Cookies on this website
We use cookies to ensure that we give you the best experience on our website. If you click 'Continue' we'll assume that you are happy to receive all cookies and you won't see this message again. Click 'Find out more' for information on how to change your cookie settings.

INTRODUCTION: In this study we determined the frequencies of antibodies (Abs) directed against muscle-specific kinase (MuSK) and lipoprotein receptor-related protein 4 (LRP4) in the sera of a South African cohort with acetylcholine receptor (AChR)-antibody-negative generalized MG and determined outcomes to therapies. METHODS: Sera negative by commercial AChR radioimmunoassay (RIA) were tested by MuSK RIA (n = 30; 2006-2012) and AChR, MuSK, and LRP4 RIA with or without cell-based assays (CBA) (n = 53; 2012-2015). RESULTS: AChR-Abs were detected in 4 of 53 and MuSK-Abs in 20 of 83 (24%) cases. Thirty-six of 53 (68%) were triple seronegative (triple-SNMG) for MuSK, AChR, and LRP4-Abs. When compared with triple-SNMG, individuals with MuSK-MG had a younger onset age (P = 0.008), a greater likelihood of African genetic ancestry (P = 0.008), and 4-fold higher odds of reaching MGFA grade IVB/V (P = 0.018), but were also 9-fold more likely to reach at least minimal manifestations status after ≥12 months of therapy (P = 0.003). CONCLUSIONS: Individuals with African genetic ancestry and severe bulbar/respiratory AChR-Ab-negative MG are likely to have MuSK-MG, but most respond favorably to maintenance immunotherapies. Muscle Nerve 54: 1023-1029, 2016.

Original publication

DOI

10.1002/mus.25154

Type

Journal article

Journal

Muscle Nerve

Publication Date

12/2016

Volume

54

Pages

1023 - 1029

Keywords

AChR, Africa, LRP4, MuSK, cell-based assay, myasthenia gravis, Adult, Autoantibodies, Cohort Studies, Female, Green Fluorescent Proteins, Humans, LDL-Receptor Related Proteins, Male, Middle Aged, Myasthenia Gravis, Odds Ratio, Receptor Protein-Tyrosine Kinases, Receptors, Cholinergic, South Africa