Inflammatory Vascular Diseases

Immune-Mediated Vasculitis

Giant cell temporal arteritis, a granulomatous inflammation of the temporal artery may also involve the ophthalmic artery, branches of the carotids, and the systemic blood vessels. Mononuclear cells and multinucleated giant cells infiltrate the vessel walls, thickening the intima and the media; thus, the lumen becomes significantly reduced, and the temporal arterial wall becomes tender and pulseless. The disease commonly affects individuals over 50 years of age. Headache in the temporal region and elevated sedimentation rate are diagnostic.

Polyarteritis nodosa usually occurs in middle-aged adults and involves the medium-sized and small lepto-meningeal and parenchymal arteries and the nutrient arterioles of the peripheral nerves. In the acute stage, the vessel wall undergoes fibrinoid necrosis, with massive polymorphonuclear infiltration. In the chronic stage, dense fibrosis with residual inflammatory cells replaces the vessel wall (Fig. 4.29). The kidneys, liver, and gastrointestinal tract are commonly involved.

Systemic lupus erythematosus (SLE) affects individuals from adolescence to old age. The small cerebral arteries undergo fibrinoid necrosis, mononuclear infiltration, hyalinization, and fibrosis. The lumen is often occluded by a thromboembolus. Characteristic visceral manifestations are verrucous endocarditis, glomerulo-nephritis, and interstitial pneumonia. Patients may harbor lupus anticoagulant antibodies and present the antiphospholipid antibody syndrome.

Wegener's granulomatosis occurs in middle-age. It is characterized by granulomatous necrosis of the small-and medium-sized arteries, often in a segmental fashion. Frequently, the respiratory tract and kidneys are also affected.

Takayasu's arteritis (aortic arch syndrome, pulseless disease) is common in young women. A granulomatous

FIGURE 4.29

Polyarteritis nodosa in a 47-year-old man. A. The walls of cerebral arteries are grayish and show segmental constriction and distension (string sign). B. A nutrient artery of the sciatic nerve shows acute fibrinoid necrosis with poly-morphonuclear and monocytic leukocytes (HE).

FIGURE 4.29

Polyarteritis nodosa in a 47-year-old man. A. The walls of cerebral arteries are grayish and show segmental constriction and distension (string sign). B. A nutrient artery of the sciatic nerve shows acute fibrinoid necrosis with poly-morphonuclear and monocytic leukocytes (HE).

FIGURE 4.30

Thromboangiitis obliterans in a 50-year-old man. A. Leptomeningeal artery shows marked intimal proliferation and thrombotic occlusion. B. The cortex shows multiple small cystic infarctions (HE).

FIGURE 4.30

Thromboangiitis obliterans in a 50-year-old man. A. Leptomeningeal artery shows marked intimal proliferation and thrombotic occlusion. B. The cortex shows multiple small cystic infarctions (HE).

proliferative inflammatory process with giant cells involves the aorta and its branches, leading to severe luminal narrowing and occlusion.

Primary central nervous system angiitis is characterized by necrosis and infiltration of small- and medium-sized arteries with mononuclear cells and multinucleated epithelial cells. The inflammation may extend into the meninges.

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