1. Diseases of arteries- inflammation

2. 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:

3. 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 .

4. Figure 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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5. 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,

6. 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.

7. Giant Cell Arteritis Rare before age 50 Throbbing pain
Visual disturbances
Temporal artery
Corticosteroids
Polymyalgia rheumatica

8. Giant Cell Arteritis

9. Figure 11-24 Temporal (giant cell) arteritis
Figure 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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10. Giant Cell Arteritis

11. Figure 11-24 Temporal (giant cell) arteritis
Figure 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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12. Figure 11-24 Temporal (giant cell) arteritis
Figure 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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13. 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.

14. Figure 11-25 Takayasu arteritis
Figure 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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15. Figure 11-25 Takayasu arteritis
Figure 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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16. Figure 11-25 Takayasu arteritis
Figure 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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17. Figure 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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18. 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, GIT
Segmental fibrinoid necrosis
Thrombotic occlusion
Part of cercumference
Branching pts
Weak wall- aneurysm, haemorr, infarction, atrophy- organ .

19. 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 .

20. Figure 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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21. 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.

22. 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 children
Rash, Edema of hands and feets
.Path: Anti endothelial cell Anbody

23. 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.

25. 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.

26. Wegener’s Granulomatosis
Acute necrotizing granulomas of the upper and lower reap tract
Focal necrotizing vasculitis
Renal disease
5th decade of life
Immunosuppressive therapy

27. Wegener’s Granulomatosis

28. Figure 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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29. 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.

30. 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. .

31. Figure 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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32. Figure 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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33. 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.

34. Figure Vasculitis with fibrinoid necrosis in a patient with active systemic lupus erythematosus.
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35. Buerger’s Disease Thromboangitis obliterans Cigarette smoking
“instep” claudication

36. Buerger’s Disease

37. Buerger’s Disease

38. Buerger’s Disease

39. 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.

40. Raynaud’s Disease
Small artery, arteriole vasospasm
White, blue, red

41. Raynaud’s Disease

42. Raynaud’s Disease