Therapie immunvermittelter Neuropathien

Zusammenfassung

Das Guillain-Barré-Syndrom (GBS), die chronische Polyneuritis (inflammatorische demyelinisierende Polyneuropathie [CIDP]), die multifokale motorische Neuropathie (MMN) und die paraproteinämischen Polyneuropathien sind behandelbare immunvermittelte Neuropathien. Beim GBS sind Plasmapherese und intravenöse Immunglobuline (IVIG) in der Akutphase in gleichem Maße wirksam. Patienten mit CIDP und MMN benötigen ne ben der Akuttherapie längerfristige Behandlungsmaßnahmen. Im Gegensatz zur CIDP, die sowohl auf polyvalente intravenöse Immunglobuline (IVIG) als auch auf Kortikosteroide gut anspricht, sind bei der MMN IVIG das Mittel der ersten Wahl. Für die Langzeittherapie sind immunsuppressive Therapien von Bedeutung, wobei jedoch kontrollierte Studien zum Einsatz dieser Medikamente bei immunvermittelten Neuropathien weitgehend fehlen. Für die paraproteinämischen Neropathien gelten ähnliche Behandlungsstrategien wie für die CIDP, wobei allerdings die Polyneuropathie mit Antikörpern gegen Myelinassoziiertes Glykoprotein (MAG) eine Sonderstellung einnimmt. Hier sind starke Immunsuppressiva nötig. Neuere Strategien wie der Einsatz von Antikörpern gegen CD20 sind hier Erfolg versprechend.

Summary

Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuritis (CIDP), multifocal motor neuropathie (MMN) and paraproteinemic polyneuropathy are immune mediated neuropathies that respond to immunomodulatory treatment. During acute disease plasmapheresis and intravenous immunoglobulins (IVIG) are first line therapeutics with similar efficacy. Furthermore patients with CIDP and MMN need long term immunomodulatory treatment. In contrast to CIDP, that responds very well to corticosteroids, MMN patients show no benefit when treated with steroids. Here IVIG are the first line therapeutics. For long term immunomodulatory treatment immunosuppressive agents are of great value, although controlled studies proving this concept are missing. For the treatment of paraproteinemic neuropathies same strategies like in CIDP should be applied. In particular CIDP with antibodies to myelin-associated glycoprotein (MAG) needs a consequent immunosuppressive treatment – new strategies like antibodies against CD20 might be of interest in the near future.

Schlüsselwörter

Guillain-Barré-Syndrom - CIDP - MMN

Keywords

Guillain-Barré syndrome - CIDP - MMN

• Literatur

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