Thymectomy and Risk of Generalization in Patients with Ocular Myasthenia Gravis: A Multicenter Retrospective Cohort Study

Li, Huanhuan; Ruan, Zhe; Gao, Feng; Zhou, Hongyu; Guo, Rongjing; Sun, Chao; Xu, Quan; Lü, Qiang; Zhou, Yunyun; Zhao, Zhengwei; Yu, Liping; Wu, Songdi; Tao, Lei; Gao, Ting; Tang, Yujie; Li, Chunhong; Huo, Feiyan; Zhu, Ying; Sun, Jie; Tang, Baoli; Zhang, Min; Lü, Xiaodan; Li, Zhuyi; Chang, Ting

doi:10.1007/s13311-021-01129-z

Cited by 15 publications

(20 citation statements)

References 41 publications

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“…Consistent with previous studies, our data indicated that the presence of thymoma has been identified as a risk factor for the generalization of OMG [10,21]. Moreover, some researchers have investigated the encouraging role of thymectomy in reducing the risk of generalization [13,22]. However, thymectomy was excluded as a potential predictor by multiple Cox regression analysis in the present study.…”

Section: Discussionsupporting

confidence: 90%

Dynamic nomogram for predicting generalized conversion in adult-onset ocular myasthenia gravis

Cao

Gui

et al. 2022

Neurol Sci

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Purpose To explore the factors and risk mapping model of progression from ocular myasthenia gravis (OMG) to generalized myasthenia gravis (GMG) in adult-onset patients. Methods A retrospective, observational cohort study was performed for 435 OMG patients with onset age older than 14 years old. Multivariate Cox regression was used to identify the independent factors affecting generalized conversions that then were incorporated into the construction of the nomogram. Results Two hundred thirty-seven patients (54.5%) had transformed into GMG after a median of 1.1 years (range 0.1-–9.1 years). The 6-, 12-, and 24-month generalized conversion rates were 31.7%, 49.8%, and 65.4%, respectively. Multivariable analysis showed that the early-onset age, male sex, concomitant autoimmune diseases (AID), positive results of anti-acetylcholine receptor antibodies, repetitive nerve stimulation abnormalities, the presence of thymoma, and prednisone treatment were significantly associated with the generalized conversions (hazard ratio [HR] = 0.598, 0.686, 1.554, 1.541, 2.020, 2.510, and 0.556, respectively). A nomogram was established to predict the possibility of generalization-free survival (GFS) in adult-onset OMG patients, and the model demonstrated good predictive performance with a C-index of 0.736 (95% confidence interval 0.703 ~ 0.769). Moreover, subgroup analyses were performed based on the presence or absence of prednisone therapy, and the results indicated that prednisone therapy has better prevention of generalized conversions in male, non-thymoma patients, and patients without other AID. Conclusion A new predictive nomograph and web-based survival calculator we developed show favorable applicability and accuracy in predicting long-term GFS in adult-onset OMG patients.

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Section: Discussionsupporting

confidence: 90%

Dynamic nomogram for predicting generalized conversion in adult-onset ocular myasthenia gravis

Cao

Gui

et al. 2022

Neurol Sci

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“…It was claimed that the patients who develop GMG very early may be different from other patients with OMG. Several studies also followed the criterion of Oosterhuis ( 10 , 19 , 30 ).…”

Section: Discussionmentioning

confidence: 99%

“…Thus, preventing progression from ocular MG to generalized MG is crucial. In this regard, several studies have reported that early immunotherapy and thymectomy reduced the risk of generalization (4)(5)(6)(7)(8)(9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%

Development and Validation of a Nomogram for Predicting Generalization in Patients With Ocular Myasthenia Gravis

et al. 2022

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BackgroundThis study aims to develop and validate a nomogram for predicting 1- and 2-year generalization probabilities in patients with ocular myasthenia gravis (OMG).MethodsIn total, 501 eligible patients with OMG treated at seven tertiary hospitals in China between January 2015 and May 2019 were included. The primary outcome measure was disease generalization. A nomogram for predicting 1- and 2-year generalization probabilities was constructed using a stepwise Cox regression model. Nomogram performance was quantified using C-indexes and calibration curves. Two-year cumulative generalization rates were analyzed using the Kaplan−Meier method for distinct nomogram-stratified risk groups. The clinical usefulness of the nomogram was evaluated using decision curve analysis (DCA).ResultThe eligible patients were randomly divided into a development cohort (n=351, 70%) and a validation cohort (n=150, 30%). The final model included five variables: sex, onset age, repetitive nerve stimulation findings, acetylcholine receptor antibody test results, and thymic status. The model demonstrated good discrimination (C-indexes of 0.733 and 0.788 in the development and validation cohorts, respectively) and calibration, with good agreement between actual and nomogram-estimated generalization probabilities. Kaplan−Meier curves revealed higher 2-year cumulative generalization rates in the high-risk group than that in the low-risk group. DCA demonstrated a higher net benefit of nomogram-assisted decisions compared to treatment of all patients or none.ConclusionThe nomogram model can predict 1- and 2-year generalization probabilities in patients with OMG and stratified these patients into distinct generalization risk groups. The nomogram has potential to aid neurologists in selecting suitable patients for initiating immunotherapy and for enrolment in clinical trials of risk-modifying treatments.

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“…Several studies reported that early thymectomy in OMG patients was associated with lower generalization than in patients without thymectomy [20][21][22]. Recently, we reported that thymectomy significantly reduced the generalization risk in OMG patients with and without thymoma or hyperplasia [23]. To reduce the influence of this strong confounding factor to the largest extent, we adopted the IPTW approach and adjusted thymectomy in the multivariable Cox regression model.…”

Section: Discussionmentioning

confidence: 99%

Association of immunosuppression treatment with generalization among patients with ocular myasthenia gravis: A propensity score analysis

Ruan

Guo

Zhou

et al. 2022

Euro J of Neurology

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Background To analyze disease generalization in patients with ocular myasthenia gravis (OMG) treated with immunosuppression compared with patients without immunosuppression treatment. Methods In this retrospective cohort study, we analyzed data from patients with OMG at seven medical centers in China from January 1, 2015 to May 1, 2019 and compared disease generalization in patients (treated with immunosuppression vs. not treated) within 2 years of disease onset using raw and inverse probability of treatment weighting (IPTW) analyses. Results In the study population of 813 patients with OMG, 425 (52.3%) with immunosuppression had a mean (SD) onset age of 50.0 (15.1) years, and 188 (44.2%) were women. The remaining 388 (47.7%) patients were not immunosuppressed (mean age, 48.4 [15.0] years; 185 [47.7%] women). Disease generalization developed in 122 (31.4%) and 37 (8.7%) patients in the non‐immunosuppression and immunosuppression groups, respectively. Relative to non‐immunosuppression, immunosuppression was associated with a lower risk of generalization in a multivariable‐adjusted Cox model (hazard ratio [HR] 0.27; 95% confidence interval [CI] 0.18–0.40; p < 0.001) and IPTW‐weighted Cox model (HR 0.28; 95% CI 0.19–0.42; p < 0.001). In sensitivity analyses, longer duration of immunosuppression was associated with a lower risk of generalization (HR 0.90 for every 1‐month increase; 95% CI 0.87–0.92; p < 0.001; IPTW‐adjusted). Combination therapy with steroids and non‐steroidal immunosuppressants showed superior efficacy in reducing the risk of generalization (HR 0.14; 95% CI 0.07–0.26; p < 0.001). Conclusion Immunosuppression significantly reduced the 2‐year risk of generalization in patients with OMG.

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Thymectomy and Risk of Generalization in Patients with Ocular Myasthenia Gravis: A Multicenter Retrospective Cohort Study

Cited by 15 publications

References 41 publications

Dynamic nomogram for predicting generalized conversion in adult-onset ocular myasthenia gravis

Dynamic nomogram for predicting generalized conversion in adult-onset ocular myasthenia gravis

Development and Validation of a Nomogram for Predicting Generalization in Patients With Ocular Myasthenia Gravis

Association of immunosuppression treatment with generalization among patients with ocular myasthenia gravis: A propensity score analysis

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