BLOOD VESSELS:

VASCULITIS

Classification and Characteristics of Selected Immune-Mediated Vasculitides

Vasculitis Type[*] Examples Description

LARGE-VESSEL VASCULITIS Giant-cell (temporal) arteritis Granulomatous inflammation; frequently involves
the temporal artery. Usually occurs in patients older than age 50 and is associated with polymyalgia
rheumatica.
Aorta and large branches to extremities, head, and neck Takayasu arteritis Granulomatous inflammation
usually occurring in patients younger than age 50
MEDIUM-VESSEL VASCULITIS Polyarteritis nodosa Necrotizing inflammation typically involving renal
arteries but sparing pulmonary vessels
Main visceral arteries and their branches Kawasaki disease Arteritis with mucocutaneous lymph node
syndrome; usually occurs in children. Coronary arteries can be involved with aneurysm formation and/or
thrombosis.
SMALL-VESSEL VASCULITIS Wegener granulomatosis Granulomatous inflammation involving the
respiratory tract and necrotizing vasculitis affecting small vessels, including glomerular vessels. Associated
with PR3-ANCAs.
Arterioles, venules, capillaries, and occasionally small arteries Churg-Strauss syndrome Eosinophil-rich
granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small
vessels. Associated with asthma and blood eosinophilia. Associated with MPO-ANCAs.
Microscopic polyangiitis Necrotizing small-vessel vasculitis with few or no immune deposits; necrotizing
arteritis of small and medium-sized arteries can occur. Necrotizing glomerulonephritis and pulmonary
capillaritis are common. Associated with MPO-ANCAs.

Diagrammatic representation of the typical vascular sites involved with the more common forms
of vasculitis, as well as the presumptive etiologies. Note that there is a substantial overlap in
distributions. ANCA, antineutrophil cytoplasmic antibody; SLE, systemic lupus erythematosus.

Giant-cell (temporal) arteritis. A, H&E stain of section of temporal artery showing giant
cells at the degenerated internal elastic lamina in active arteritis (arrow). B, Elastic
tissue stain demonstrating focal destruction of internal elastic lamina (arrow) and
intimal thickening (IT) characteristic of long-standing or healed arteritis. C,
Examination of the temporal artery of a patient with giant-cell arteritis shows a
thickened, nodular, and tender segment of a vessel on the surface of head (arrow).

Takayasu arteritis. A, Aortic arch angiogram showing narrowing of brachiocephalic, carotid,

and subclavian arteries (arrows). B, Gross photograph of two cross-sections of the right
carotid artery taken at autopsy of the patient shown in A, demonstrating marked intimal
thickening with minimal residual lumen. C, Histologic view of active Takayasu aortitis,
illustrating destruction of the arterial media by mononuclear inflammation with giant cells
(arrows).

Polyarteritis nodosa. There is segmental fibrinoid necrosis and thrombotic

occlusion of the lumen of this small artery. Note that part of the vessel wall
at the upper right (arrow) is uninvolved.

Representative forms of ANCA-associated small-vessel vasculitis. A, Leukocytoclastic

vasculitis (microscopic polyangiitis) with fragmentation of neutrophils in and around
blood vessel walls. B and C, Wegener granulomatosis. B, Vasculitis of a small artery
with adjacent granulomatous inflammation including epithelioid cells and giant cells
(arrows). C, Gross photo from the lung of a patient with fatal Wegener
granulomatosis, demonstrating large nodular centrally cavitating lesions.

Thromboangiitis obliterans (Buerger disease). The lumen is

occluded by a thrombus containing abscesses (arrow), and the
vessel wall is infiltrated with leukocytes.

Raynaud's phenomenon. A, Sharply demarcated pallor of the distal fingers

resulting from the closure of digital arteries. B, Cyanosis of the fingertips.

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