Do early prednisolone and other immunosuppressant therapies prevent generalization in ocular myasthenia gravis in Western populations: a systematic review and meta-analysis

Abstract

Background: The majority of ocular myasthenia gravis (OMG) patients will progress to generalized myasthenia gravis (GMG), usually within 2 years of disease onset. The aim of this meta-analysis was to evaluate the effect of early prednisolone and other immunosuppressants therapy on the generalization rate in OMG patients. Methods: We searched the CENTRAL, EMBASE, and MEDLINE databases via the Ovid SP database for all relevant publications on 16 July 2018. Results: Eight studies comprising a total of 547 participants were included in our meta-analysis. Compared with pyridostigmine treatment, prednisolone and other immunosuppressants therapy produced an odds ratio (OR) for the development of GMG of 0.19 [95% confidence interval (CI), 0.11–0.30; I2 = 37%], indicating that early prednisolone and other immunosuppressants therapy reduced the generalization rate in OMG by 81%. Conclusions: Early prednisolone and other immunosuppressants therapy can significantly reduce the risk of generalization in OMG patients, and should be considered in newly diagnosed OMG patients. Due to the inclusion of retrospective studies, this noted effect might have been related to corticosteroids, especially when immunosuppressants used at low dosages and in mild disease. Additionally, the data derived from Western populations, thus a prospective randomized controlled trial (RCT) is warranted to confirm this effect of early prednisolone and other immunosuppressants therapy on OMG generalization both in Western and Asian populations.

Independent risk factors for myasthenic crisis and disease exacerbation in a retrospective cohort of myasthenia gravis patients

“…We analyzed this parameter to understand if achieving the proposed treatment target is associated with a reduced risk for MC [ 17 ]. Treatment strategies were previously suggested to affect the course of disease in MG. As such, a recent meta-analysis suggested that cortisone treatment reduces the risk for secondary generalization for MG patients with ocular manifestation [ 28 ]. Thymectomy is also evidenced to improve clinical readouts over a 3-year time span as demonstrated in a recent, randomized, controlled trial [ 19 , 20 ].…”

Risk Factors for Generalization in Patients with Ocular Myasthenia Gravis: A Multicenter Retrospective Cohort Study

“…Our study has several strengths. First, most previous studies included immunosuppressed patients, potentially affecting the reported outcomes [ 1 , 2 , 5 – 14 , 20 , 28 ], as immunosuppression may reduce the risk of generalization [ 3 , 4 ]. Excluding this patient group may increase study validity.…”

“…Early immunosuppression therapy with steroids and other non-steroidal immunosuppressants may prevent the progression to GMG [ 3 , 4 ]. However, not all patients will ultimately develop generalization, with approximately 20% of patients not progressing to generalized disease irrespective of immunosuppressive therapy.…”

Pediatric Ocular Myasthenia Gravis: A Review

“…The factors which are proposed to be associated with generalization are older age at onset, RNS abnormalities in facial nerve, AChR antibody positivity, less use of early immunosuppressive therapy, presence of thymoma, and shorter disease duration. [97][98][99][100] However, these studies were retrospective and the patients were treated with immunosuppressive agents, which might have confounded the association results due to their disease modifying effects. 74 Though a study to evaluate the risk factors in nonimmunosuppressed patients is required; it might not be ethically justified.…”