Vasculitis

• 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