Abstract
Cerebral vasculitis is a rare disorder but plays a major role in the differential diagnosis of stroke, encephalopathy and headache. This guideline was developed in order to support clinicians in the diagnosis and treatment of cerebral manifestations of systemic vasculitides and rheumatic diseases. It is based on a medline research and was developed in a modified Delphi process and approved by the involved societies.This article is an abridged and translated version of the guideline published in DGNeurologie: Berlit, P. & Krämer, M. DGNeurologie (2018) 1: 17. https://doi.org/10.1007/s42451-018-0001-y
Highlights
Cerebral vasculitis is a rare disorder but plays a major role in the differential diagnosis of stroke, encephalopathy and headache
against the cytoplasm of neutrophilic leukocytes (ANCA)-Associated Vasculitides (AAV) are considered three different entities based on the differences in genetic background and immunological and pathological characteristics: Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA)
GPA is associated with proteinase 3 (PR3)-specific ANCA; MPA and – less often – EGPA with myeloperoxidase (MPO)-specific ANCA
Summary
Giant Cell Arteriitis (GCA) is the most common primary systemic vasculitis. A thoracic aortitis can be demonstrated using PET-CT or MRI in more than 50% of the patients and is associated with the risk of aortic aneurysm and dissection. GC therapy for remission induction is started at an initial dose of 1 mg/kg prednisolone p. Start of dose reduction after clinical improvement and normalization of the inflammation parameter by 10 mg every 2 weeks down to 20 mg, by 2.5 mg every 2–4 weeks down to 10 mg, by 1 mg per month. In GCA with visual symptoms or serious vascular complications, methylprednisolone i.v. In the treatment of Takayasu arteritis (TA) besides prednisolone (initial 0.5–1 mg/kg) in combination with MTX 15–25 mg/week infliximab (5 mg/kg i.v. day 1, 14, 42, every 6 weeks) can be considered. ANCA-Associated Vasculitides (AAV) are considered three different entities based on the differences in genetic background and immunological and pathological characteristics: Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA)
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