4.5 Article

Thymectomy in ocular myasthenia gravis-prognosis and risk factors analysis

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ORPHANET JOURNAL OF RARE DISEASES
卷 17, 期 1, 页码 -

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BMC
DOI: 10.1186/s13023-022-02454-y

关键词

Conversion; Ocular myasthenia gravis; Remission; Thymectomy; Thymoma

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This study aimed to explore the clinical predictors of post-thymectomy OMG prognosis. The study found that RNS-positivity and histotype B2/B3 thymoma were independent predictors of conversion to GMG in OMG patients after thymectomy, while thymic hyperplasia and stage I thymoma predicted CSR.
Background Several retrospective studies have identified risk factors associated with ocular myasthenia gravis (OMG) generalization in non-surgical patients. However, the outcomes of OMG after thymectomy have not been investigated fully. This study aimed to explore the clinical predictors of post-thymectomy OMG prognosis. Methods We performed a retrospective review of OMG patients who underwent thymectomy at our institution from January 2012 to December 2021. Kaplan-Meier and Cox proportional hazard regression analyses were used to evaluate associations between clinical features and prognosis. The main outcome measures were OMG conversion, complete stable remission (CSR), and clinical improvement. Results Fifty-eight patients were identified for conversion analysis. Thirteen (22.4%) developed generalized myasthenia gravis (GMG) at a median time of 12.7 (3-37.3) months from symptom onset. Repetitive nerve stimulation (RNS)-positivity was associated with increased risk of conversion to GMG (P = 0.002). Patients with histotype B2/B3 thymoma showed a higher risk of conversion (P = 0.002) than did patients with hyperplasia and AB/B1 thymoma. Fifty-two patients fulfilled the criteria for CSR and improvement. Sixteen (30.8%) achieved CSR at a median time of 28.7 (15-54) months after thymectomy. Fifteen (28.8%) showed clinical improvement at last follow up. Patients who achieved CSR showed a younger age of onset (P = 0.022), lower percentage of acetylcholine receptor antibody-seropositivity (P = 0.029). Histologically, patients with thymic hyperplasia and stage I thymoma showed a higher chance of CSR (P = 0.010) than did patients with stage II/III thymoma. Multivariate analysis revealed that RNS-positivity (hazard ratio [HR] 6.007, P = 0.021) and histotype B2/B3 thymoma (HR 4.611, P = 0.048) were associated with OMG conversion. Thymic hyperplasia and stage I thymoma (HR 0.300, P = 0.026) were associated with OMG CSR after thymectomy. Conclusion For OMG patients after thymectomy, RNS-positivity and histotype B2/B3 thymoma are independent predictors of conversion to GMG. On the other hand, thymic hyperplasia and stage I thymoma independently predict CSR.

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