Extraocular muscle susceptibility to myasthenia gravis: unique immunological environment?

摘要: Extraocular muscle (EOM) is susceptible to neuromuscular junction disorders, in particular, myasthenia gravis (MG). While EOM physiological characteristics and the ocular motor system requirements contribute propensity of deficits observed among patients with MG, authors propose that have immunological features place muscles at risk for immune attack. Genomic profiling studies demonstrated genes associated response are differentially expressed EOM, particular differences both classical alternative complement-mediated pathways. Intrinsic complement regulators lower levels rodent junctions, which would put them injury occurs MG. In fact, systemic C inhibition experimental autoimmune MG (EAMG) induced by administration acetylcholine receptor (AChR) antibodies or immunization AChR will eliminate deposition junctions other skeletal muscle, but not EOM. Also, greater active passive EAMG several measures, suggesting lack puts risk. Among patients, serum antibody low, support concept more than junctions. These observations suggest inhibitory therapies may prove be particularly effective treatment myasthenia.