Diseases of the Arterial Wall

I Cellular and Molecular Biology of the Normal Arterial Wall.- 1 Endothelial and Smooth-Muscle Cells.- Structure of the Normal Arterial Wall.- The Endothelial Cell.- Biological Behaviour and Morphology of Endothelial Cells.- Functional Properties of Vascular Endothelium.- The Smooth-Muscle Cell.- Biological Behaviour of Smooth-Muscle Cells.- Functional Morphology of Vascular Smooth Muscle.- Interactions Between Endothelial and Smooth-Muscle Cells.- Communication Between Endothelial and Smooth-Muscle Cells Mediated Via Humoral Mechanisms.- Communication Between Endothelial and Smooth-Muscle Cells Mediated Via Direct Cell-to-Cell Contacts.- References.- 2 Extracellular Matrix of the Arterial Vessel Wall.- Collagens.- Biochemistry.- Morphology.- Elastin.- Biochemistry.- Morphology.- Proteoglycans.- Biochemistry.- Morphology.- Structural Glycoproteins.- Biochemistry.- Morphology.- General Arrangement of Extracellular Matrix of the Vascular Wall.- References.- 3 Organogenesis of the Arterial Wall.- Structural Organization of Vessel Walls.- Early Development of the Circulation.- Structural Organization of the Arterial Wall.- Static Mechanics.- Scleroproteins in the Aortic Wall.- Developmental Implications.- Arterial Wall Nutrition.- Exceptional Areas in Vascular Development.- Ductus Arteriosus.- Coarctation.- Coronary Vessels and Vascular Cushions.- Conclusions.- References.- 4 Age-Related Morphological Changes of the Arterial Wall.- "Elastic"-Type Arteries.- "Muscular"-Type Arteries.- Assessment of Ageing of the Arterial Wall.- References.- 5 Effects of "Ageing" on Arterial Cells In Vitro.- Cell Culture Techniques.- Smooth-Muscle Cells.- Endothelial Cells.- Growth of Arterial Cells.- Smooth-Muscle Cells.- Endothelial Cells.- Growth Factors and the Proliferation of Arterial Cells.- Metabolic Activities of Arterial Cells.- Glycosaminoglycans and Proteoglycans.- Fibrous Proteins: Collagen and Elastin.- Low-Density Lipoproteins.- Conclusion.- References.- 6 Structural Modifications of the Arterial Wall in Hypertension.- Structural Modifications of the Arterial System as a Consequence of Hypertension.- Structural Modifications of the Arterial Wall as a Result of High Blood Pressure.- Complications: Lacunes and Atherosclerosis.- Effect of Treatment.- Similarity Between the Ageing Process and Hypertension in the Arterial Wall.- References.- 7 Adrenergic and Non-adrenergic Neural Control of the Arterial Wall.- Structure of the Vascular Neuroeffector Junction.- Pattern of Perivascular Innervation.- Development and Regeneration of Perivascular Innervation.- Adrenergic Nerves.- Cholinergic Nerves.- Non-adrenergic, Non-cholinergic Nerves.- Purinergic Nerves.- Aminergic Nerves.- Peptidergic Nerves.- Neuromodulation and Co-transmission.- Concluding Comments.- References.- 8 The Arterial Wall and the Haemostatic Process.- Thromboresistance of the Endothelium.- Thromboresistance in Relation to Primary Haemostasis.- Thromboresistance in Relation to Plasma Coagulation.- Thromboresistance in Relation to Fibrinolysis.- The Thrombogenicity of the Subendothelium.- Ex Vivo Study of the Thrombogenicity of the Subendothelium.- In Vitro Study of the Vascular Thrombogenic Macromolecules.- Structural Determinants of Collagen and Microfibrils Involved in the Thrombogenicity of Subendothelium.- In Vivo Studies on the Thrombogenicity of Subendothelium.- Mechanism of Platelet Activation.- Conclusion.- References.- 9 The Vascular Wall and Hormonal Control of Vasomotor Function.- Vasoactive Peptide Metabolism of the Vascular Endothelium.- Converting Enzyme or Kininase II.- Does a Complete Renin-Angiotensin System Exist in the Vascular Endothelium?.- Other Peptidases of the Vascular Endothelium.- Modulation by the Endothelium of the Physiological and Pharmacological Activity of Vasoactive Compounds.- Stimulation of Prostaglandin Formation by Vasoactive Agents in the Vascular Endothelium.- Vasomotor Reactions Depending on the Vascular Endothelium.- Physiological and Physiopathological Consequences of the Role of the Endothelium in Controlling Vasomotor Activity.- Endocrine Function of the Smooth-Muscle Cell: The Renin-Secreting Cell.- Morphology of Renin-Secreting Cells.- Renin Structure and Renin Precursor Maturation.- Role of Renin Secretion.- Note Added in Proof.- References.- II Pathophysiology of Experimental and Human Atherosclerosis.- 10 The Lesions of Atherosclerosis.- Early Changes, Fatty Streaks and Gellatinous Elevations.- The Fibroatheromatous Plaque and Early Established Lesion.- The Complicated Lesion.- The Pathogenesis of the Lesions.- The Role of Lipids.- References.- 11 Atherosclerosis: Cellular Aspects.- Role of Endothelium.- Modifications of the Endothelium in Pathological Conditions.- Endothelial Dysfunction as a Predisposing Factor for the Development of Atherosclerosis.- Role of Smooth-Muscle Cells.- The Arterial Smooth-Muscle Cell in Experimental Atherosclerosis.- Arterial Smooth-Muscle Cells in Human Atherosclerosis.- Growth Factors for Arterial Smooth-Muscle Cells.- Monoclonality of Atheromatous Smooth-Muscle Cells.- The Role of the Macrophage.- Conclusions.- References.- 12 Extracellular Matrix Changes in Atherosclerosis.- Collagens.- Proteoglycans.- Elastin.- Glycoproteins.- Summary.- References.- 13 Lipid Accumulation in the Vessel Wall.- Lesions Induced by Injury.- Injury from Indwelling Aortic Catheter.- Immunological Injury.- Inhibition of Lesion Development.- Balloon-Catheter Removal of the Endothelium.- Lipid Accumulation in Lesions.- Lipid-Containing Lesions Following Vascular Injury in Man.- Relationship of Injury to "Early" Human Atherosclerotic Lesions.- Mechanisms of Lipid Accumulation in Injury-Induced Lesions.- Proteoglycans and Lipid Accumulation.- Modification of Low-Density Lipoprotein.- Qualitative Change in Proteoglycans and Lipoprotein Binding.- Alterations of Lipoprotein and Vessel Wall Uptake.- Summary.- References.- 14 Role of Platelets in Atherosclerosis and its Complications.- Factors in Endothelial Injury.- Metabolic Factors.- Toxic Factors.- Immunological Factors.- Infectious and Viral Factors.- Role of Mononuclear Cells.- Role of Platelets in Atherosclerosis.- A Few Data on PDGF and its Role.- Progression of the Atherosclerotic Lesion.- Involvement of Haemostatic Factors in the Complication of the Arterial Lesions.- Thrombosis.- Thromboembolic Occlusion.- Arterial Spasm.- Conclusion.- References.- 15 Mechanical Factors in the Pathogenesis, Localization and Evolution of Atherosclerotic Plaques.- The Mechanical Forces.- Blood Flow.- Pressure and Wall Tension.- Pulsatile Flow.- The Physical State of the Plaque.- Mechanical Stresses and the Distribution of Intimal Plaques.- Methodological Problems.- Fluid Dynamic Characteristics.- Blood Pressure and Tensile Stress.- Proposed Mechanisms for Plaque Initiation.- Endothelial Disruption.- Wall Shear Stress.- Transport Through the Artery Wall.- Mechanical Factors in Lesion Progression.- Secondary Disruption.- Artery Adaptation.- Mechanical Factors in Plaque Complication.- Direct Interventions on Plaques.- References.- 16 Progression, Topographical Aspects and Regression of Atherosclerosis.- Natural History and Progression of Atherosclerosis.- Progression.- Complications.- Specific Findings.- Problems of Regression.- References.- III Management of Patients with Atherosclerosis.- 17 A Pharmacological Approach to Degenerative Alterations of Arterial Walls.- The Primary Effects of Drugs on Arteries.- Role of Exaggerated Constriction of Arteries in Vascular Disease.- Response of Arteries to Acute Pharmacological Interventions.- Differences in the Response of Large and Small Arteries to Pharmacological Interventions.- Arterial Consequences of the Physiological Counter-regulations Induced by Drugs.- Long-term Effects of Drugs on Arteries.- Secondary Effects of Drugs on the Function of Arteries.- Conducting Function.- Buffering Function.- Baroreflex Function.- References.- 18 The Arterial Wall: Relationships Between Haemodynamic and Structural Aspects in Man.- Methods of Studying the Vascular System in Man.- Linear Model of the Vascular System.- Measuring the Diameter of Arteries of Large Calibre.- Haemodynamic Consequences of Diseases of the Large Arterial Trunks.- Reduction of Arterial Compliance.- Increase in Systolic Pressure.- Reversibility and Aggravation of Haemodynamic Disorders.- Restructuring of the Vascular System in Permanent Essential Systolo-Diastolic Arterial Hypertension.- Arguments in Favour of Structural Modifications in the Cardiovascular System of the Hypertensive Subject.- Reversibility of Structural and Haemodynamic Alterations of the Cardiovascular System in Hypertensive Subjects.- References.- 19 The Epidemiology of Atherosclerosis.- Atherosclerosis in Human Populations.- Temporal Variations.- The Search for Associations.- Secular and Geographical Relationships.- Group Comparisons.- The Search for Causes.- The Classical Risk Factors.- Other Risk Factors.- Risk Factors of the Other Locations of Atherosclerosis.- Conclusions and Hypotheses.- Preventive Trials.- Treatment of Hypertension.- Nutritional Prevention.- Hypocholesterolaemic Treatments.- Multifactorial Prevention.- Prevention: A Public Health Strategy.- References.- 20 Risk Factors for Atherosclerosis: A Clinician's Pragmatic Appraisal.- Ignorance and Concern.- Genesis of Risk Factors.- Definition and Nature of Risk Factors.- Meaning of Risk Factors.- Correlation and Causation.- Arterial Diseases and Atherosclerosis.- Triglycerides and Atherosclerosis.- Possible Mechanisms of Action for Risk Factors.- Development of Atherosclerotic Plaques.- Complications of Atherosclerotic Plaques.- Consequences of Arterial Obstructions due to Atherothrombosis.- Other Unsolved Issues.- Do Risk Factors Exert the Same Influence in all Populations?.- Are Risk Factors Similar for all Arterial Localizations of Atherosclerosis.- Is Amending Risk Factors Controlling Atherosclerosis?.- Hypercholesterolaemia: A Nearly Perfect Example of a Risk Factor.- Agreement.- Disagreement.- Conclusion.- References.- 21 Diagnostic Principles and Therapeutic Management of Patients with Atherosclerosis.- Lower Limb Arteriopathies.- Diagnosis.- Clinical Diagnosis.- Investigations.- The Strategy of Investigation.- Principles of Treatment.- Treatment of Atherosclerosis.- Treatment of Ischaemia.- References.- Arteriosclerotic Lesions of Carotid Arteries.- Haemodynamic Factors.- Pathophysiology of Cerebrovascular Thrombosis.- Mechanism of Vascular Occlusion.- The Diagnosis of Cerebral Ischaemia Due to Thrombosis.- Clinical Features.- Carotid Bruits.- The Role of Computerized Axial Tomography.- Differential Diagnosis.- Transient Ischaemic Attacks.- Initial Approach.- Clinical Aspects and the Limits of Classification: Prolonged Reversible Ischaemic Neurological Deficits (PRIND) and Lacunar Infarction.- Ancillary Investigations.- Treatment.- Cerebrovascular Infarction.- Diagnosis.- Clinical Course.- Initial Medical Therapy.- The Role of Surgery in Carotid Artery Stenosis.- Long-Term Medical Therapy.- References.- Atheromatous Stenosis of Renal Arteries.- Strategy of Diagnosis.- Diagnosis of Renovascular Hypertension.- Diagnosis of the Stenosis.- Renal Consequence of the Stenosis.- Diffusion of the Disease.- Therapeutic Approach.- References.- Coronary Arteries.- Diagnosis.- Clinical Findings.- Complementary Investigations.- Arguments in Favour of a Diagnosis of Coronary Atherosclerosis.- Treatment.- References.- IV The Arterial Wall in Diabetes.- 22 Clinical and Physiopathological Features in Diabetic Vascular Disease.- Links Between Diabetes and Arterial Disease.- Pathogenic Factors.- Epidemiological Factors and Prevention.- Specific Organic Involvement.- Coronary Heart Disease.- Diabetic Foot and Peripheral Arterial Disease.- Cerebrovascular Disease.- Sexual Impotence.- Conclusion.- References.- 23 Diabetic Retinopathy.- Predisposing Factors.- Metabolic Disturbance.- Genetic Considerations.- Arterial Disease.- Duration.- Pre-retinopathy.- Breakdown of the Blood-Retinal Barriers.- Increased Blood Flow.- Background Retinopathy.- Venous Dilatation.- Oedema.- Exudates.- Haemorrhages.- Microaneurysms.- Capillary Non-perfusion.- Pericyte Degeneration.- Basement Membrane Thickening.- Proliferative Retinopathy.- Intraretinal Vascular Proliferation.- Pre-retinal Fibrovascular Proliferation.- Conclusion.- References.- 24 Diabetic Nephropathy.- Anatomopathological Features.- Gross Anatomy.- Light Microscopy.- Electron Microscopy.- Immunohistochemistry.- Specificity of Diabetic Glomerulosclerosis.- Superimposed Renal Lesions.- Papillary Necrosis.- Clinical Manifestations.- Pre-clinical Phase.- Clinical Phase.- Pathogenesis.- Genetic Factors.- Role of Insulin Deficiency and Hyperglycaemia.- Haemodynamic Factors.- References.- 25 Vascular Connective Matrices and Haemostasis in Diabetic Angiopathy.- Glycoprotein Biosynthesis Modification.- Increase in the Biosynthesis of Glycoproteins.- Decrease of Glycoprotein Degradation.- Metabolic Deviation: The Polyalcohol Pathway.- Non-enzymatic Glycosylation: Molecular and Cellular Consequences.- Non-enzymatic Glycosylation (NEG) of Proteins.- Incidence in the Vascular Connective Matrix.- Endothelial Cells, Platelets and Haemostasis in Diabetic Angiopathy.- Endothelial Cells.- Platelet Function and Coagulation Factors in Diabetic Microangiopathy.- Conclusion.- References.- V Inflammatory Diseases of the Arterial Wall.- 26 Immunological Processes in the Vascular Wall.- Effector Mechanisms in Immunologically Mediated Vascular Damage.- Immune Complexes and Antibodies to Vascular Structures.- Complement.- Cellular Effector Mechanisms.- Endothelial Cells.- Interactions Between Immunological Effectors and the Coagulation and Fibrinolytic Systems.- Experimental Models of Immunologically Mediated Vascular Lesions.- Immunologically Mediated Vascular Lesions in Man.- Laboratory Assessment of Immunologically Mediated Vascular Lesions.- In Situ Analysis.- Assessment of Humoral Abnormalities Associated with Immunologically Mediated Vasculitis.- References.- 27 The Vasculitis Syndromes in Small- and Medium-Sized Vessels.- Lesions and Mechanisms.- Lesions.- Mechanisms.- Classification.- Clinical and Nosological Aspects.- The Vasculitis Syndrome.- Diagnostic Considerations.- "Primary" Vasculitis.- Angiitis Secondary to Known Aetiological Agents.- Necrotizing Angiitis in Connective Tissue Disease and in Malignant Disorders.- Localized Angiitis.- References.- 28 The Vasculitis Syndromes in Aorta and Large Arteries.- Pathological Aspects.- Takayasu's Disease.- Pathological Features.- Sites of Involvement and Visceral Consequences.- Associated Findings.- Aetiological Factors.- Temporal Arteritis (Temporal Arteritis/Polymyalgia Rheumatica Syndrome).- Pathological Aspects.- Immunopathological Aspects.- Patterns of Arterial Involvement.- Thromboangiitis Obliterans (Von Winiwarter-Buerger's Disease).- Pathological Features.- Pattern of Arterial Involvement.- Aetiological Factors.- Infectious Aortitis and Arteritis.- Syphilitic Aortitis.- Tuberculous Aortitis and Arteritis.- Aortitis and Arteritis in Systemic Diseases and Miscellaneous Conditions.- Behcet's Syndrome.- Cogan's Syndrome.- Kawasaki Disease.- Rheumatic Fever.- Rheumatoid Arthritis.- Ankylosing Spondylitis.- Reiter's Syndrome.- Relapsing Polychondritis.- Periaortitis or Inflammatory Aneurysms of the Aorta.- Miscellaneous Conditions.- Non-specific Aortitis.- References.- Principles of Diagnosis and Therapeutic Approach.- Takayasu's Disease.- Principles of Diagnosis.- Therapeutic Approach.- Temporal Arteritis.- Clinical Diagnosis.- Other Investigations.- Strategy of Exploration.- Treatment.- Buerger's Disease.- Clinical Features.- Special Investigations.- Strategy of Diagnosis.- Principles of Treatment.- References.- VI Arteriopathies of Diverse Origin.- 29 Fibromuscular Arterial Dysplasias Pathological Aspects and Current Concepts.- FMD of the Renal Artery.- Background Histopathology.- Complications.- FMD of the Renal Artery in Children.- FMD of the Renal Artery of Transplanted Kidney.- FMD of the Cervicoencephalic Arteries.- Other Sites of FMD.- Pathophysiology.- FMD is a Systemic Arterial Disease.- The Predilection for FMD to Occur in Elective Sites Suggests a Role for Local Factors.- FMD Affects Mainly the Media of Middle-Sized Muscular Arteries.- References.- Principles of Diagnosis and Therapeutic Approaches.- Internal Carotid Artery.- C.F. Degos.- Clinical Characterization.- Angiographic Characteristics.- Therapeutic Approach.- Renal Artery.- J.-B. Michel, P. Lagneau and J. Ménard.- Strategy of Diagnosis.- Therapeutic Approach.- References.- 30 Cardiovascular Alterations in Inherited Connective Tissue Disorders.- Fibrous Protein Diseases.- Marfan's Syndrome.- Ehlers-Danlos Syndrome (EDS).- Cutis Laxa.- Osteogenesis Imperfecta (OI).- Pseudoxanthoma Elasticum (PXE).- Storage Diseases.- Polysaccharidoses.- Mucolipidoses.- Glycoproteinoses.- Lipidoses.- Inborn Metabolic Diseases with Secondary Effects on Fibrous Proteins.- Homocystinuria.- Alcaptonuria.- Menkes Syndrome.- Various Hereditary Disorders Affecting Connective Tissue and the Vascular Network.- References.- 31 Aneurysms and Arterial Dissections.- Aortic Aneurysms.- Extra-aortic Aneurysms.- Arterial Dissections/Dissecting Aneurysms.- Aortic Dissections.- Extra-aortic Dissecting Aneurysms.- References.- 32 Traumatic Arterial Disease.- Direct Trauma to Arteries.- Arterial Lesions.- Concomitant Lesions.- Results of Arterial Lesions.- Traumatic Mechanisms and Agents.- Arterial Injuries Due to Biological Effects of Certain Physical Agents.- Arterial Injuries Caused by Electromagnetic Radiation.- Effects of Vibration.- Effects of Cold.- Electrocution.- Injuries Caused by High Pressure.- References.- 33 Perioperative Arterial Changes.- Disorders of Operated Arteries Related to Surgical Procedures.- Arterial Exposure.- Arterial Clamping.- Arterial Ligation.- Arteriotomy.- Arterial Suture.- Arterial Anastomosis.- Pathology of the Operated Artery Due to Inclusion of a Foreign Body in the Wall of the Artery.- Pathological Changes in the Operated Artery Due to Interactions Between the Blood and the Artery Wall.- Thromboendarterectomy.- Arterial Embolectomy.- Transluminal Angioplasty.- Patch-Graft Angioplasty.- Bypass by Graft or Prosthesis.- References.- VII Pathology of the Pulmonary Arteries.- 34 Pulmonary Vasculitides.- Wegener's Granulomatosis (WG).- Allergic Granulomatosis and Angiitis (Churg-Strauss Syndrome).- Necrotizing Sarcoid Granulomatosis (NSG).- Lymphomatoid Granulomatosis (LYG).- Bronchocentric Granulomatosis (BCG).- Pulmonary Hyalinizing Granuloma.- Conclusion.- References.- 35 Pulmonary Hypertension.- The Normal Pulmonary Artery and Circulation.- Normal Macroscopic Morphology and Morphometry.- Normal Microscopic Morphology and Morphometry.- Growth and Development.- Age-Related Changes.- Normal Physiology.- Pulmonary Hypertension and the Pulmonary Artery.- General Features and Morphological Grading.- Pulmonary Hypertension in Congenital Cardiac Left to Right Shunts.- Primary Plexogenic Pulmonary Arteriopathy.- Pulmonary Hypertension Due to Thromboembolic Disease.- Pulmonary Veno-Occlusive Disease and Capillary Haemangiomatosis.- Pulmonary Capillary Haemangiomatosis.- Pulmonary Hypertension Secondary to Pulmonary Venous Hypertension.- Hypoxic Pulmonary Hypertension.- Pulmonary Hypertension in Parenchymal Lung Disease.- References.- VIII Angiodysplasias, Tumours and Tumour-like Lesions of the Blood Vessels.- 36 Arteriovenous Malformations and Arterial Dysplasias.- Arteriovenous Malformations.- The Relationship Between Haemangiomas and Vascular Malformations.- Pathophysiology.- Arteriovenous Malformations in the CNS.- Arteriovenous Malformations of the Spinal Cord.- Cobb Syndrome.- Aneurysm of the Great Vein of Galen.- Some Specific Entities.- Arterial Dysplasias.- Idiopathic Arterial Calcification in Infancy.- Supravalvular Aortic Stenosis.- Macaroni Arteries.- Neurofibromatosis.- Angiodysplasia of the Colon.- Other Gastrointestinal Vascular Anomalies.- Intracranial Aneurysms.- References.- 37 Tumours and Tumour-like Lesions.- Tumour-like Lesions.- Pseudohaemangiomas.- Subadventitial Cystic Degeneration of the Popliteal Artery (Cystic Disease of the Popliteal Artery, Adventitial Mucoid Cyst).- Haemangiomas.- Capillary Haemangiomas.- Cavernous Haemangiomas.- Venous Haemangioma.- Specific Types.- Other Benign Tumours.- Miscellaneous Parietal Tumours.- Glomus Tumours and Glomangiomatosis.- Vascular Leiomyoma.- Tumours of the Juxtaglomerular Apparatus (J.-P. Camilleri).- Tumours with Malignant Potential.- Epithelioid Haemangioendothelioma.- Haemangiopericytoma.- Chemodectomas/Paragangliomas.- Malignant Tumours.- Angiosarcoma.- Angioendotheliosarcoma.- Leiomyosarcoma.- Glomangiosarcoma.- Kaposi's Sarcoma (KS).- References.- 38 Diagnostic and Therapeutic Principles in Angiodysplasia.- Haemodynamic Categories.- Sequestrated Vascular Malformations.- Haemodynamically Venous Vascular Malformations.- Low-Flow Arteriovenous Malformations.- High-Flow Arteriovenous Malformations.- Ancillary Investigations.- Evolution.- Treatment.- References.