New insights into the pathogenesis and treatment of autoimmune neuromuscular disorders
By Paolo Ripellino
Prof. Hugh Willison from Glasgow discussed the pathogenesis of immune-mediated neuropathies, focusing especially on GBS. New inputs are the uptake of GM1 antibodies into spinal cord/motor roots, glycoarray techniques to detect antibodies against gangliosides -complexes, and neuropathies related to immune check-point inhibitors. The role of complement in immune-mediated diseases and upcoming treatments were discussed. Some diseases (CIDP variants related to Neurofascin antibodies, Musk + MG) may respond to Rituximab.
Prof. Bart Jacobs from Rotterdam presented GBS variants, that are differently represented around the globe (Brain publication from IGOS study). Only 50% of GBS have increased protein level in the CSF in the acute phase. Current dilemmas in GBS are: treatment related fluctuations; if/how to treat mild GBS or Mller Fisher; if to provide a second course of IVIg in severe cases of GBS. This last issue has been investigated in a Dutch RCT with surprisingly negative results (no benefits, higher risk of systemic complications). Current diagnostic criteria for CIDP were discussed, highlitening the risks of misdiagnosis (20% over – or underdiagnosis).
Prof. Stenzel from Berlin presented clinical and pathological details of myositis, discussing variants associated to specific autoantibodies. A special focus was made on immune-mediated necrotizing myopathy (IMMN), sporadic IBM and anti-synthetase syndrome.
Prof. Gilhus provided an update on Myasthenia, discussing subtypes that may have slightly different treatment strategies (see review in NEJM 2016). A special focus was provided on thymectomy, based on recent RCT (MGTX; long-term data are now available).