Vasculitis and vasculopathy
Definition
Vasculitis
- Vasculitis is inflammation of blood vessel walls causing: Vessel wall damage. Stenosis or occlusion. Ischemia and infarction of tissues, including the nervous system
Vasculopathy: Vasculopathy refers to non-inflammatory diseases of blood vessels causing vascular dysfunction, thrombosis, or occlusion.
1. Vasculitis
Classification
1.Large-Vessel Vasculitis: Giant Cell Arteritis. Takayasu Arteritis
2.Medium-Vessel Vasculitis: Polyarteritis Nodosa
3.Small-Vessel Vasculitis:
- ANCA-associated vasculitis: Granulomatosis with Polyangiitis. Microscopic Polyangiitis
- Immune-complex vasculitis
Neurological Manifestations of Vasculitis
- Central Nervous System: Ischemic stroke. Transient ischemic attacks (TIA). Headache. Seizures. Cognitive dysfunction. Encephalopathy
- Peripheral Nervous System: Mononeuritis multiplex (most characteristic). Sensorimotor polyneuropathy
Mononeuritis Multiplex
Definition: Asymmetric involvement of multiple peripheral nerves due to ischemic nerve injury.
Features: Acute/subacute onset. Painful neuropathy. Asymmetrical weakness. Sensory loss
Primary Angiitis of the CNS (PACNS)
Features: Headache. Stroke-like episodes. Cognitive decline. Seizures.
Diagnosis: MRI abnormalities. Angiography. Brain biopsy (gold standard)
Diagnosis of Vasculitis
- Laboratory Findings: ↑ ESR and CRP. ANCA positivity (certain forms). Autoimmune markers
- CSF: Mild inflammatory changes (CNS vasculitis)
- Imaging: MRI brain. CT/MR angiography
- Biopsy: Definitive diagnosis when feasible
Treatment of Vasculitis
- First-Line: Corticosteroids
- Severe Disease: Cyclophosphamide. Rituximab. Other immunosuppressants
2. Vasculopathy
Definition: Non-inflammatory vascular disorders affecting blood vessels.
Important Types
A. Atherosclerotic Vasculopathy: Most common. Causes ischemic stroke and TIA
B. Hypertensive Vasculopathy: Small vessel disease. Lacunar infarcts. Intracerebral hemorrhage
C. Diabetic Vasculopathy: Microvascular disease. Stroke risk increased
D. Cerebral Amyloid Angiopathy
- Pathology: Amyloid deposition in cerebral vessels
- Features: Lobar intracerebral hemorrhage. Elderly patients
Clinical Manifestations of Vasculopathy
- CNS: Stroke. TIA. Vascular dementia. Intracerebral hemorrhage
- Peripheral Nervous System: Ischemic neuropathies (less common)