CNS Vasculitis Diagnosis and Management

Clinical Presentation

The American College of Rheumatology suggests considering CNS vasculitis in patients presenting with ischemic or hemorrhagic stroke, recurrent stroke, stroke with encephalopathic changes, or stroke accompanied by fever, multifocal neurological events, unexplained skin lesions, glomerulopathy, or elevated sedimentation rate 1, 2
Cognitive deficits affecting attention, visual memory, verbal memory, executive function, and psychomotor speed are common in CNS vasculitis patients, as reported by the Annals of the Rheumatic Diseases 3
Seizures occur as presenting symptoms in a subset of patients with primary CNS vasculitis, according to Praxis Medical Insights 1, 2
Focal neurological deficits occur in only 20-30% of patients with primary CNS vasculitis, as stated by Praxis Medical Insights 1
Intracranial hemorrhage may be the initial presentation of CNS vasculitis, as noted by Praxis Medical Insights 1
A progressive course with small-vessel strokes developing over weeks to many months is characteristic of primary CNS vasculitis, according to Praxis Medical Insights 1

Elevated ESR or CRP is a common laboratory finding in CNS vasculitis patients, although ESR is often normal or only minimally elevated in primary CNS vasculitis, as reported by Praxis Medical Insights 1, 2
Positive ANCA (by both immunofluorescence and ELISA) in the appropriate clinical context is a diagnostic marker for CNS vasculitis, according to Praxis Medical Insights 1, 2
Positive antiphospholipid antibodies in SLE patients with stroke is a diagnostic marker for antiphospholipid syndrome, as reported by the Annals of the Rheumatic Diseases 3
Cryoglobulinemia in HCV-infected patients is a diagnostic marker for cryoglobulinemic vasculitis, according to Autoimmunity Reviews 4

Multiple infarcts of variable ages on MRI are present in up to 50% of CNS vasculitis cases, as reported by Praxis Medical Insights 1
Mass lesions (5% of cases), meningeal enhancement (8% of cases), and hemorrhage (9% of cases) are neuroimaging findings in CNS vasculitis patients, according to Praxis Medical Insights 1
MRI abnormalities are present in >90% of CNS vasculitis cases, as stated by Praxis Medical Insights 1

Known systemic vasculitis (ANCA-associated vasculitis, polyarteritis nodosa) presenting with new neurological symptoms is a red flag for CNS vasculitis, according to Stroke 5, 2
Systemic lupus erythematosus with stroke, especially if antiphospholipid antibodies are present, is a diagnostic consideration for CNS vasculitis, as reported by the Annals of the Rheumatic Diseases 3
Hepatitis C infection with cryoglobulinemia and neurological symptoms is a diagnostic consideration for cryoglobulinemic vasculitis, according to Autoimmunity Reviews 4
Recent varicella zoster infection followed by stroke (VZV vasculitis) is a diagnostic consideration for CNS vasculitis, as reported by Stroke 5