From the Guidelines
High-dose steroids should be administered to patients with confirmed primary CNS angiitis, followed by slow tapering of steroids and the addition of a steroid-sparing agent, as this approach has been supported by observational data and is associated with better outcomes compared to long-term monotherapy with steroids. The treatment of CNS vasculitis typically involves immunosuppressive therapy, with the goal of reducing inflammation and preventing further damage to the blood vessels in the brain and spinal cord. According to the 2021 guideline for the prevention of stroke in patients with stroke and transient ischemic attack, primary CNS angiitis or vasculitis is a rare cause of stroke, affecting predominantly younger populations, and is slightly more prevalent in men 1. Some key points to consider in the treatment of CNS vasculitis include:
- The use of high-dose steroids, such as intravenous methylprednisolone, as the initial treatment, followed by oral prednisone 1
- The addition of a steroid-sparing agent, such as cyclophosphamide, mycophenolate mofetil, azathioprine, methotrexate, or rituximab, to reduce the risk of relapse and improve outcomes 1
- The importance of interdisciplinary follow-up and consideration of the profile of each agent and associated comorbidities when choosing a steroid-sparing agent 1
- The need to avoid the use of infliximab in patients with stroke and giant cell arteritis, due to the increased risk of disease activity and lower chances of remission 1. Regular monitoring of treatment response and side effects is essential for optimal management, and treatment typically continues for 2-3 years with gradual tapering based on clinical response. Diagnosis requires a combination of clinical evaluation, MRI with contrast, cerebrospinal fluid analysis, and sometimes brain biopsy, as CNS vasculitis causes inflammation and damage to vessel walls, leading to narrowing, thrombosis, and potential ischemia or hemorrhage, which explains the varied neurological symptoms including headaches, cognitive changes, seizures, and focal deficits.
From the FDA Drug Label
The FDA drug label does not answer the question.
From the Research
Definition and Classification of CNS Vasculitis
- CNS vasculitis is a rare disorder that affects the blood vessels of the brain and spinal cord, and can be classified into two categories: primary and secondary 2.
- Primary CNS vasculitis is a rare disorder of unknown cause that is restricted to the brain and spinal cord, while secondary CNS vasculitis occurs as part of systemic vasculitis or secondary to autoimmune diseases or infections 2, 3.
Diagnosis of CNS Vasculitis
- The diagnosis of CNS vasculitis is extremely challenging, and several conditions can mimic CNS vasculitis and require totally different treatment 4.
- Histopathological evidence of transmural inflammation of blood vessels of CNS is the gold standard for diagnosis, but is generally pursued subsequent to conventional angiogram (CA) because of its invasive nature 3.
- Conventional angiogram (CA) can detect vasculitic changes in the large to medium cerebral arteries, but the specificity is low 3.
- Recent advancements in vessel wall imaging techniques have further enabled the distinction of various intracranial vasculopathies from CNS vasculitis 3.
Treatment of CNS Vasculitis
- The treatment of CNS vasculitis typically involves the use of glucocorticoids as first-line agents, combined with additional immunosuppressants for the most severe cases, mainly cyclophosphamide for induction, followed by less-toxic maintenance therapy with azathioprine, methotrexate, or mycophenolate mofetil 4, 5.
- Biological agents such as rituximab and anti-tumour necrosis factor alpha inhibitors (infliximab and etanercept) may be used in refractory cases 6, 3.
- Combination therapy with cyclophosphamide, rituximab, and corticosteroids may be effective in arresting rapidly progressive primary CNS vasculitis 6.