Increased complement consumption in musk-antibody-positive myasthenia gravis patients

ABSTRACT

To investigate the activation of different complement pathways in myasthenia gravis [MG] subtypes. Levels of complement breakdown products for different complement pathways were measured using ELISA in sera of acetylcholine receptor antibody [AChR-Ab]-positive [n = 21], muscle-specific receptor tyrosine kinase [MuSK]-Ab-positive [n = 23] and seronegative generalized MG patients [n = 21] and healthy controls [n = 22]. Levels of factor Bb [FBb], the breakdown product of factor B, and C4d, the breakdown product of C4, were measured to evaluate the activity of the alternative and classical complement pathways, respectively. Serum iC3b levels were analyzed to assess total complement activity. The results were expressed as OD values. MuSK-Ab-positive MG patients had a significantly higher mean concentration of serum FBb [0.638] than other MG subtypes [0.446 for AChR-Ab-positive, 0.537 for seronegative MG patients] and healthy controls [0.434] [p = 0.045]. Mean serum iC3b [1.549-1.780] and C4d [0.364-0.395] levels were comparable among the groups. Our results suggest that MuSK-Ab-positive MG patients might have a complement-activating serum factor and the alternative complement pathway might be involved in the pathogenesis of the disease