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Cerebral amyloid angiopathy: possible relationship to rheumatoid vasculitis
Insights
Cerebral amyloid angiopathy can coexist with chronic cerebral vasculitis, particularly in patients with rheumatoid arthritis. This combined condition affects brain blood vessels and may contribute to dementia.
Area of Science:
- Neurology
- Pathology
- Rheumatology
Background:
- Cerebral amyloid angiopathy (CAA) is a cerebrovascular disorder characterized by amyloid-beta deposition in vessel walls.
- Rheumatoid arthritis (RA) is a systemic autoimmune disease that can affect various organs, including the central nervous system.
Observation:
- Three cases presented with concurrent CAA and chronic cerebral vasculitis.
- The vasculitis exhibited features similar to rheumatoid vasculitis, including fibrinoid necrosis and inflammatory infiltrates.
- Two patients had a history of rheumatoid arthritis, and one had unspecified arthritis at dementia onset.
Findings:
- Both vasculitis and amyloidosis affected leptomeningeal and cerebral cortical vessels.
- In autopsy-verified cases, vascular disease was confined to the brain.
- Amyloid plaques were present in all cases; abundant plaques and Alzheimer tangles were noted in one case.
Implications:
- This association suggests a potential link between autoimmune inflammatory processes and the pathogenesis of CAA.
- Understanding this comorbidity is crucial for diagnosing and managing dementia in patients with inflammatory conditions.
- Further research is needed to elucidate the mechanisms underlying this combined cerebrovascular pathology.
Abstract:
In three cases of cerebral amyloid angiopathy there was also a chronic cerebral vasculitis characterized by segmental fibrinoid necrosis, chronic adventitial inflammatory infiltrates, obliterative "endarteritis" and hyaline arteriolar change, resembling rheumatoid vasculitis. Two of these cases had rheumatoid arthritis, and one had unspecified "arthritis" at the onset of dementia. Both vasculitis and amyloidosis involved the leptomeningeal and cerebral cortical vessels. In the two autopsy-verified cases, the vascular disease was limited to the brain. In the third case, only a brain biopsy was available. Amyloid-containing neuritic plaques were present in the cerebral cortex in all three cases, but they were abundant only in one, which also showed numerous Alzheimer tangles.