Long-term Outcomes of Steroid Dosing Regimens and Withdrawal in Myasthenia Gravis: A Single-center Cohort Study
Yangyu Huang1, Ying Tan1, Jingwen Yan1, Jiayu Shi1, Yuzhou Guan1
1Department of Neurology, Peking Union Medical College and Chinese Academy of Medical Sciences, Peking Union Medical College Hospital
Objective:
To investigate the impact of different steroid regimens and steroid withdrawal in MG with steroid monotherapy.
Background:
Corticosteroids (steroids) are the first-line immunotherapy for myasthenia gravis (MG). However, the optimal steroid dosing regimen and the effects of discontinuing immunotherapy remain unclear.
Design/Methods:
This is a cohort study based on a single-center prospective registry, including patients who achieved sustained minimal manifestations or better status with steroid monotherapy. The primary outcome was relapse. Group-based trajectory modeling (GBTM) identified distinct steroid regimens, and Cox proportional hazards models and propensity score matching (PSM) assessed the impact of regimens and steroid withdrawal.
Results:
In 209 patients (median follow-up 54.0 months), 113 (54.1%) experienced relapse, and 65 (31.1%) discontinued steroids. GBTM identified three regimens: "High Start, Fast Taper" (Regimen 1), "Low Start, Slow Taper" (Regimen 2), and "Moderate Start, Gradual Taper" (Regimen 3). Compared to Regimen 1, Regimen 2 (HR = 0.15, 95% CI 0.07-0.32, p < 0.001) and Regimen 3 (HR = 0.28, 95% CI 0.15-0.53, p < 0.001) had significantly lower relapse risks. In patients who withdrew steroids, the median time to relapse was 7.0 (3.0, 22.0) months. After PSM, the steroid withdrawal group had a significantly higher 1-year relapse risk (HR = 1.58, 95% CI 1.03-2.44, p = 0.039) compared to the low-dose maintenance group.
Conclusions:
High initial dose, rapid-tapering regimen increases relapse risk, while steroid withdrawal also significantly raises relapse risk compared to low-dose maintenance. Therefore, caution is advised when rapid tapering or discontinuing steroids in patients on steroid monotherapy.
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