Vaskulitische Neuropathien bei rheumatischen Erkrankungen

Zusammenfassung

Vaskulitische Neuropathien sind immunvermittelte entzündliche Gefäßerkrankungen des peripheren Nervensystems. Diese kommen bei den primären systemischen Vaskulitiden wie der Panarteriitis nodosa, den ANCA-assoziierten Vaskulitiden (z. B. Eosinophile Granulomatose mit Polyangiitis, Granulomatose mit Polyangiitis, mikroskopische Polyangiitis), der kryoglobulinämischen Vaskulitis sowie bei den sekundären Vaskulitiden bei Kollagenosen (systemischer Lupus erythematodes, primäres Sjögren Syndrom, systemische Sklerose) sowie der rheumatoiden Arthritis, Infektionen, Malignomen oder Medikamenten vor. Die vaskulitische Neuropathie ist bei den genannten Erkrankungen eine häufige Organmanifestation und teilweise syndromdefinierend. Typisch für die Neuropathie sind eine asymmetrische sensomotorische oder multifokale (Multiplex-Typ), häufig schmerzhafte Polyneuropathie mit einem axonalen Schädigungsmuster durch eine ischämische Nervenschädigung. Letztlich kommen aber alle Polyneuropathiemuster vor, in der Regel mit einem progressiven, teils schubförmigen, in der Regel jedoch nicht selbstlimitierendem Verlauf. Die immunologischen Mechanismen sind vielfältig und führen häufig zu einer Vaskulitis der Vasa vasorum der Nerven mit der Notwendigkeit zu einer suffizienten immunsuppressiven Therapie, welche zu einer Besserung der Neuropathie führt.

Abstract

Vasculitic neuropathies are immune-mediated inflammatory vascular diseases of the peripheral nervous system. They occur in primary systemic vasculitides such as panarteritis nodosa, ANCA-associated vasculitides (e. g. eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, microscopic polyangiitis), cryoglobulinemic vasculitis and secondary vasculitides associated with connective tissue diseases (systemic lupus erythematosus, primary Sjögren’s syndrome, systemic sclerosis) and in rheumatoid arthritis, infections and malignancies and may also be induced by medications. In the aforementioned diseases, vascular neuropathy is a frequent organ manifestation, which partly defines the syndrome. Neuropathy is characterised by asymmetric sensorimotor or multifocal (multiplex type) polyneuropathy, frequently painful, with axons being damaged by ischemic nerve injury. Ultimately, however, all patterns of polyneuropathy occur, usually with a progressive, partly relapsing course, which is not self-limiting. The condition’s underlying immunological mechanisms are diverse and often lead to vasculitis of the vasa vasorum of the nerves, which requires an adequate immunosuppressive treatment aiming to alleviate the neuropathy.

Schlüsselwörter

Vaskulitische Neuropathie - Kollagenose - systemische Vaskulitis - Neuritis - Polyneuropathie

Key words

connective tissue disease - systemic vasculitis - neuritis - polyneuropathy - vasculitic neuropathy

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