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IV. VASCULITIS SYNDROMES (VASCULITIDES)

These inflammatory and often necrotizing vascular lesions occur in almost any organ and are

usually mediated by immune mechanisms, most often immune complex depositions.

Frequent antigens in immune complexes include DNA, hepatitis B surface antigen, and hepatitis C RNA.

A. Polyarteritis nodosa

1. This condition is characterized by necrotizing immune complex inflammationof small- and

medium-sized arteries. It is marked by the destruction of arterial media and internal elastic lamella, resulting

in aneurysmal nodules.

2. There is an association with hepatitis B viral infectionin 30% of patients.

3. Clinical manifestations often include fever, weight loss, malaise, abdominal pain, headache, myalgia, and

hypertension. Polyarteritis nodosa is seen in the following sites:

a. Kidneys, with immune complex vasculitis in the arterioles and glomeruli; renal lesions and hypertension

cause most deaths from polyarteritis nodosa.

b. Coronary arteries, resulting in ischemic heart disease

c. Musculoskeletal system, resulting in myalgia, arthralgia, or arthritis

d. Gastrointestinal tract, manifesting as nausea, vomiting, or abdominal pain

e. Central nervous system (CNS) or peripheral nervous system, the eye, or skin

B. Churg-Strauss syndrome (allergic granulomatous angiitis).This is a necrotizing vasculitis considered by

some to be a variant of polyarteritis nodosa. It is characterized by prominent involvement of the pulmonary

vasculature, marked peripheral eosinophilia, and clinical manifestations of asthma.

C. Hypersensitivity (leukocytoclastic) vasculitis

1. This is a group of immune complex-mediated vasculitidescharacterized by acute inflammation of small

blood vessels (arterioles, capillaries, venules); the multiple lesions tend to be of the same age. These are in

contrast to the findings in polyarteritis nodosa.

2. It is manifest by palpable purpurawhen the skin is involved but can involve any site, including the

glomeruli or the gastrointestinal tract.

3. It may be precipitated by exogenous antigens, such as drugs, foods, or infectious organisms; may also occu

as a complication of systemic illnesses, such as connective tissue disorders or malignancies.

4. It presents clinically in distinctive syndromes, including:

a. Henoch-Schiilein purpurais most common in young children.

(1) Characteristics include hemorrhagic urticariaof extensor surfaces of the arms, legs, and buttocks,

with fever, arthralgias, and gastrointestinal and renal involvement

(2) This disorder can sometimes be post streptococcal in origin. It is associated with antecedent uppe

respiratoryinfections, suggesting that infectious agents may be the inciting antigens; other antigens may

include drugs or foods.

b. Serum sickness

(1) This syndrome is seen in the experimental model in which rabbits, after serial injections of bovine serum

albumin, develop generalized deposition of antigen anti body complexesin the heart, joints, and kidneys.

(2) Serum sickness is now rare in humans, but in the past, it was caused by therapeutic administration of

various antitoxins (foreign serum containing specialized antibodies prepared by immunization of animals, such

as horses) .

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D. Wegener granulomatosis. This disease of unknown etiology is characterized by necrotizing granulomatous

vasculitis of the small- to medium-sized vessels of the respiratory tract. Kidneysand other organs.

1.Wegener granulomatosis is dominated clinically by respiratory tract signs and symptoms, especially of the

paranasal sinusesand lungs, and necrotizing glomerulonephritis (sometimes with immune complex

deposition). Manifestations include fibrinoid necrosisof small arteries and veins, early infiltration by

neutrophils, subsequent mononuclear cell infiltration, and fibrosis. Granuloma formationwith giant cells is

prominent.

2.In most cases, Wegener granulomatosis is associated with circulating anti-neutrophil cytoplasmic antibodie

with a cytoplasmic staining pattern (C-ANCAs).

E. Giant cell arteritidesare seen in medium- to large-sized arteries and are characterized by granuloma

formation with giant cells, as well as by infiltrates of mononuclear cells, neutrophils, and eosinophils. They

include two distinct clinical syndromes:

1. Temporal arteritisis the most frequently occurring form of vasculitis.

a. This systemicvasculitis occurs most often in elderly persons. It usually affects branches of the carotid

artery, particularly the temporal artery.

b. Clinical manifestations include:

(1) Malaise and fatigue (2) Headacheor claudication of the jaw

(3) Tenderness, absent pulse, and palpable nodules along the course of the involved artery

(4) Visual impairment, especially with involvement of the ophthalmic artery

(5) Polymyalgia rheumatica, a complex of symptoms including proximal muscle pain, periarticular pain, and

morning stiffness

(6) Markedly elevated erythrocyte sedimentation rate

2. Takayasu arteritis (pulseless disease) is characterized by inflammation and stenosis of medium- and large-

sized arteries with frequent involvement of the aortic archand its branches, producing aortic arch syndrome.

Clinical manifestations include:

a. Absent pulsesin carotid, radial, or ulnar arteries

b. Nonspecific findings, such as fever, night sweats, malaise, myalgia, arthritis and arthralgia; eye problems;

and painful skin nodules

F: Mucocutaneous lymph node syndrome (Kawasaki disease) is an acute, self-limited illness of infants and

young childrencharacterized by acute necrotizing vasculitisof small- and medium-sized vessels.

1. This syndrome is manifest clinically by fever; hemorrhagic edema of conjunctivae, lips,

and oral mucosa; and cervical lymphadenopathy.

2. It can be a cause of coronary artery vasculitis with aneurysm formation.

G. Thromboangiitis obliterans (Buerger disease)is an acute inflammation involving small- to medium-sized

arteriesof the extremities, extending to adjacent veins and nerves. It occurs with greater frequency in Jewish

populations and is most common in young men.

1. This disease results in painful ischemic diseaseoften leading to gangrene.

2. It is clearly exacerbated by heavy cigarette smoking.

H. Lymphomatoid granulomatosisis a rare granulomatous vasculitis characterized by infiltration

by atypical lymphocytoid and plasmacytoid cells. It may progress from a chronic inflammatory

condition to a fully developed lymphoproliferative neoplasm, most often a T-cell non- Hodgkin lymphoma.

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