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13. Vasculature Pathology.pdf
- 1. Diseases of the Blood Vessels Dr Mbayah Etabalé
- 2. Outline 1. The Structure and Function 2. Congenital Anomalies 3. Vascular Wall Cells & their Response to Injury 4. Arteriosclerosis 5. Hypertensive Vascular Disease 6. Atherosclerosis 7. Aneurysms and Dissections 8. Vasculitides 9. Raynaud’s Phenomenon 10. Veins & Lymphatics 11. Tumours 8 mai 2015 2
- 3. 1. The Structure and Function 3 8 mai 2015
- 4. The Cells 1. Endothelial cells 2. Smooth muscle cells The Layers 1. Intima Endothelium Subendothelial connective tissue Internal Elastic Lamina 2. Media Smooth muscle cells Vasa vasora (outer 1/2 – 2/3) External Elastic Lamina 3. Adventitia Connective tissue Nerve endings Vasa vasora 4 What varies 1. Smooth muscle cells 2. Extracellular matrix 8 mai 2015 The Extracellular Matrix
- 5. The Arteries 1. Large/Elastic Arteries Aorta Big branches: Innominate, subclavian, common carotid, iliac Pulmonary artery ↑ elastic fibres – Propulsion 2. Medium- sized/Muscular Arteries Small branches of aorta: coronary, renal ↑ circular/spiralled SM fibres 3. Small Arteries: 2 mm ø Peripheral R 4. Arterioles: 0.02 – 0.1 mm ø Peripheral R 5 8 mai 2015
- 6. The Capillaries 7 – 8 µm ø Endothelium only Dramatically reduced flow Rapid exchange of diffusible substances Highest density in metabolically demanding tissues e.g. myocardium 6 8 mai 2015
- 7. The Veins Post-Capillary Venules • Vascular response to inflammation, diapedesis Collecting venules and veins (small, medium-sized and large) • Capacitance vessels - 2/3 of all blood • Large lumina, thin walls • Poor CT support predisposing to ectasia, compression, degeneration, penetration by tumours/inflammation • Valves prevent reverse flow 7 8 mai 2015
- 8. 8 mai 2015 8
- 9. 9 8 mai 2015
- 10. The Lymphatics Thin-walled and endothelial-lined Drainage for interstitial fluid and inflammatory cells Pathway for disease dissemination: infection, tumours 10 8 mai 2015
- 11. Principles of Vasculature Pathology 1. Narrowing/complete obstruction of the lumina • Progressive – atherosclerosis, arteriosclerosis • Precipitous – thrombosis/embolism 2. Weakening of the vessel wall • Dilatation • Rupture 11 8 mai 2015
- 12. 2. Congenital Anomalies I. Anatomical variation II. Developmental/berry aneurysms III. Arteriovenous fistulae 12 8 mai 2015
- 13. 8 mai 2015 13
- 14. Developmental Aneurysm 14 Occur in cerebral vessels Fatal cerebral haemorrhage 8 mai 2015
- 15. Arteriovenous Malformation Abnormal communication between arteries and veins, bypassing the capillary bed Short-circuits blood from arterial side to venous side Typically occurs in the CNS Causes: Developmental (AVM) Rupture of arterial aneurysm into adjacent vein Penetrating injuries involving adjacent arteries and veins Inflammatory necrosis of adjacent arteries and veins Complication High-output cardiac failure Rupture with haemorrhage 15 8 mai 2015
- 16. 16 8 mai 2015
- 17. 8 mai 2015 17
- 18. 8 mai 2015 18
- 19. Fibromuscular dysplasia Focal irregular thickening of the walls of medium and large muscular arteries Thickening is due to hyperplasia and fibrosis of the intima and media Leads to weak and narrow vessels Aetiology: ?developmental Vessels: renal, carotid, splanchnic, vertebral Ischemia, related to degree of blockage Complications include aneurysm, emboli, and sudden death 8 mai 2015 19
- 20. 3. Vascular Wall Cells & their Response to Injury I. Endothelial cells II. Vascular smooth muscle cells 20 8 mai 2015
- 21. Endothelial Cells Contain Weibel-Palade bodies, vWF storage organelles Loss leads to thrombogenesis and SMC proliferation Activators: Cytokines Bacterial products Haemodynamic stress Lipids Glycosylation products Viruses Complement Hypoxia Acidosis Cigarette toxins 21 8 mai 2015
- 22. Endothelial Dysfunction Activated ECs → 1. Expression of adhesion molecules 2. Vasoconstriction/vasodilatation through autocrine and paracrine action (cytokines, chemokines, GF, vasoactive molecules) 3. Expression of MHC proteins 4. Production of procoagulant moieties 8 mai 2015 22
- 23. Vascular SM Cells Synthesise collagen, elastin, proteoglycans Elaborate GFs, cytokines Responsible for vasoconstriction/vasodilatation Migrate to the intima & proliferate following vascular injury PDGF Endothelin-1 Thrombin FGF IF-γ IL-1 Inhibitors of migration/proliferation Heparan-sulphate NO TGF-β 23 8 mai 2015
- 24. Vascular SMC Response to Injury SMC and SMC precursors (from underlying media or circulating precursors) migrate to the intima Synthesis of ECM forming neointima Resultant thickening of intima Recurrent insults may result in luminal narrowing Eventual regeneration of the overlying endothelium 8 mai 2015 24
- 25. 8 mai 2015 25
- 26. 4. Arteriosclerosis 26 8 mai 2015
- 27. Arteriosclerosis “Hardening of the arteries” Arterial wall thickening and loss of elasticity 3 general patterns 1. Arterio(lo)sclerosis • Small arteries and arterioles • Possible downstream ischaemic injury • Variants: • Hyaline arteriolosclerosis • Hyperplastic arteriolosclerosis 2. Mönckeberg medial calcific sclerosis 3. Atherosclerosis • “gruel” + “hardening” 27 8 mai 2015
- 28. Mönckeberg Medial Calcific Sclerosis Minor clinical significance, as this lesion rarely, if at all, produces vascular narrowing Occurs typically in individuals older than 50 The cause is unknown Usually affects femoral, tibial, radial, ulnar arteries and vascular supply to genitalia Occasionally with bone and bone marrow formation locally 28 8 mai 2015
- 29. Mönckeberg Medial Calcific Sclerosis 8 mai 2015 29
- 30. 5. Hypertensive Vascular Disease 30 8 mai 2015
- 31. Role of Kidneys in BP Regulation RAAS: regulation of PVR; Na+ PGs, NO (relaxing factors) ↓ blood volume → ↓GFR Natriuretic peptides → ↓Na+ reabsorption → diuresis 8 mai 2015 31
- 32. HTN: Types and Causes I. Essential hypertension: 90 – 95% II. Secondary hypertension: I. Renal: AGN; CKD; PCKD; renal artery stenosis; renal vasculitis; “renin-omas” II. Endocrine: Adrenocortical; phaechromocytoma; pituitary; thyrotoxicosis; myxoedema; pregnancy- induced; exogenous III. Cardiovascular IV. Neurologic Malignant/accelerated HTN: severe HTN (>200/120mmHg) with end-organ damage –renal failure, retinal haemorrhage/exudate 8 mai 2015 32
- 33. Clinical Forms of Hypertension 1. Benign HTN 𝐵𝑃 < 200 120 𝑚𝑚𝐻𝑔 Compatible with long life 2. Malignant HTN Aka accelerated hypertension MEDICAL EMERGENCY 𝐵𝑃 ≥ 200 120 𝑚𝑚𝐻𝑔 De novo or on pre- existing benign HTN Rapid end-organ damage: i. Renal dysfunction Proteinuria ii. Hypertensive retinopathy Grade 3 and 4 lesions iii. Hypertensive encephalopathy Untreated, fatal in 1 – 2 years 8 mai 2015 33
- 34. Mechanism of Essential Hypertension Multifactorial 1. Genetic Single gene defects Defects in aldosterone metabolism Mutations in Na+ reabsorption proteins 2. Environmental Stress Obesity Smoking Sedentary lifestyle Heavy salt intake 8 mai 2015 34
- 35. Essential HTN 8 mai 2015 35
- 37. GENETIC ACQUIRED
- 38. Vascular Pathology 1. Large arteries 2. Medium-sized arteries 3. Small arteries – Arteriolosclerosis 8 mai 2015 38
- 39. Large Arteries Degenerative changes in the media Leads to progressive weakening of the vessel wall Possible complications of weakening Aneurysms Dissections Rupture of vessels Accelerated atherogenesis 8 mai 2015 39
- 40. Medium-Sized (Muscular) Arteries HTN leads to increased thickness of the arterial wall Nature of the lesion depends on the type of HTN: benign vs malignant 8 mai 2015 40
- 41. Benign Hypertension 8 mai 2015 41 Progressive thickening of the walls • Symmetrical hypertrophy of the muscular media • Extensive reduplication of the internal elastic lamina • Fibrotic thickening of the intima Consequent luminal narrowing
- 42. Malignant Hypertension 8 mai 2015 42 Severe thickening of the tunica intima in concentric lamellae (“onion-skin” appearance) Consequent luminal narrowing IEL and tunica media remain unchanged
- 43. Small Arteries Hypertension results in thickening of the walls of small arteries - arteriolosclerosis Benign and malignant hypertension lead to distinct histological lesions Benign HTN – hyaline arteriolosclerosis Malignant HTN – hyperplastic arteriolosclerosis 8 mai 2015 43
- 44. Hyaline arteriolosclerosis 8 mai 2015 44 • Endothelial damage leads to leakage of plasma proteins • Normal layers of the wall become ill-defined and replaced by a homogenous pink hyaline thickening with luminal narrowing • Also seen in normotensive elderly people but worse in hypertensives • Leads to ischaemic end-organ damage, most notably, kidneys - hypertensive nephrosclerosis
- 45. Hyperplastic arteriolosclerosis 8 mai 2015 45 The intima undergoes rapid concentric proliferation The endothelium is disrupted with leakage of plasma proteins (fibrinogen) into and beyond the arteriole wall There is obliteration of the wall by intensely eosinophilic amorphous proteinaceous material – “fibrinoid necrosis”/necrotising arteriolitis The lumen is often completely thrombosed
- 46. 6. Atherosclerosis 8 mai 2015 46
- 47. Atheroma Intimal lesions protruding into the vascular lumina Soft yellow grumous core Cholesterol and cholesterol esters Lipid-laden Mø, lipid- laden Lø Necrotic centre, calcification, inflammation Surrounding fibrous cap, SMC Overlying endothelium Disease of: Elastic arteries Muscular arteries Significant pathology in: Coronary artery disease Cerebral circulation 8 mai 2015 47
- 48. Major Risk Factors Non-Modifiable Modifiable Others 1. Increasing age 2. Male sex 3. Family history 4. Genetic abnormalities 1. Hyperlipidaemia 2. Hypertension 3. Cigarette smoking 4. Diabetes 1. C-reactive protein 2. Hyperhomocy- steinaemia 3. Metabolic syndrome 4. Lipoprotein (a) 5. Pro-coagulant factors 6. Lack of exercise 7. Competitive stressful lifestyle 8. Obesity 8 mai 2015 48
- 51. A. Endothelial Injury 1. Haemodynamic disturbance 2. Hyperlipidaemia 8 mai 2015 51
- 52. Haemodynamic Disturbance Smooth laminar flow within vessels induces the endothelium to activate genes whose products are protective e.g. SOD When the flow becomes turbulent, these products reduce exposing the endothelium to injury Turbulent flow typically occurs at ostia of exiting vessels, branch points and along the posterior wall of the abdominal aorta; areas in which plaque formation is typically observed 8 mai 2015 52
- 53. Hyperlipidaemia ↑ cholesterol → ↑ ROS production by EC • → endothelial injury • → consumption of NO Chronic ↑lipidaemia → accumulation of lipoproteins in the intima → ROS oxidisation by Mø and EC • → ingestion by Mø → Foam cells (pathognomonic) • Oxidised LDL stimulates release of GF, cytokines, chemokines → monocyte recruitment • Oxidised LDL is cytotoxic to EC and SM cells → endothelial dysfunction 8 mai 2015 53
- 54. The Stars of the Atherosclerosis Drama 1. Dysfunctional EC 2. Inflammatory cells – Monocytes, Mø, Lø 3. Lipids 4. SMC 8 mai 2015 54
- 55. 8 mai 2015 55
- 56. Evolution of Atherosclerosis 1. Fatty streak 2. Atherosclerotic plaque 8 mai 2015 56
- 57. Fatty Streak 8 mai 2015 57
- 59. 8 mai 2015 59
- 60. Atheroma: Common Sites 1. Abdominal Aorta - AAA 2. Coronary Arteries - MI 3. Popliteal Arteries – Peripheral artery disease 4. Internal Carotid Arteries – Cerebral infarct (stroke) 5. Circle of Willis 8 mai 2015 60
- 61. Fate of an Atheroma 1) Calcification 2) Rupture, ulceration, erosion → thrombosis* 3) Haemorrhage into the plaque* 4) Atheroembolism 5) Aneurysm formation * - acute plaque change 8 mai 2015 61
- 62. 8 mai 2015 62
- 63. Consequences of Atherosclerosis 8 mai 2015 63 Myocardial infarction, cerebral infarction, aortic aneurysm, leg gangrene
- 64. Prevention of Atherosclerotic Disease 1. Primary prevention Delaying atheroma formation Encouraging regression of established lesions in patients who haven’t suffered a serious complication 2. Secondary prevention 1. Prevention of recurrence of complicated events (strokes, MIs) 8 mai 2015 64
- 65. 10 Prevention Risk factor identification and modification 1. Cessation of smoking 2. BP control 3. Weight loss 4. Exercise 5. Lowering (LDL) cholesterol levels 20 Prevention Reduction of recurrent myocardial or cerebral events 1. Antiplatelet agents 2. Statins 3. β-blockers 4. Surgical intervention 8 mai 2015 65
- 66. 7. Aneurysms and Dissections 66 8 mai 2015
- 67. Aneurysm - Definition An abnormal localised dilatation of the heart or blood vessels A true aneurysm is covered by intact/attenuated layers of the blood vessel or heart A false/pseudo-aneurysm is an accumulation of blood bound by connective tissue and formed through a defect in the vessel wall 8 mai 2015 67
- 68. Aneurysm - Shape Wall deformity Aneurysm type Symmetrical stretching of the whole circumference Fusiform aneurysm Segmental stretching of part of the circumference Saccular /berry aneurysm 8 mai 2015 68
- 69. Pathogenesis 1. Poor quality of CT 2. Degradation of blood vessel CT 3. Weakening of blood vessel CT 8 mai 2015 69
- 70. 1. Poor Quality of CT Syndrome Defect Consequence Marfan’s syndrome Defect in synthesis of fibrillin Weak elastic tissue Ehlers-Danlos syndrome Defect in synthesis or structure of fibrillar collagen Abnormal collagen III Scurvy Inadequate collagen hydroxylation No collagen cross-linking → ↓ tensile strength 8 mai 2015 70
- 71. 8 mai 2015 71
- 72. 2. Degradation of blood vessel CT 1. Inflammation ↑MMP synthesis with subsequent destruction of all ECM components ↓TIMP expression 2. Possible polymorphisms in MMP/TIMP 8 mai 2015 72
- 73. 3. Weakening of Blood Vessel CT Ischaemic Intimal atheroma impairing diffusion Systemic HTN narrowing vasa vasora SMC loss → Scarring Loss of elastic fibres Inadequate ECM synthesis Production of abundant amorphous ground substance (glycosaminoglycans) These changes are collectively called cystic medial degeneration 8 mai 2015 73
- 74. 8 mai 2015 74
- 75. Type Common sites Aetiology Comment Atherosclerotic Abdominal aorta Weakening of media due to an atheroma Massive haemorrhage Saccular Cerebral arteries Developmental defect in media and elastic lamina SAH Syphilitic Ascending aorta Damaged media due to syphilitic arteritis Massive haemorrhage Microaneurysm Brain, retina Hypertensive, diabetes small vessel disease Brain, retinal haemorrhages Mycotic (infective) Any artery Damage of media by infected thrombus Haemorrhage Traumatic Scalp or anywhere “duh” False aneurysm 8 mai 2015 75
- 76. Abdominal Aortic Aneurysm Majorly due to atherosclerosis Below renal arteries but above bifurcation of aorta 8 mai 2015 76
- 77. 8 mai 2015 77
- 78. Clinical Consequences AAA Rupture – massive/fatal haemorrhage Obstruction of a branch vessel Iliac Renal Mesenteric Vertebral Embolism (thrombus, atheroma) Impingement on an adjacent structure (ureter, vertebra) Mass mimicking a tumour 8 mai 2015 78
- 79. Thoracic Aortic Aneurysm Aetiology: Hypertension CT disorders Syphilitic Clinical consequence: 1. Mediastinal encroachment 2. Respiratory difficulties – lungs, airways 3. Difficulty in swallowing 4. Coughing – recurrent laryngeal nerve 5. Rib/vertebral pain – erosion 6. Cardiac disease: 1. Aortic valve insufficiency 2. Coronary ostia obstruction 7. Rupture 8 mai 2015 79
- 80. Aortic Dissection Splaying apart of the laminar planes of the tunica media with entry of blood and extension along the length of that vessel May or may not be associated with aortic dilatation Risk factors: • Hypertension • CT disorder: Marfan’s Ehlers Danlos, Scurvy, Cu metabolic defects • Pregnancy • Bicuspid aortic valve • Myxoid medial degeneration from whatever cause • Atherosclerotic (Abdominal aorta) 8 mai 2015 80
- 81. 8 mai 2015 81
- 82. Presentation Severe chest/back pain Hypotension/shock Cardiac tamponade IHD The more common (and dangerous) lesions involve the aorta from the aortic valve to the arch 2 classification systems: DeBakey System Stanford System 8 mai 2015 82
- 83. Classification DeBakey DeBakey I – Ascending aorta DeBakey II – Ascending and descending aorta DeBakey III – Descending aorta Stanford Stanford A – Ascending only or Ascending and descending aorta Stanford B – Descending aorta only 8 mai 2015 83
- 84. 8 mai 2015 84
- 85. 8 mai 2015 85
- 86. 8. Vasculitis 86 8 mai 2015
- 87. Introduction The presence of a primary vascular inflammation The inflammation is either 1. Immune-mediated (95%) 2. Infectious: due to direct invasion of vascular walls by infectious agents (5%) 8 mai 2015 87
- 88. 2. Infectious Vasculitides Localised inflammation due to direct invasion of vascular walls by infectious agents Bacterial, fungal (examples?) Direct extension from adjacent tissue inflammation or haematogenous seeding: a. Septicaemia b. Septic emboli Complications i. Arterial wall weakening and aneurysm formation (mycotic aneurysm) ii. Arterial thrombosis with distal ischaemic injury iii. Spread of infection into tissue outside vessel wall 8 mai 2015 88
- 89. 1. Immune-Mediated Vasculitides Immune-mediated vasculitides can be classified as follows: I. Based on the Immune Mechanism II. Based on Blood Vessel Size 8 mai 2015 89
- 90. I. Immune Mechanisms of Vasculitis a. Immune complex deposition on blood vessel walls b. ANCAs c. Anti-endothelial cell antibodies 8 mai 2015 90
- 91. a. Immune Complex Associated Vasculitis Antibodies are produced against an antigen. Binding of the Ab to Ag leads to the formation of an immune complex The immune complex ends up in the endothelial basement membrane: a. Circulating immune complex is deposited in the BM or; b. Ag is deposited in the BM; circulating Ab then binds the Ag forming an immune complex in the BM The immune complex activates complement system leading to vessel wall inflammation 8 mai 2015 91
- 92. Immune Complex Vasculitis - Examples 1. Drug-induced immune complex vasculitis 2. Vasculitis associated with systemic autoimmune conditions e.g. SLE, RA 3. Infection-induced immune complex vasculitis Hepatitis B sAg in Henoch Schönlein Purpura 8 mai 2015 92
- 93. b. Antineutrophilic Cytoplasmic Ab Most vasculitis patients have circulating Abs against neutrophil cytoplasmic Ags – ANCA 2 types of Abs: 1. c-ANCA (cytoplasmic) Abs target proteinase 3, neutrophil granule constituent Prototypical examples: Wegener’s granulomatosis 2. p-ANCA (perinuclear) Abs target MPO Prototypical examples: Microscopic polyangiitis Churg-Strauss disease 8 mai 2015 93
- 94. c. Anti-endothelial cell Abs Kawasaki disease 8 mai 2015 94
- 95. II. Size of Blood Vessel 1. Large vessel vasculitis – aorta and large branches i. Giant-cell (temporal) arteritis ii. Takayasu arteritis 2. Medium vessel vasculitis – main visceral arteries and branches i. PAN ii. Kawasaki disease 3. Small vessel vasculitis – small arteries, arterioles, capillaries and venules i. Wegener’s granulomatosis ii. Churg-Strauss disease iii. Microscopic polyangiitis 8 mai 2015 95
- 96. 8 mai 2015 96
- 97. Large-Vessel Vasculitis 1. Giant-cell/temporal/granulomatous/cranial arteritis 2. Takayasu disease 8 mai 2015 97
- 98. Case Scenario 1 60 year old male presents with severe headache over the right forehead and loss of vision of the right eye. On examination, there is tenderness overlying vessels on the right side of the forehead. A biopsy of the temporal artery over the tender area is obtained 8 mai 2015 98
- 99. 8 mai 2015 99
- 101. Takayasu’s Arteritis Morphologic features are identical to giant-cell arteritis Only differ in clinical findings and vascular distribution (heavily elastic arteries) 8 mai 2015 101 Vasculitis Temporal arteritis Takayasu’s arteritis Clinical finding > 80% female, Asian > 50 years 10 – 40 years Vascular distribution Branches of the external carotid artery Aorta Left subclavian artery Pulmonary artery
- 102. 8 mai 2015 102
- 103. Medium-sized vasculitides 1. Kawasaki disease 2. PAN 8 mai 2015 103
- 104. Kawasaki Disease Febrile illness of childhood of unknown aetiology 80% less than 4 years of age Self-limiting acute vasculitic syndrome; fatal in only 1% Predilection for coronary arteries → aneurysm → rupture/thrombosis → acute MI Red tongue, lymphadenopathy hence the name mucocutaneous lymph node syndrome Unknown Ag → Delayed-type T-Lø AO → cytokine production/Mø AO Polyclonal B-Lø AO → anti-endothelial/anti-vascular SM Abs 8 mai 2015 104
- 105. 8 mai 2015 105 Straw berry tongue Coronary artery aneurysm
- 106. Poly-Arteritis Nodosa Systemic necrotising vasculitis of medium-sized and small muscular arteries Aetiology: Unknown in 70% 30% associated with chronic hepatitis B HBsAg-HBsAb complexes in the vessel wall Kidney > Heart > Liver > GIT Lesions of different ages/in different stages Segmental lesions, branch points of vessels May cause mural weakening, aneurysm formation and subsequent rupture small aneurysms in segments of a vessel constitute the “rosary sign” 8 mai 2015 106
- 107. 8 mai 2015 107 Segmental inflammatory lesions
- 108. Small-Vessel Vasculitides I. ANCA+ Vasculitis II. ANCA- Vasculitis 8 mai 2015 108
- 109. I. ANCA-Positive Vasculitides 1. Wegener’s granulomatosis 2. Microscopic polyangiitis 3. Churg-Strauss syndrome 4. Drug-induced vasculitis 8 mai 2015 109
- 110. Wegener’s Granulomatosis Triad: 1. Necrotising granulomas of the respiratory tract (nose, sinuses, throat, lungs) 2. Necrotising/granulomatous vasculitis 3. Focal, necrotising gromerulonephritis 8 mai 2015 110
- 111. Wegener’s Granulomatosis 8 mai 2015 111 Unknown inhaled agent → delayed-type T-Lø AO → cytokine production → Mø AO → granuloma formation Positive c-ANCA titres Necrosed blood vessel with surrounding giant cells
- 112. Wegener’s Granulomatosis C/F 1. M > F 2. 40 years of age 3. Pneumonitis: cavitary and nodular lesions 4. Chronic sinusitis 5. Mucosal ulcerations of nasopharynx 6. Renal disease Rx 1. Steroids 2. Cyclophosphamides 3. TNF-antagonists 8 mai 2015 112
- 113. Churg-Strauss Syndrome 8 mai 2015 113 Rare: 1 in 106 Associated with atopic conditions: asthma, allergic rhinitis ↑ Eø Extravascular necrotising granulomas p-ANCA positive in <50%
- 114. Microscopic Polyangiitis All lesions are of the same age/same stage Sites: skin, mucous membranes, lungs, brain, heart, GIT, kidney, muscle Necrotising glomerulonephritis (90% of cases) Pulmonary capillaritis 8 mai 2015 114
- 115. Microscopic Polyangiitis 8 mai 2015 115 Segmental transmural fibrinoid necrosis Small vessels → microscopic infarcts Infiltrating and fragmenting Nø (leucocytoclasis) in post-capillary venules – leucocytoclastic vasculitis
- 116. Atopic features: ↑ Eø, asthma Granulomata Wegener’s Disease Churg-Strauss Syndrome Microscopic PolyAngiitis Small Vessel Vasculitis Present Present Churg- Strauss Syndrome Absent Wegener’s Disease Absent Microscopic Polyangiitis 8 mai 2015 116
- 117. II. ANCA-Negative Vasculitides 1. Henoch-Schönlein purpura 2. Cryoglobulinaemia 3. Non-ANCA small vessel disease i. Paraneoplastic small vessel vasculitis ii. Inflammatory bowel disease vasculitis iii. Immune complex small vessel vasculitis a. Lupus vasculitis b. Rheumatoid arthritis c. Goodpasture’s syndrome d. Sjögren’s syndrome e. Drug-induced immune complex disease f. Behçet disease g. Infection-induced immune-complex disease 8 mai 2015 117
- 118. Serum cryoglobulins Ig A immune complex deposits Henoch- Schönlein Purpura Cryoglobulin aemia Non-ANCA small vessel disease Small Vessel Vasculitis Absent Present Cryoglobulin aemia Absent Non-ANCA small vessel disease Present Henoch- Schönlein Purpura 8 mai 2015 118
- 119. Buerger’s Disease (Thromboangitis Obliterans) And lastly… 8 mai 2015 119
- 120. Thromboangitis Obliterans Segmental, thrombosing, acute and chronic inflammation of medium-sized and small arteries Heavy smokers, usually before 35 Lumen obliterated Extremities most often involved (radial, tibial arteries) Men >>>>> Women Unknown mechanism 8 mai 2015 120
- 122. 9. Raynaud's Phenomenon/Disease 122 8 mai 2015
- 123. Raynaud’s Phenomenon 8 mai 2015 123 Exaggerated vasoconstriction of digital arteries and arterioles Proximal → distal: red → white → blue Primary or secondary
- 124. Primary Raynaud’s Phenomenon (Raynaud’s Disease) Exaggerated central and local (peptidergic) vasomotor response to cold or emotional response Predominantly affects females 3 – 5% of the population Long standing disease leads to atrophy of skin, soft tissue, muscle Other sites: nose tip, ears 8 mai 2015 124
- 125. Secondary Raynaud’s Phenomenon Vascular insufficiency of the extremities secondary to arterial disease caused by other entities: 1. SLE 2. Scleroderma (Systemic sclerosis – CREST syndrome) 3. Buerger’s disease 4. Atherosclerosis 5. Trauma from use of vibratory tools 6. Toxins (ergot alkaloids, α-blockers, PVC monomers) 7. Hyperviscosity syndromes Trophic changes, (ulceration and gangrene) may occur 8 mai 2015 125
- 126. 10. Veins & Lymphatics 1. Varicose veins 2. Phlebothrombosis/thrombophlebitis 8 mai 2015 126
- 127. Varicose veins 8 mai 2015 127 Abnormally dilated tortuous veins Produced by: Prolonged ↑ intraluminal pressure ↓ed vessel wall support Upper and lower legs
- 128. Varicose veins Predispositions Female sex Prolonged dependency Obesity Pregnancy Familial predisposition Stasis → congestion, oedema, pain, thrombosis (but thromboembolism is rare) Chronic sequelae: Ischemic skin changes Stasis dermatitis Varicose ulcers 8 mai 2015 128
- 129. Oesophageal varices 8 mai 2015 129
- 130. Other varicosities Haemorrhoids Cause: Chronic liver disease Primary dilatation at the anorectal junction Pregnancy, chronic constipation Inflammation, thrombosis, ulceration Varicocoele Varicosity involving the pampiniform plexus of the testis 8 mai 2015 130
- 131. Thrombophlebitis Phlebothrombosis Venous thrombosis and inflammation Sites: 1. Deep veins of the legs (> 90%) 2. Periprostatic venous plexus ♀ /pelvic venous plexus ♂ 3. Large veins of the skull and dural sinuses 4. Portal vein 8 mai 2015 131
- 132. DVT Risk Factors: Prolonged immobilisation Congestive heart failure Pregnancy and puerperium Obesity Trauma, surgery Old age Familial thrombophilia OCPs Cancers particularly adenocarcinomas – migratory thrombophlebitis (Trousseau’s sign) (Recall the paraneoplastic syndromes) 8 mai 2015 132
- 133. 8 mai 2015 133
- 134. Vena Caval Syndrome SVCS Neoplasms compressing or invading the SVC Leads to dilatation of head, neck and arm veins; and cyanosis IVCS Compression of IVC by clot propagated from hepatic, renal or lower extremities veins; or tumour (HCC, RCC) Lead to Marked lower extremity distension Distension of superficial lower abdominal veins Renal vein involvement – massive proteinuria 8 mai 2015 134
- 135. 135
- 136. Lymphangitis Commonly group A β-haemolytic streptococci Lymphatics dilated and filled with Nø and Mø Infiltrate may extend into perilymphatic tissue (cellulitis, abscesses) Red painful subcutaneous streaks, painful enlarged draining lymph nodes 8 mai 2015 136
- 137. 8 mai 2015 137
- 138. Lymphoedema Primary 1. Lymphatic agenesis 2. Lymphatic hypoplasia Secondary Aka obstructive lymphoedema Tumours Surgical procedures e.g. axillary dissection Post-irradiation fibrosis Filariasis Post-inflammatory thrombosis and scarring 8 mai 2015 138
- 139. Lymphoedema 8 mai 2015 139 ↑ hydrostatic pressure → ↑interstitial fluid accumulation → ↑interstitial CT deposition → peau d’orange → ulcers (due to inadequate tissue perfusion Obstructed dilated lymphatics can also rupture into cavities: Chylous ascites Chylothorax Chylopericardium Breast cancer What is the translation of the French phrase “peau d’orage”? Note the nipple retraction
- 140. 11. Tumours 140 8 mai 2015
- 141. Benign Intermediate Grade Malignant 1. Capillary haemangioma 2. Cavernous haemangioma 3. Glomus tumour 1. Kaposi sarcoma 1. Angiosarcoma 8 mai 2015 141
- 142. At birth At 2 years after spontaneous regression 142
- 143. Capillary and Cavernous Haemangiomas
- 144. Lymphangioma – cystic hygroma
- 145. Kaposi Sarcoma
- 146. Kaposi Sarcoma
- 147. Kaposi Sarcoma
- 148. End 8 mai 2015 148
- 149. Robbins and Cotran – Pathologic Basis of Disease, Edition 8 Essentials of Anatomic Pathology Muir’s Textbook of Pathology, Edition 14 Sternberg’s Diagnostic Surgical Pathology, 5th Edition Wheater’s Basic Pathology Davidson’s Principle and Practice of Medicine, Edition 19 Cardiovascular Pathology Seminar in Kenya by Dr Debra L. Kearney Wikipedia 8 mai 2015 149 References