Principles of Therapeutic Apheresis in Neurological Disease

BACKGROUND: Therapeutic plasma exchange (TPE) is a well-known apheresis technology since many years and is available worldwide. Myasthenia gravis is one of the first neurological diseases successfully treated with TPE. TPE is also frequently applied in acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barré syndrome). Both neurological disorders are immunologically mediated and might cause life-threatening symptoms in patients. SUMMARY: There is a large body of evidence from many randomized controlled trials (RCTs) that the application of TPE in myasthenia gravis crisis or in acute Guillain-Barré syndrome is effective and safe. Thus, TPE is recommended as first-line therapy with a grade 1A recommendation during the critical course of these neurological diseases. Even chronic inflammatory demyelinating polyneuropathies characterized by complement-fixing autoantibodies to myelin are successfully treated with TPE. The plasma exchange reduces inflammatory cytokines, complements activating antibodies, and leads to an improvement of neurological symptoms. TPE is no standalone treatment but often combined with immunosuppressive therapy. Recent studies (clinical trials, retrospective analysis, meta-analysis, and systematic reviews) evaluate special apheresis technology (i.e., immunoadsorption [IA], small volume plasma exchange), compare different treatments of these neuropathies, or report on the therapy of rare immune-mediated neuropathies in case reports. KEY MESSAGES: TA is a well-established treatment and is safe in acute progressive neuropathies (myasthenia gravis, Guillain-Barré syndrome) with an immune etiology. TPE has been applied for decades and thus has the best evidence so far. The indication for IA depends on the availability of that technology and the evidence by RCTs in special neurological diseases. The treatment with TA should improve the clinical outcome of patients, reducing acute or chronic (chronic inflammatory demyelinating polyneuropathies) neurological symptoms. The informed consent of the patient should carefully weight risks and benefits of the apheresis treatment and consider alternative therapies.