Klinik, Diagnostik und Therapie der vaskulitischen Neuropathie

D. Heuss; B. Schlotter-Weigel; C. Sommer

doi:10.1055/s-2003-38510

Fortschritte der Neurologie · Psychiatrie, Inhaltsverzeichnis

Fortschr Neurol Psychiatr 2003; 71(4): 172-186
DOI: 10.1055/s-2003-38510

Originalarbeit

Klinik, Diagnostik und Therapie der vaskulitischen Neuropathie

Bundeseinheitliche Konsensuspapiere der Muskelzentren im Auftrag der Deutschen Gesellschaft für Muskelkranke e. V. (DGM)Diagnosis and Therapy of Vasculitic Neuropathy Consensus Statement of the German Centers for Neuromuscular Disease D. Heuss¹ , B. Schlotter-Weigel² , C. Sommer³

¹Neurologische Klinik der Friedrich-Alexander-Universität Erlangen-Nürnberg
²Friedrich-Baur-Institut an der Neurologischen Klinik der Ludwig-Maximilians-Universität München
³Neurologische Klinik der Julius-Maximilians-Universität Würzburg

Unter Mitarbeit von E. Reinhold-Keller und W. L. Gross, Abteilung für Rheumatologie der Medizinischen Universitätsklinik Lübeck, A. Engelhardt, Neurologische Klinik, Evangelisches Krankenhaus Oldenburg, R. Kiefer, Neurologische Klinik, Universitätsklinikum Münster, K. V. Toyka, Neurologische Klinik der Julius-Maximilians-Universität Würzburg, D. Pongratz, Friedrich-Baur-Institut an der Neurologischen Klinik der Ludwig-Maximilians-Universität München und B. Neundörfer, Neurologische Klinik der Friedrich-Alexander-Universität Erlangen

Abstract

Zusammenfassung

Unter vaskulitischen Neuropathien versteht man immunvermittelte Erkrankungen des peripheren Nervensystems, bei denen die Schädigung der Nerven durch eine Entzündung der Blutgefäße bedingt ist. Unterschieden werden vaskulitische Neuropathien bei primären und sekundären systemischen Vaskulitiden, bei entzündlich-rheumatischer Systemerkrankung, bei malignen Erkrankungen, medikamenteninduzierte Vaskulitiden und die nicht systemische vaskulitische Neuropathie (NSVN). Das typische klinische Erscheinungsbild einer vaskulitischen Neuropathie ist eine asymmetrische oder multifokale, schmerzhafte, sensomotorische Neuropathie mit akutem/subakutem oder chronischem Verlauf mit schubförmigen Verschlechterungen. Charakteristisch sind weiterhin der elektrophysiologische Nachweis einer aktiven, asymmetrischen, axonalen sensomotorischen Neuropathie und die Besserung durch immunsuppressive Therapie. Eine gesicherte Vaskulitis liegt vor, wenn ein positiver Biopsiebefund erhoben wird, das Fehlen eindeutiger morphologischer Befunde schließt sie jedoch nicht aus. Es gibt keinen Labortest, der eine Vaskulitis definitiv nachweist oder ausschließt, so ist auch bei ausschließlich unauffälligen Ergebnissen von durchaus umfangreichen Blutuntersuchungen das Vorliegen einer NSVN möglich. Systemische Vaskulitiden treten mit einer Inzidenz von mindestens 4/100 000 jährlich auf und haben unbehandelt eine schlechte Prognose, die durch immunsuppressive Therapie entscheidend verbessert wird. Bei der NSVN ist die Prognose deutlich besser, eine längerfristige immunsuppressive Therapie ist auch hier häufig erforderlich. Die aktuellen Therapiestandards werden in diesem Konsensuspapier dargestellt.

Abstract

Vasculitic neuropathies are immune mediated diseases of the peripheral nervous system, in which inflammation of the blood vessels causes damage to the nerves. We distinguish neuropathies associated with primary and secondary systemic vasculitis, with rheumatic diseases, with malignant disorders, drug-induced vasculitis and the non-systemic vasculitic neuropathies (NSVN). The typical clinical picture consists in an asymmetric or multifocal, painful sensorimotor neuropathy with an acute, subacute or chronic course and acute relapses. Neurophysiology reveals an active, asymmetric, axonal sensorimotor neuropathy. The disorders usually respond to immunosuppressive treatment. A diagnosis of definite vasculitis can be made with evidence of vasculitis in a biopsy specimen. The absence of positive morphological evidence, however, does not exclude the diagnosis. There is no single laboratory test that can prove or exclude vasculitis, in NSVN even an elaborate panel of blood tests can show normal findings. Systemic vasculitis has an incidence of 4/100 000 per year and, untreated, has a poor prognosis, which is greatly improved by the use of immunosuppressive treatment. The prognosis of NSVN is generally better, although many patients need long term immunosuppression. Current treatment recommendations for vasculitic neuropathies are presented.

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Referenzen

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Prof. Dr. D. Heuss

Neurologische Universitätsklinik

Schwabachanlage 6

91054 Erlangen