Clinical Predictors of Relapse in a Cohort of Steroid-Treated Patients With Well-Controlled Myasthenia Gravis

Abstract

ObjectiveDespite the high efficiency of glucocorticoids (GCs), ~18–34% patients with myasthenia gravis (MG) may experience relapses of the disease. Here, we aim to identify clinical factors related to relapses during steroid tapering or after withdrawal in MG patients who were well-managed on steroid monotherapy.MethodsWe conducted a retrospective study on 125 MG patients from the Xuanwu Hospital MG Trial Database. Patients were treated with corticosteroids and achieved minimal manifestation status (MMS) or better. Patients were divided into steroid reduction subset (N = 74) and steroid withdrawal subset (N = 51). Clinical characteristics and therapeutic data were compared between patients with disease relapse and those who maintained clinical remission at the last follow-ups. Cox proportional hazards regression models were used to identify risk factors of relapse in each subset.ResultsThirty-seven (29.6%) patients experienced relapses during the follow-up periods. Relapse during the steroid reduction was significantly associated with drug reducing duration (HR = 0.81, 95%CI 0.74–0.89, P < 0.001). Risk of relapse was augmented if the drug reducing duration was <11.5 months (HR 27.80, 95%CI 5.88–131.57, P < 0.001). Among patients who discontinued the steroids, those with onset symptoms of bulbar weakness (adjusted HR 3.59, 95%CI 1.19–10.81, P = 0.023) were more likely to experience relapse.ConclusionOur study demonstrated that patients could benefit from prolonged steroid-reducing duration to prevent disease relapse. Patients with bulbar weakness at disease onset should be proposed to take long-term steroids or other immunosuppressants.

Clinical and immune-related factors associated with exacerbation in adults with well-controlled generalized myasthenia gravis

“…Similar to other studies, most exacerbations (58.9%) occurred within the first 2 years of remission status being achieved ( 12 , 14 ). The MGFA classification was identified as an independent contributor to GMG relapse, suggesting that more severe clinical symptoms tend to be associated with a higher risk of exacerbation ( 14 ). We also found, in contrast to a previous study, that gender showed noassociation with exacerbation ( 2 ).…”

“…exhibiting GMG after disease onset. The results indicated that nearly 69.1% of adults with GMG experienced exacerbations, higher than the 33.7%-60.6% reported in previous literature, which may be related to higher severity and longer follow-up periods in the patients enrolled in our study (9,(12)(13)(14). Similar to other studies, most exacerbations (58.9%) occurred within the first 2 years of remission status being achieved (12,14).…”

“…The results indicated that nearly 69.1% of adults with GMG experienced exacerbations, higher than the 33.7%–60.6% reported in previous literature, which may be related to higher severity and longer follow-up periods in the patients enrolled in our study ( 9 , 12 – 14 ). Similar to other studies, most exacerbations (58.9%) occurred within the first 2 years of remission status being achieved ( 12 , 14 ). The MGFA classification was identified as an independent contributor to GMG relapse, suggesting that more severe clinical symptoms tend to be associated with a higher risk of exacerbation ( 14 ).…”

Short-term outcome prediction for myasthenia gravis: an explainable machine learning model

“…16 However, these studies mainly focused on specific subtypes, for example, anti-AChR antibody-positive generalized MG; or pre-defined conditions, for example, well-controlled patients treated with steroids, and were not suitable for a consecutive follow-up setting. 17,18 In the current study, we aimed to develop and validate a data-driven, machine learning (ML)based, predictive model to forecast the short-term outcome for MG. The advantages of this model include (1) accommodating three subtypes of MG (AChR, MuSK, negative); (2) being robust and replicable; (3) feasible for health professional evaluations.…”

“… 16 However, these studies mainly focused on specific subtypes, for example, anti-AChR antibody-positive generalized MG; or pre-defined conditions, for example, well-controlled patients treated with steroids, and were not suitable for a consecutive follow-up setting. 17 , 18 …”