Immuntherapie der chronisch inflammatorischen demyelinisierenden Polyneuropathie

Zusammenfassung

Die chronisch inflammatorische demyelinisierende Polyneuropathie (CIDP) gilt als die häufigste behandelbare chronische Erkrankung des peripheren Nervensystems bei einer Prävalenz von etwa 1 - 2/100 000. Randomisierte und kontrollierte Studien belegen eine vergleichbare therapeutische Wirksamkeit von Glukokortikosteroiden, intravenösen Immunglobulinen und der Plasmapherese. Bei Beobachtungszeiträumen von jeweils weniger als 2 Monaten ist die Effektivität der genannten therapeutischen Optionen in der Akutintervention, nicht jedoch auf vergleichbarem Niveau in der Langzeittherapie gesichert. Die vorliegenden Therapiestudien zeigen zudem übereinstimmend, dass etwa ein Drittel aller Patienten nicht auf die initial gewählte therapeutische Option anspricht. Aufgrund positiver Fallserien in der Literatur können bei unzureichendem Ansprechen der Einsatz von Cyclophosphamid und Cyclosporin A, alternativ Kombinationstherapien erwogen werden. Welcher therapeutische Ansatz initial gewählt wird, hängt wesentlich von der Krankheitsdynamik und der funktionellen Beeinträchtigung des Patienten sowie eventuellen Begleiterkrankungen ab. Die vorliegende Übersicht fasst die derzeitige Studienlage zur Immuntherapie der CIDP zusammen und bewertet vorhandene therapeutische Optionen nach deren Wirksamkeit, Verträglichkeit und Kosten.

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated disease of the peripheral nervous system with an estimated prevalence of 1 - 2/100 000. The clinical presentation is heterogeneous, but the most common form causes symmetrical progressive or relapsing weakness affecting proximal and distal muscles. CIDP is among the most treatable peripheral nerve disorders and corticosteroids, plasmapheresis and intravenous immunoglobulin have been shown to be effective in short-term prospective, randomized controlled trials. Data however indicate that approximately one-third of patients do not respond to these treatment modalities, nor do they provide equivalent evidence for a durable clinical response. There is a lack of good quality controlled trials of any other immunosuppressive agent, but cyclophosphamide and cyclosporin may be of value in patients with poor response to first-line modalities. Alternatively, the use of combination therapy may increase the efficacy in unresponsive patients. This review highlights the current status of CIDP treatment trials and discusses the significance of any therapeutic option in terms of efficacy, tolerability and cost-effects.

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