Vasculitis

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  • i. Most forms of angiitis involving the CNS affect the microvasculature; only two types affect the major arteries feeding the CNS
    • 1. giant cell arteritis (a.k.a. temporal arteritis)
      • a. anomalous granulomatous disease in the elastic component of the arterial walls; occurs almost exclusively in elderly patients; can cause blindness due to central retinal artery occlusion; diagnosis is by biopsy but disease may produce skip lesions so that a negative result does not exclude the disease
      • b. most often has elevated ESR but ESR is normal in 5% of cases
      • c. treatment: steroids
      • d. related disease: polymyalgia rheumatica
        • i. both have perivascular infiltrates with giant cells about cranial arteries and exacerbations may be associated with fever, weight loss, and anemia; pain in temporal arteritis is confined to the head while in polymyalgia rheumatica it is disseminated; patients with polymyalgia rheumatica have an elevated ESR, anemia, malaise and weight loss; CPK is normal in polymyalgia rheumatica; both may be treated with steroids
    • 2. Takayasu’s arteritis (a.k.a. aortic arch syndrome)
      • a. Disease usually affects the aorta but may also involve the carotid arteries
      • b. May cause visual loss and loss of peripheral pulses
  • ii. Three most common microvasculopathies
    • 1. arteriosclerosis
    • 2. lipohyalinosis (closely linked with arteriosclerosis)
    • 3. cerebral amyloid angiopathy (CAA)
    • 4. others
      • a. polyarteritis nodosa – multifocal panarteritis with destruction of the elastic affecting small and medium sized arteries; see neutraphil infiltration and fibrinoid necrosis; associated with mononeuritis multiplex, kidney and skin involvement
      • b. allergic angiitis and granulomatosis (Churg-Strauss syndrome) – produces fibrinoid necrosis and eosinophilic granulomatous inflammation
      • c. Wergener’s granulomatosis – results in fibrinoid necrosis, presence of inflammatory cells in small arteries and veins; associated with anti-neutrophil cytoplasmic autoantibodies
      • d. Binswanger’s subcortical arteriosclerotic leukoencephalopathy
      • e. SLE and TTP – cause siderocalcinosis of small vessels
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