Chronic immune-mediated neuropathies are orphan diseases and mainly include chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy with persistent conduction blocks (MMN), and paraproteinemic neuropathies. The first step is crucial in characterizing these neuropathies on clinical, electrophysiological and sometimes immunochemical and pathological criteria, as the response to treatment is different according to the type of the neuropathy. The second step is to choose the best type and the regimen of the immunomodulatory treatment, which may be only a first-line short-term therapy, for example in relapsing or rapidly worsening CIDP, but frequently has to be a long-term therapy if the neurological condition needs it. Lastly, the selection of outcome measures for use in trials of treatment of chronic immune-mediated neuropathy is an emerging but essential concern for future trials.
