Diseases of arteries- inflammation

D

Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Infectious Vasculitides: bacterial- Neisserial, Rickettsial, Spirochetal, fungal, viral, Non-Infectious Vasculitides: so-called systemic necrotizing vasculitides (affecting aorta, medium-sized vessels) and small vessel vasculitides (affecting arterioles, venules and capillaries). Pathogenesis involves immune compexes, antineutrophil cytoplasmic antibodies (ANCA) and antibodies to endothelial cells. Classification on the basis of the size of the involved blood vessels, the anatomic site, clinical manifestation and histological characteristics of the lesion:

Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Large Vessel- Vasculitis

Giant cell ateritis

Takayasu art

Medium vessel- vasculitis

Plyarteritis nodosa

Kawasaki

Small vessels- vasculitis

Wegener granulomatosis

Churg Strauses

Microscopic polyangitis

.

Figure 11-23 Diagrammatic representation of the sites of the vasculature involved by the major forms of vasculitis. The widths of the trapezoids indicate the frequencies of involvement of various portions. LCA, leukocytoclastic angiitis. (Reproduced from Jennette JC, and Falk RJ: Small-vessel vasculitis. New Engl J Med 337:1512, 1997.)

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Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides l Vessel Vasculitis

Pathogenesis

(1) Immune complex:

SLE, HSP, Cryoglobulinemia, Goodpasture, Druds, Sjogren

(2) Anti endothelial cell Antibody:

Kawasaki

(3) ANCA Positive: MPA, Wegner G, Chug Struss,

Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Large Vessel Vasculitis

Giant cell (temporal) arteritis: Granulomatous arteritis- aorta, major branches( extracranial branches) of the carotid artery and the temporal artery. Age 50 years polymyalgia rheumatica.Takayasu arteritis (pulseless disease): Granulomatous inflammation of the aorta and its major branches. Usually accurs in patients younger than 50 years.

Giant Cell Arteritis• Rare before age 50

• Throbbing pain

• Visual disturbances

• Temporal artery

• Corticosteroids

• Polymyalgia rheumatica

Giant Cell Arteritis

Figure 11-24 Temporal (giant cell) arteritis. A, H&E stain of section of temporal artery showing giant cells at the degenerated internal elastic membrane in active arteritis (arrow). B, Elastic tissue stain demonstrating focal destruction of internal elastic membrane (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). (C Reproduced from Salvarani C, et al.

Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med 347:261, 2002.)

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Giant Cell Arteritis

Figure 11-24 Temporal (giant cell) arteritis. A, H&E stain of section of temporal artery showing giant cells at the degenerated internal elastic membrane in active arteritis (arrow). B, Elastic tissue stain demonstrating focal destruction of internal elastic membrane (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). (C Reproduced from Salvarani C, et al.

Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med 347:261, 2002.)

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Figure 11-24 Temporal (giant cell) arteritis. A, H&E stain of section of temporal artery showing giant cells at the degenerated internal elastic membrane in active arteritis (arrow). B, Elastic tissue stain demonstrating focal destruction of internal elastic membrane (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). (C Reproduced from Salvarani C, et al.

Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med 347:261, 2002.)

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Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Large Vessel Vasculitis

Takayasu arteritis (pulseless disease): Granulomatous inflammation of the aorta and its major branches. Usually accurs in patients younger than 50 years.

Figure 11-25 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.

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Figure 11-25 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.

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Figure 11-25 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.

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Figure 11-23 Diagrammatic representation of the sites of the vasculature involved by the major forms of vasculitis. The widths of the trapezoids indicate the frequencies of involvement of various portions. LCA, leukocytoclastic angiitis. (Reproduced from Jennette JC, and Falk RJ: Small-vessel vasculitis. New Engl J Med 337:1512, 1997.)

Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 25 October 2005 05:10 AM)

© 2005 Elsevier

Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Medium-Sized Vessel Vasculitis

Polyarteritis nodosa: Necrotizing inflammation( without glomerulonephritis or vasculitis in arterioles, capillaries, or venules) ANCA- negative, HbsAg- positive Vessels- Kidney, heart, liver, GITSegmental fibrinoid necrosisThrombotic occlusion Part of cercumference Branching pts Weak wall- aneurysm, haemorr, infarction, atrophy- organ.

Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Medium-Sized Vessel Vasculitis

Polyarteritis nodosa:Acute-phaseTransmural inflamm- wall( Neutro, Eosin, Monocytes Fibrinoid necrosis, Thrombosis, Late- phase- Fibrosis- up to adventitia

clinical features- children, old

acute, sub acute, chronic

malaise, fever, muscular pains, malena

neuritis

.

Figure 11-26 Representative forms of systemic medium-sized to small vessel vasculitis. A, Polyarteritis nodosa. B, Leukocytoclastic vasculitis. C and D, Wegener granulomatosis. E, Thromboangiitis obliterans (Buerger disease). In polyarteritis nodosa (A), 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. In leukocytoclastic vasculitis (B), shown here from a skin biopsy, there is fragmentation of neutrophils in and around blood vessel walls. In Wegener granulomatosis (C), there is inflammation (vasculitis) of a small artery along with adjacent granulomatous inflammation, in which epithelioid cells and giant cells (arrows) are seen. D, Gross photo from the lung of a patient with fatal Wegener granulomatosis, demonstrating large nodular lesions. In a typical case of Buerger disease (E), the

lumen is occluded by a thrombus containing two abscesses (arrow). The vessel wall is infiltrated with leukocytes. (A, and D, courtesy of Sidney Murphree, MD, Department of Pathology, University of Texas Southwestern Medical School, Dallas, TX; B, courtesy of Scott Granter, M.D., Brigham and Women's Hospital, Boston.)

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Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Medium-Sized Vessel Vasculitis

Kawasaki disease: Arteritis associated with mucocutaneous lymph node syndrome. Coronary arteries are often involved. Aorta and veins may be involved. Usually occurs in children.

Thromoangitis obliterans (Buerger disease): Segmental, thrombosing acute and chronic inflammation of medium-sized and small arteries, principally the tibial artery in younger men who were heavy smokers.

Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Medium-Sized Vessel Vasculitis

Kawasaki disease: Arteritis associated with mucocutaneous lymph node syndrome. Coronary arteries are often involved. Aorta . Usually occurs in childrenRash, Edema of hands and feets.Path: Anti endothelial cell Anbody

Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Medium-Sized Vessel Vasculitis

.Thromoangitis obliterans (Buerger disease): Segmental, thrombosing acute and chronic inflammation tibial artery and radial Art younger men who were heavy smokers.

Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Small Vessel Vasculitis

Wegener granulomatosis: Granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting capillaries, venules, arterioles and arteries. Necrotizing glomerulonephritis is common.Churg-Strauss syndrome: Eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small and medium-sized vessels associated with asthma and blood eosinophilia.Microscopic polyangiitis: Necrotizing vasculitis with few or no immune deposits affecting small and medium-sized vessels. Necrotizing glomerulonephritis is common. Pulmonary capillaritis often occurs. Henoch-Schönlein purpura: Vasculitis with IgA-dominant immune deposits affecting small vessels. Typically involves skin, gut, and glomeruli. Associated with arthralgia or arthritis.Goodpasture syndrome: Glomerulitis and pneumonitis caused by anti-basement membrane of capillaries antibodies.

Wegener’s Granulomatosis• Acute necrotizing granulomas of the upper

and lower reap tract

• Focal necrotizing vasculitis

• Renal disease

• 5th decade of life

• Immunosuppressive therapy

Wegener’s Granulomatosis

Figure 11-26 Representative forms of systemic medium-sized to small vessel vasculitis. A, Polyarteritis nodosa. B, Leukocytoclastic vasculitis. C and D, Wegener granulomatosis. E, Thromboangiitis obliterans (Buerger disease). In polyarteritis nodosa (A), 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. In leukocytoclastic vasculitis (B), shown here from a skin biopsy, there is fragmentation of neutrophils in and around blood vessel walls. In Wegener granulomatosis (C), there is inflammation (vasculitis) of a small artery along with adjacent granulomatous inflammation, in which epithelioid cells and giant cells (arrows) are seen. D, Gross photo from the lung of a patient with fatal Wegener granulomatosis, demonstrating large nodular lesions. In a typical case of Buerger disease (E), the

lumen is occluded by a thrombus containing two abscesses (arrow). The vessel wall is infiltrated with leukocytes. (A, and D, courtesy of Sidney Murphree, MD, Department of Pathology, University of Texas Southwestern Medical School, Dallas, TX; B, courtesy of Scott Granter, M.D., Brigham and Women's Hospital, Boston.)

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Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Small Vessel Vasculitis

.Churg-Strauss syndrome: Eosinophil-rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small and medium-sized vessels associated with asthma and blood eosinophilia.

Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Small Vessel Vasculitis

Microscopic polyangiitis: Necrotizing vasculitis with few or no immune deposits affecting small and medium-sized vessels. Necrotizing glomerulonephritis is common. Pulmonary capillaritis often occurs. .

Figure 11-26 Representative forms of systemic medium-sized to small vessel vasculitis. A, Polyarteritis nodosa. B, Leukocytoclastic vasculitis. C and D, Wegener granulomatosis. E, Thromboangiitis obliterans (Buerger disease). In polyarteritis nodosa (A), 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. In leukocytoclastic vasculitis (B), shown here from a skin biopsy, there is fragmentation of neutrophils in and around blood vessel walls. In Wegener granulomatosis (C), there is inflammation (vasculitis) of a small artery along with adjacent granulomatous inflammation, in which epithelioid cells and giant cells (arrows) are seen. D, Gross photo from the lung of a patient with fatal Wegener granulomatosis, demonstrating large nodular lesions. In a typical case of Buerger disease (E), the

lumen is occluded by a thrombus containing two abscesses (arrow). The vessel wall is infiltrated with leukocytes. (A, and D, courtesy of Sidney Murphree, MD, Department of Pathology, University of Texas Southwestern Medical School, Dallas, TX; B, courtesy of Scott Granter, M.D., Brigham and Women's Hospital, Boston.)

Downloaded from: Robbins & Cotran Pathologic Basis of Disease (on 25 October 2005 05:10 AM)

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Figure 11-26 Representative forms of systemic medium-sized to small vessel vasculitis. A, Polyarteritis nodosa. B, Leukocytoclastic vasculitis. C and D, Wegener granulomatosis. E, Thromboangiitis obliterans (Buerger disease). In polyarteritis nodosa (A), 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. In leukocytoclastic vasculitis (B), shown here from a skin biopsy, there is fragmentation of neutrophils in and around blood vessel walls. In Wegener granulomatosis (C), there is inflammation (vasculitis) of a small artery along with adjacent granulomatous inflammation, in which epithelioid cells and giant cells (arrows) are seen. D, Gross photo from the lung of a patient with fatal Wegener granulomatosis, demonstrating large nodular lesions. In a typical case of Buerger disease (E), the

lumen is occluded by a thrombus containing two abscesses (arrow). The vessel wall is infiltrated with leukocytes. (A, and D, courtesy of Sidney Murphree, MD, Department of Pathology, University of Texas Southwestern Medical School, Dallas, TX; B, courtesy of Scott Granter, M.D., Brigham and Women's Hospital, Boston.)

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Diseases of arterias

D. Inflammatory Disease – Arteritides, Vasculitides Small Vessel Vasculitis

Wegener granulomatosis:Churg-Strauss syndrome:.Microscopic polyangiitis: Henoch-Schönlein purpura: Vasculitis with IgA-dominant immune deposits affecting small vessels. Typically involves skin, gut, and glomeruli. Associated with arthralgia or arthritis.

Goodpasture syndrome: Glomerulitis and pneumonitis caused by anti-basement membrane of capillaries antibodies.

Figure 11-27 Vasculitis with fibrinoid necrosis in a patient with active systemic lupus erythematosus.

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Buerger’s Disease• Thromboangitis obliterans

• Cigarette smoking

• “instep” claudication

Buerger’s Disease

Buerger’s Disease

Buerger’s Disease

Diseases of arterias

E. Raynaud Disease

Paroxysmal pallor or cyanosis of the digits of the hands or feet and infrequently the tips of the nose or ears (acral parts) caused by intense vasospasm of local small arteries or arterioles. Typically in young, otherwise healthy women. No organic changes are present in the arterial walls except late, when intimal proliferation can appear.

Raynaud’s Disease• Small artery, arteriole vasospasm

• White, blue, red

Raynaud’s Disease

Raynaud’s Disease