Lu sur http://onlinelibrary.wiley.com/wol1/doi ... 4/abstract
Traduction disponible directement en cliquant en bas à droite de ce message sur notre forum
Characteristics Of acetylcholine-receptor-antibody–negative myasthenia gravis in a South African cohort
Saif Huda MRCP1, Mark R. Woodhall PhD1, Angela Vincent FRS1 andJeannine M. Heckmann PhD2,*
Version of Record online: 13 OCT 2016
Muscle Nerve, 2016
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.
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).
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).
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.