) of facial or axillary nerve had higher conversion rate than those with juvenile onset and negative RNS ( p = 0.001; p = 0.019; p = 0.015, respectively). Adult-onset patients converted earlier than juvenile-onset OMG patients ( p = 0.014). Upon multivariate logistic regression analysis, age of onset (Odds ratio [OR] 1.023, 95% confidence interval [CI] 1.006–1.041, p = 0.007) and positive facial nerve RNS (OR 2.826, 95%CI 1.045–5.460, p = 0.038) were found to be positively associated with generalized development. Moreover, an obviously negative association was found for disease duration (OR 0.603, 95%CI 0.365–0.850, p = 0.019). Conclusions Age of onset, disease duration and facial nerve RNS test can predict conversion of OMG under immunosuppressive treatment. Adult-onset, shorter disease duration and facial nerve RNS-positive patients have a higher risk of generalized development.

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Ocular presentation of myasthenia gravis: A natural history cohort

Abstract: As patients were steroid-naive before generalization, our cohort approximated the natural history of the condition. Muscle Nerve 57: 622-627, 2018.

Cited by 24 publications

References 21 publications

Prediction of generalization of ocular myasthenia gravis under immunosuppressive therapy in Northwest China

Prediction of generalization of ocular myasthenia gravis under immunosuppressive therapy in Northwest China

Prognostic predictors of remission in ocular myasthenia after thymectomy

Prediction of myasthenia gravis generalization under immunosuppressive therapy in Northwest China

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