Non-atherosclerotic Vascular Disease

1. Non-atherosclerotic Vascular Disease

2. Vasculitis-inflammation of the walls of vessels • Fever, myalgias, arthralgias and malaise • Immune-mediated inflammation • Invasion of vascular walls by infectious pathogens

3. Inflammation of vessel wall with endothelial damage In this case by Rickettsial organisms the classic Typhus nodule-

4. Role of Immune Complexes • DNA-anti-DNA complexes are found in vessels in SLE • Hypersensitivity to drugs cause approximately 10% of vasculitic skin lesions • Vasculitis associated with viral infections: HBsAg-antiHBsAg

5. Antineutrophil Cytoplasmic Antibodies • Antibodies against granules of neutrophils • C-ANCA- cytoplasmic staining-proteinase 3 • Seen in Wegener’s • P-ANCA- perinuclear staining-myeloperoxiduse • Microscopic polyangitis and Churg-Strauss

7. Classification of Vasculitis • Large vessel vasculitis • Giant cell arteritis • Takayasu arteritis • Medium sized vessel vasculitis • Polyarteritis nodosa (PAN) • Kawasaki disease

8. Small vessel vasculitis • Wegener granulomatosis • Churg -Strauss syndrome • Microscopic polyangiitis • Henoch-Schonlein purpura • Essential cryoglobulinemic vasculitis • Cutaneous leukocytoclastic angiitis

9. Giant Cell Arteritis-(temporal) • Arteries of head • Inflammation of vessel with giant cells • Breakdown of elastic fibers • Fever, headaches, facial pain, vision disturbances

10. Giant cell arteritis

11. Giant cell arteritis- granulomatous reaction against elastic fibers

12. Giant cell arteritis

15. Takayasu Arteritis- Pulseless Disease • Ocular disturbances • Marked weakening of the pulses • Narrowing of arteries of the arch • Granulomatous inflammation • Unknown cause

16. Takayasu’s arteritis

17. Takayasu arteritis

18. Polyarteritis nodosa • Transmural necrotizing inflammation of small or medium size arteries • Focal, random and episodic • May produce aneurysmal dilation, nodularity and obstruction • Varied symptoms • 30% have hepatitis Bs antigen • Little association with ANCA

19. Polyarteritis nodosa

20. Polyarteritis nodosa long standing with thickening of the wall

21. Healed polyarteritis with occlusion

22. Kawasaki Syndrome (mucocutaneous lymph node syndrome) • Young children (<4) • Fever, oral erythema, edema of hands & feet • Erythema of the palms & soles • Viral immune cross reaction (retrovirus) • Up to 20% develop coronary lesions-aneurysms, occlusions & MI

23. Microscopic Polyangitis • Arterioles, capillaries and venules • Acute inflammations, few or no immune deposits • Most have P-ANCA • Most clear up by removing offending agent

24. Small vessel arteritis

25. Wegener Granulomatosis • Acute necrotizing granulomas of upper respiratory tract, lower respiratory tract • Focal necrotizing vasculitis of medium size arteries • Focal necrotizing, crescentic glomerulonephritis • Most have C-ANCA

26. Wegener’s granulomatosis

27. Thromboangititis obliterans (Buerger’s Disease) • Segmental, thrombosing, acute and chronic inflammation of medium sized arteries • Upper extremities • Men who are heavy cigarette smokers

28. Buerger disease (thromboangititis obliterans) Most upper extremity Only in smokers

29. Raynaud Disease • Paroxysmal pallor or cyanosis of the digits, tips of nose or ears • Intense vasospasm of small arteries-young healthy women • No organic change in the vessels

30. Raynaud Disease - no underlying vascular damage Raynaud phenomenon - underlying pathology