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Primäre Vaskulitis des Zentralnervensystems

Primary central nervous system vasculitis

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Zusammenfassung

Die primäre Angiitis des zentralen Nervensystems (PACNS) ist eine seltene Erkrankung. Trotzdem wird sie häufig differenzialdiagnostisch bei vaskulären oder entzündlichen Erkrankungen des Zentralnervensystems (ZNS) diskutiert. Die Diagnosestellung ist schwierig, da spezifische Biomarker fehlen und die klinischen Symptome variabel sind. Eine sichere Diagnose ist nur durch den bioptischen Nachweis entzündlicher Gefäßwandveränderungen möglich. Alternativ kann in Zusammenschau von klinischen, bildgebenden und laborchemischen Befunden die Diagnose gestellt werden. In den letzten Jahren wurden verschiedene Subtypen der PACNS anhand der Größe der betroffenen Gefäße und mithilfe histopathologischer Kriterien beschrieben. Basierend auf einer selektiven Literaturrecherche in der Datenbank PubMed zum Thema primäre ZNS-Vaskulitis gibt dieser Beitrag eine Übersicht über die diagnostischen Charakteristika der Erkrankung und ihrer Differenzialdiagnosen. Es wird eine Stufendiagnostik vorgeschlagen, wobei je nach Verteilungsmuster der betroffenen Gefäße unterschiedliche Zusatzuntersuchungen empfohlen werden. Zuletzt wird auf die therapeutischen Optionen und die Prognose der PACNS eingegangen.

Abstract

Primary angiitis of the central nervous system (PACNS) is a rare disorder. However, it is often considered in the differential diagnosis of vascular or inflammatory CNS diseases. Diagnosis is challenging, as specific biomarkers are lacking and the clinical presentation can be variable. A definitive diagnosis can only be established by biopsy of the inflammatory changes in the vascular wall. Alternatively, the diagnosis of PACNS can also be based on the synopsis of clinical, radiological, and laboratory findings. Different subtypes of PACNS have been described in recent years, depending on the size of the affected vessels or histopathological patterns. Based on selective literature research in the database PubMed on the subject of CNS vasculitis, this article reviews the diagnostic characteristics and differential diagnosis of the condition. We suggest a diagnostic algorithm customized to the size of the affected vessels. Lastly, therapeutic options and the outcome of PACNS are briefly outlined.

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Correspondence to T. Magnus.

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S. Schuster und T. Magnus geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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B. Hellmich, Kirchheim-Teck

P. Lamprecht, Lübeck

F. Moosig, Neumünster

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Schuster, S., Magnus, T. Primäre Vaskulitis des Zentralnervensystems. Z Rheumatol 74, 854–862 (2015). https://doi.org/10.1007/s00393-015-0007-9

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  • DOI: https://doi.org/10.1007/s00393-015-0007-9

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