Diagnosis and treatment of vasculitis

ABSTRACT

Primary angiitis of the central nervous system (PACNS) is an inflammatory disease affecting exclusively small and medium-sized vessels of the central nervous system. CNSvasculitis may also occur in systemic diseases like giant cell arteritis, Takayasu arteritis, granulomatosis with polyangiitis, or Behçet syndrome. The most common presenting symptoms of CNS vasculitis are multifocal symptoms associated with recurrent episodes of ischemia or hemorrhage, encephalopathy-related cognitive and affective abnormalities, and headaches. Diagnostic work up of CNS vasculitis includes MRI, CSF examination, digital subtraction angiography and brain biopsy. High-resolution, contrast-enhanced, compensated and fat-saturated MRI imaging of the cerebral vessel walls (black-blood imaging) may be of some value for the detection of CNS-vasculitis. Patients with normal CSF findings are unlikely to have CNS vasculitis. Brain biopsy should be performed in suspected PACNS. Important differential diagnoses include reversible cerebral vasoconstriction syndrome, moyamoya angiopathy and infectious vasculopathies (VZV, SarsCoV2, borreliosis, bacterial endocarditis). The adherence to diagnostic criteria and the avoidance of inappropriate therapies are essential. Treatment recommendations for CNS-vasculitis include glucocorticoids in combination with cyclophosphamide or rituximab;however, randomized clinical trials of PACNS treatment do not exist. Induction therapy is recommended for 6 to 12 months. After remission is achieved, treatment may be continued with substances as mycophenolate mofetil, methotrexate, or azathioprine. Repeated clinical, CSF- and neuroradiological monitoring is needed to determine the individual duration of maintenance therapy.