Cerebral involvement in systemic vasculitides
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(2019) 1:13
Neurological Research and Practice
GUIDELINES
Open Access
Cerebral involvement in systemic vasculitides Extracts from the guideline of the German neurological society Peter Berlit1* , Markus Krämer2 and for the Consensus Group
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 Keywords: Cerebral vasculitis, Giant cell Arteriitis (GCA), Takayasu arteritis (TA), Polyarteritis nodosa (PAN), ANCAassociated Vasculitides (AAV), Granulomatosis with polyangiitis (GPA), Microscopic polyangiitis (MPA), Eosinophilic granulomatosis with polyangiitis (EGPA), Systemic lupus erythematosus (SLE)
Main recommendations at a glimpse 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. Immediate performance of color duplex sonography in specialized emergency consultation (Fast Track Clinic) shortens the time to diagnosis, and reduces the risk of permanent visual damage. The core finding in GCA is a circular echo-poor wall edema, the so-called halo sign. GC therapy for remission induction is started at an initial dose of 1 mg/kg prednisolone p. o. per day. Start of dose reduction after clinical improvement and normalization of the inflammation parameter by 10 mg every 2 weeks down to 20 mg, then by 2.5 mg every 2–4 weeks down to 10 mg, then by 1 mg per month. In GCA with visual symptoms or serious vascular complications, methylprednisolone i.v. * Correspondence: [email protected] 1 Deutsche Gesellschaft für Neurologie, Reinhardtstr. 27 C, 10117 Berlin, Germany Full list of author information is available at the end of the article
should initially be given for 3 days in a dose of 500 to 1000 mg. GC-sparing therapy should be initiated at the latest when there is recurrence under GC reduction and/ or a GC requirement of more than 7.6 mg after 6 months. Tocilizumab is the first approved steroidsparing therapy of GCA. 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, then every 6 weeks) can be considered. Polyarteritis nodosa (PAN) with and without proof of hepatitis are two different entities. PAN should primarily be treated with a combination of prednisolone (1 mg/kg BW/d) and CYC if the course is organ-threatening. In the HBV-associated form, the target shou
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