Treatment of chronic Guillain-Barre syndrome

  Chronic Guillain-Barre syndrome (CIDP) is an acquired immune-mediated inflammatory demyelinating disease of peripheral nerves and is one of the most common and treatable chronic polyneuropathies available. Numerous randomized controlled trials (RCTs) have shown that glucocorticoids, plasma exchange, and intravenous infusion of gammaglobulin (IVIg) are the first-line treatments for CIDP and should be preferred in the vast majority of cases. The efficacy of these three treatments is generally comparable, with IVIg (Grade A recommendation) or glucocorticoids (Grade C recommendation) being recommended for both sensory and motor CIDP, while IVIg should be preferred for pure motor CIDP, with PE (Grade A recommendation) considered when both are ineffective. For patients on glucocorticoids, starting adequate doses is recommended, but slow tapering to discontinuation is advocated at about six months, usually no more than one year. For elderly patients who cannot tolerate the side effects of glucocorticoids and plasma exchange, long-term, intermittent application of IVIg can significantly improve the prognosis of patients, with infusion intervals of 1 month to 3 months depending on economic conditions.  If these measures are not tolerated, subcutaneous gammaglobulin may be tried. For patients with refractory CIDP who have failed or had poor results with initial therapy, other treatments should be tried, or a combination of immunosuppressive or immunomodulatory agents, including azathioprine, cyclosporine A, cyclophosphamide, mycophenolate, methotrexate, interferon, and rituximab. The efficacy of cyclophosphamide, azathioprine, and cyclosporine A has been confirmed in several studies, but side effects should be fully considered. Methotrexate and mycophenolate are currently highly recommended second-line agents. Rituximab is gaining ground in the treatment of autoimmune diseases year by year, and CIDP is no exception and could be a promising treatment. However, they are expensive and carry the risk of fatal progressive multifocal leukoencephalopathy (PML), so the pros and cons should be critically evaluated before application.