A study of the factors inducing the development of childhood-onset myasthenia gravis using CDR3 spectratyping analysis of the TCR repertoire

Myasthenia gravis (MG) is an autoimmune disease. AChR-specific autologous helper T (Th) cells are essential to the pathogenesis of MG. Factors correlated with the development of childhood-onset MG are unknown. In longitudinal studies, we found TCR Vβ 2/5.1/6/7 usage in the development or relapse phases, but not in the remission phase. We also found that TCR Vβ 8/9/13.1/15/18/20 usage persisted. The polyclonally expanded TCR Vβ 2/5.1/6/7 by CDR3 spectratyping was found to be associated with the development of disease. These data suggest that in patients with childhood-onset MG, stimuli such as superantigens induced by a preceding infection, which cause development of the polyclonal pattern in TCR Vβ families, play an important role in the development of the disease.