From the Guidelines
Pediatric CNS vasculitis requires prompt diagnosis and treatment with high-dose corticosteroids and immunosuppressive agents to prevent permanent neurological damage and improve long-term outcomes. The initial management typically involves high-dose corticosteroids such as methylprednisolone (30 mg/kg/day, maximum 1g/day) for 3-5 days, followed by oral prednisone (1-2 mg/kg/day) with a slow taper over 12-24 months, as suggested by the American Heart Association stroke council and the council on cardiovascular disease in the young 1. For moderate to severe cases, cyclophosphamide is often added (500-750 mg/m² monthly for 6-7 doses) followed by maintenance therapy with mycophenolate mofetil (600-1200 mg/m² twice daily) or azathioprine (2-3 mg/kg/day) 1.
Diagnosis and Monitoring
Diagnosis requires a comprehensive approach including:
- MRI with contrast
- MR angiography
- Sometimes conventional angiography or brain biopsy Disease monitoring should include:
- Regular neurological examinations
- Inflammatory markers (ESR, CRP)
- Repeat imaging every 3-6 months initially The pathophysiology involves immune-mediated inflammation of blood vessel walls leading to narrowing, occlusion, and subsequent ischemia or hemorrhage, as described in the management of stroke in infants and children 1.
Treatment Approach
The treatment approach should be individualized based on the severity of the disease and the patient's response to therapy. Early aggressive treatment is crucial as it can prevent permanent neurological damage and improve long-term outcomes in affected children. Anti-platelet therapy with aspirin (3-5 mg/kg/day) is typically used to prevent thrombotic complications. The use of immunosuppressive agents such as cyclophosphamide and mycophenolate mofetil has been shown to be effective in treating pediatric CNS vasculitis, as reported in the study by the American Heart Association stroke council and the council on cardiovascular disease in the young 1.
Key Considerations
Key considerations in the management of pediatric CNS vasculitis include:
- Prompt diagnosis and treatment to prevent permanent neurological damage
- Individualized treatment approach based on disease severity and patient response
- Regular monitoring of disease activity and treatment side effects
- Use of immunosuppressive agents and anti-platelet therapy to prevent thrombotic complications, as suggested by the study 1.
From the Research
Definition and Classification of Pediatric CNS Vasculitis
- Pediatric CNS vasculitis is an inflammatory brain disease that can be primary or secondary to various conditions, including infections, collagen vascular diseases, systemic vasculitides, and malignancies 2, 3, 4, 5.
- Primary CNS vasculitis, also known as primary angiitis of the central nervous system of childhood (cPACNS), is a reversible cause of severe neurological impairment, including acute ischemic stroke, intractable seizures, and cognitive decline 3, 4.
Clinical Presentation and Diagnosis
- The most common presenting features of pediatric CNS vasculitis are headaches and focal neurologic deficits 5.
- Diagnosis of primary CNS vasculitis is based on the Calabrese criteria, and may involve laboratory tests, neuroimaging, and brain biopsy 2, 5.
- Angiography is a key imaging modality, and can help distinguish between large-medium vessel and small-vessel vasculitis, each with distinct clinical and radiological features 3.
Treatment and Prognosis
- Treatment of primary CNS vasculitis often consists of induction therapy with steroids and cyclophosphamide, followed by maintenance therapy with a prednisone taper and medications such as azathioprine or mycophenolate mofetil 6.
- Relapse is common, and may require an increased dose of steroids or a change in therapy, with medications such as rituximab and mycophenolate mofetil as potential alternatives 6.
- Mortality rates for primary CNS vasculitis are reported to be around 8-9% 6.
Differential Diagnosis
- The differential diagnosis for pediatric CNS vasculitis has expanded in recent years to include vasoconstrictive disorders and neuronal antibody-associated conditions, which can have overlapping clinical features with cPACNS 4.
- Conditions that result in vasospasm or are associated with noninflammatory vasculopathies may also mimic the features of CNS vasculitis 5.