Overview
Primary angiitis of the central nervous system (PACNS) is a rare inflammatory vasculopathy affecting cerebral blood vessels, leading to neurological symptoms through ischemic or hemorrhagic events 1.Diagnosis
Clinical Presentation: Common symptoms include focal neurologic signs (63%), headache (51%), and cognitive impairment (41%) 2.
Diagnostic Tests:
- Biopsy: Gold standard for confirmation, though invasive 2.
- Angiography: Positive in 33% of biopsy-confirmed cases 2.
- MRI: Abnormalities present in nearly all cases (97%) 2.
- CSF Analysis: Abnormalities seen in 75% of cases 2.
Differentiation: LV-PACNS and SV-PACNS require distinct considerations, though evidence varies 1.Management
First-Line Treatment:
- Corticosteroids: Often initial therapy, though specific dosing is not detailed 1.
- Immunosuppressive Agents: Cyclophosphamide and rituximab are frequently used adjunctively 1.
Adjunctive Treatments:
- Plasma Exchange: Considered in refractory cases 1.
- Anticoagulants: Use may be considered based on specific vascular involvement 1.Special Populations
Pregnancy: Limited data; management typically involves balancing maternal and fetal risks with immunosuppressive therapy 1.
Pediatrics: Specific guidelines sparse; treatment often mirrors adult protocols with caution 1.
Elderly: Tailored approach considering comorbidities and drug interactions; close monitoring essential 1.
Comorbidities: Management must account for interactions with existing conditions, often requiring multidisciplinary input 1.Key Recommendations
Biopsy Confirmation: Preferred for definitive diagnosis despite invasiveness (Evidence: Expert opinion) 1.
Initial Therapy with Corticosteroids: Recommended as first-line treatment (Evidence: Expert opinion) 1.
Use of Immunosuppressive Agents: Cyclophosphamide or rituximab for refractory or severe cases (Evidence: Expert opinion) 1.
MRI as Essential Diagnostic Tool: Given high sensitivity in detecting abnormalities (Evidence: Moderate) 2.
Consider CSF Analysis: Useful in supporting diagnosis despite variable specificity (Evidence: Moderate) 2.
Tailored Management in Special Populations: Requires individualized care plans considering unique risks and benefits (Evidence: Expert opinion) 1.References
1 Pascarella R, Antonenko K, Boulouis G, De Boysson H, Giannini C, Heldner MR et al.. European Stroke Organisation (ESO) guidelines on Primary Angiitis of the Central Nervous System (PACNS). European stroke journal 2023. link
2 Beuker C, Strunk D, Rawal R, Schmidt-Pogoda A, Werring N, Milles L et al.. Primary Angiitis of the CNS: A Systematic Review and Meta-analysis. Neurology(R) neuroimmunology & neuroinflammation 2021. link