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J Korean Med Sci. 2026 May 25;41(20):e151. doi: 10.3346/jkms.2026.41.e151.

ABSTRACT

BACKGROUND: There is limited knowledge regarding the real-world treatment status of muscle-specific tyrosine kinase antibody-positive myasthenia gravis (MuSK-MG) patients in clinical practice. The aim of this study was to analyze the overall treatment patterns and outcomes of MuSK-MG in Korea.

METHODS: In this single-center cohort study, we retrospectively included patients diagnosed with MuSK-MG. We analyzed their demographic features, clinical characteristics, treatment patterns focusing on corticosteroids (CSs), non-steroidal immunosuppressants (NSISs), and rituximab, and treatment outcomes. Specifically, we investigated the achievement of minimal manifestation status with a prednisolone dose 5 mg/day or below (MM-5).

RESULTS: The study included 30 patients with MuSK-MG, predominantly female (93.3%). The average age at time of diagnosis was 43.9 ± 16.0 years, with the onset age of 42.3 ± 16.2 years. All patients initially received CSs, and 24 patients (80%) were treated with NSISs. Tacrolimus was the most frequently prescribed NSIS, used in 70.8% of cases, followed by azathioprine (62.5%). All 10 patients who received rituximab therapy showed clinical improvement, with 5 patients (50%) achieving MM-5.

CONCLUSION: This study summarized the treatment patterns and outcomes of patients with MuSK-MG in real-world clinical settings. As patients who received rituximab demonstrated a favorable prognosis, early administration of rituximab may be an effective treatment strategy for MuSK-MG, potentially reducing the adverse effects associated with long-term use of CSs and NSISs. Rituximab should be considered early in MuSK-MG patients who show insufficient response to initial immunosuppressive treatment.

PMID:42190696 | DOI:10.3346/jkms.2026.41.e151


Source: https://pubmed.ncbi.nlm.nih.gov/4219069 ... 0&v=2.20.0