Chapter 159: Vasculitis

DEFINITION AND PATHOGENESIS

A clinicopathologic process characterized by inflammation of and damage to blood vessels, compromise of vessel lumen, and resulting ischemia. Clinical manifestations depend on size and location of affected vessel. Most vasculitic syndromes appear to be mediated by immune mechanisms. May be primary or sole manifestation of a disease or secondary to another disease process. Unique vasculitic syndromes can differ greatly with regards to clinical features, disease severity, histology, and treatment.

PRIMARY VASCULITIS SYNDROMES

Granulomatosis with Polyangiitis (Wegener’s)

Granulomatous vasculitis of upper and lower respiratory tracts together with glomerulonephritis; upper airway lesions affecting the nose and sinuses can cause purulent or bloody nasal discharge, mucosal ulceration, septal perforation, and cartilaginous destruction (saddlenose deformity). Lung involvement may be asymptomatic or cause cough, hemoptysis, dyspnea; eye involvement may occur; glomerulonephritis can be rapidly progressive and asymptomatic and can lead to renal failure.

Microscopic Polyangiitis

Small-vessel vasculitis that can affect the glomerulus and lungs; medium-sized vessels also may be affected.

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)

Granulomatous vasculitis of multiple organ systems, particularly the lung; characterized by asthma, peripheral eosinophilia, eosinophilic tissue infiltration; glomerulonephritis can occur.

Polyarteritis Nodosa

Medium-sized muscular arteries involved; frequently associated with arteriographic aneurysms; commonly affects renal arteries, liver, GI tract, peripheral nerves, skin, heart; can be associated with hepatitis B and C.

Giant Cell Arteritis

Inflammation of medium- and large-sized arteries; primarily involves temporal artery but systemic and large vessel involvement may occur; symptoms include headache, jaw/tongue claudication, scalp tenderness, fever, musculoskeletal symptoms (polymyalgia rheumatica); sudden blindness from involvement of optic vessels is a dreaded complication.

Takayasu Arteritis

Vasculitis of the large arteries with strong predilection for aortic arch and its branches; most common in young women; presents with inflammatory or ischemic symptoms in arms and neck, systemic inflammatory symptoms, aortic regurgitation.

IgA Vasculitis (Henoch-Schönlein)

Characterized by involvement of skin, GI tract, kidneys; more common in children; may recur after initial remission.

Cryoglobulinemic Vasculitis

Majority of cases are associated with hepatitis C where an aberrant immune response leads to formation of cryoglobulin; characterized by cutaneous vasculitis, arthritis, peripheral neuropathy, and glomerulonephritis.

Idiopathic Cutaneous Vasculitis

Cutaneous vasculitis is defined broadly as inflammation of the blood vessels of the dermis; due to underlying disease in >70% of cases (see “Secondary Vasculitis Syndromes” below) with 30% occurring idiopathically.

Miscellaneous Vasculitic Syndromes

• Kawasaki disease

• Primary central nervous system vasculitis

• Behçet’s disease

• Cogan’s syndrome

• Polyangiitis overlap syndrome

SECONDARY VASCULITIS SYNDROMES

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