Cerebral involvement in systemic vasculitides

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. 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 steroid-sparing 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 should be virus elimination.
• 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.
• With neurological involvement, therapy with GC and CYC or RTX is recommended for remission induction. After remission is achieved, MTX and AZA are considered medications of equal value for remission maintenance.
• Systemic Lupus erythematodes (SLE) is an autoimmune multiorgan disease with a complex disruption of immunoregulation, which leads to a loss of immunological self-tolerance to nuclear autoantigens and formation of autoantibodies. CNS involvement is reported in 6.4 to 93% of the cases. The American College of Rheumatology described 19 different neuropsychiatric manifestations of SLE (NPSLE). Antiphospholipid antibodies (aPL) show an association with cerebrovascular manifestations in SLE, but not with inflammatory CNS manifestations.
• The specific therapy of NPSLE depends on whether the underlying impairment is inflammatory (CNS-vasculitis) or thrombo-embolic in nature. If an inflammatory process is assumed, treatment is made with GC alone or in combination with immunosuppressives (AZA or CYC). In severe, refractory NPSLE cases, plasmapheresis, IVIG and an anti-B-cell therapy with RTX are recommended. Anticoagulation appears to be superior to antiaggregants for secondary prevention of ischemic stroke in APS.