Journal
MUSCLE & NERVE
Volume 63, Issue 6, Pages 824-830Publisher
WILEY
DOI: 10.1002/mus.27233
Keywords
B‐ cell activating factor (BAFF); low dose; muscle specific tyrosine kinase (MuSK); myasthenia gravis; rituximab
Categories
Funding
- National Key Research and Development Program of China [2016YFC0901504]
- National Natural Science Foundation of China [81901279]
- Science and Technology Commission of Shanghai Municipality [18140900303]
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The study demonstrated that low-dose rituximab had clinical efficacy in MuSK-MG patients, leading to a decrease in MuSK antibody titers and an increase in serum BAFF levels. Serum BAFF levels were negatively correlated with B-cell counts and clinical severity.
Introduction/Aims The study aims to investigate the short-term efficacy of low-dose rituximab and its effect on immunological biomarker levels in myasthenia gravis (MG) patients with antibodies against muscle-specific tyrosine kinase (MuSK-MG). Methods Twelve MuSK-MG patients were enrolled in this prospective, open-label, self-controlled pilot study. Clinical severity was evaluated at baseline and 6 mo after a single rituximab treatment (600 mg). B lymphocyte subtypes, MuSK antibody titers, together with levels of immunoglobulins, serum B-cell activating factor (BAFF), a proliferation-inducing ligand (APRIL), soluble CD40L, and four exosomal microRNAs were evaluated. A correlation matrix to reveal pairwise relationships among above variables was also generated. Results The single rituximab treatment significantly lowered the clinical severity scores and reduced daily dosage of prednisone (P = .032) at 6 mo. MuSK antibody titers decreased (P = .035) without significant changes in immunoglobulin levels. Serum BAFF level increased (P = .010), which negatively correlated with the percentages of B cells in lymphocytes as well as clinical severity. Additionally, serum exosomal miR-151a-3p showed a reduction of 28.1% (P = .031). Discussion We confirmed the clinical efficacy of low-dose rituximab in MuSK-MG, accompanied by a decrease in MuSK antibody titers and an increase in serum BAFF. Serum BAFF levels negatively correlated with B-cell counts as well as clinical severity.
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