Inflammatory Diseases of Blood Vessels -- Contents -- List of Contributors -- Preface -- PART I Biology of Blood Vessels and Mechanisms of Vascular Inflammation -- CHAPTER 1 Vascular Development -- Development of the cardiovascular system -- Endothelial cell heterogeneity and organ specificity -- Arterial and venous endothelial cell distinctions -- Lymphatic capillaries -- Blood-brain barrier -- Implications of vascular diversity for disease expression and therapy -- Demonstrated or accepted -- Hypothetical -- Conclusions -- Acknowledgments -- References -- CHAPTER 2 Vascular Repair -- Introduction -- History -- Embryonic development -- Initial stimulus of arteriogenesis -- Role of physical factors -- Growth factors -- Role of bone marrow derived cells -- Morphology and remodeling of collateral arteries -- Proliferative phase -- Synthetic phase -- Maturation phase -- Tunica interna - the endothelium -- Tunica media - smooth muscle cells -- Tunica externa - the adventitia -- Pathways involved in arteriogenesis -- Fluid shear stress sensing - calcium signaling - Trpv4 -- The actin cytoskeleton - Abra -- Phenotypic switch - fetal gene program -- References -- CHAPTER 3 Leukocyte Trafficking -- Introduction -- Leukocyte recruitment cascade -- Tethering and rolling -- Activation and firm adhesion -- Intraluminal crawling -- Transendothelial migration (diapedisis) -- Migration through tissues -- Normal trafficking of leukocyte subsets -- Granulocytes -- Monocytes, macrophages, and dendritic cells -- Lymphocytes -- Microvascular traffic control -- Clinical implications of leukocyte trafficking as a therapeutic target -- Antiadhesion molecule therapies -- Therapeutic targeting of chemoattractants -- Conclusions -- References -- CHAPTER 4 Dendritic Cells and Vascular Inflammation -- Functions of DC in the immune system.

VasDC - defining the immunologic identity of arteries -- VasDC in vasculitis -- DC in atherosclerosis -- VasDC as therapeutic targets in inflammatory vasculopathy -- References -- CHAPTER 5 T Cells and Vascular Inflammation -- Introduction -- Principles of T-cell biology -- Antigen specificity -- T-cell generation and homeostasis -- T-cell activation -- T-cell differentiation and function -- T cells in vascular inflammation -- Genetic risk factors of vascular inflammation -- T-helper cell function for autoantibody production -- T-cell cytokine pathways in vascular inflammation -- T regulatory cells in vascular inflammation -- End-differentiated effector T cells in vascular inflammation -- T-cell-targeted therapy -- T-cell depletion -- Targeting TCR stimulation and signaling -- Inhibition of co-stimulation -- Inhibition of cytokine signaling -- Neutralization of T-cell-derived cytokines -- Conclusions -- References -- CHAPTER 6 Autoantibodies and Vascular Inflammation -- Introduction -- Antineutrophil cytoplasmic antibodies -- Antigenic specificities and method of detection -- Associations of PR3-ANCA and MPO-ANCA with clinical disease entities in systemic vasculitis -- Pathogenicity of ANCA -- Therapeutic implications -- Anti-cPR3 antibodies -- Anti-LAMP-2 antibodies -- Antiendothelial cell antibodies -- Methods of detection -- Endothelial antigens -- Disease associations -- Pathogenetic role -- References -- CHAPTER 7 Neutrophils and Vascular Inflammation -- Introduction -- Neutrophils in the ANCA associated vasculitides -- Neutrophil effector functions -- Conclusions -- References -- CHAPTER 8 Cytokines and Vascular Inflammation -- Introduction -- Cytokines in the pathogenesis of vascular inflammation -- Cytokines in early phases of vascular inflammation -- Inflammatory infiltration of the vessel wall and amplification mechanisms.

Cytokines and growth factors in vascular regeneration and remodeling -- Angiogenesis -- Vascular remodeling -- Translational impact of cytokine research in systemic vasculitis -- Common genetic variants in cytokine genes and disease susceptibility -- Cytokines as biomarkers of vascular inflammation -- Cytokines as therapeutic targets -- Acknowledgments -- References -- CHAPTER 9 Oxidative Stress and Vascular Inflammation -- Reactive oxygen species -- Oxidative stress - old vs. new concepts -- Sources of reactive oxygen species -- NADPH oxidases -- Mitochondrial electron transport -- Xanthine oxidase -- Uncoupled nitric oxide synthase -- Interplay between sources or reactive oxygen species -- Relationship between oxidation and inflammation -- Oxidative stress and inflammation in atherosclerosis -- Effect of early atherosclerosis/ hypercholesterolemia on vascular oxidative stress -- Early events in atherosclerosis -- Progression and advanced stages of atherosclerosis -- Vascular inflammation in hypertension -- Vascular inflammation in diabetes, obesity and the metabolic syndrome -- Failure of current therapies and future directions -- References -- CHAPTER 10 Hemostasis and Vascular Inflammation -- Overview of hemostasis and its regulation -- Thrombin-thrombomodulin leads to activation of protein C and plasma procarboxypeptidase -- Anti-inflammatory role of plasma carboxypeptidase B -- Lectin domain of thrombomodulin and its effect on myeloid dendritic cells -- Other platelet mediators -- Conclusions -- References -- CHAPTER 11 Animal Models of Vasculitis -- General aspects of animal models of vasculitis -- Immune complex-associated vasculitis models -- Vasculitis models mediated by adaptive immunity against vascular components -- ANCA associated vasculitis models -- Cytokine-mediated vasculitis models -- Infectious agent-associated vasculitis models.

Autoimmune-mediated vasculitis models/lupus models -- Polygene models of vasculitis -- Conclusions -- References -- CHAPTER 12 Arteries, Smooth Muscle Cells and Genetic Causes of Thoracic Aortic Aneurysms -- Structures of arteries: elastic versus muscular -- VSMCs and their diverse roles in the vascular system -- Thoracic aortic aneurysms and acute aortic dissections: causative mutations disrupt smooth muscle function -- References -- CHAPTER 13 Innate Immunity in Atherosclerosis -- Introduction -- Initiation and progression of atherogenesis -- Potential role of foreign pathogens in atherosclerosis -- Immunity and host defense -- Toll-like receptors: front-line defense against pathogens -- TLRs participate in atherosclerosis -- TLRs expression and atherosclerosis -- Genetic variants of TLRs and atherosclerosis -- TLRs and atherosclerosis: animal experiments -- Exogenous TLR ligands implicated in the progression of atherosclerosis -- Endogenous TLR ligands and atherosclerosis -- Cross-talk between TLRs and lipid metabolism by liver X receptor -- TLRs, dendritic cells and atherosclerosis: the critical link? -- Future therapy: Re-engineering immune defenses -- Acknowledgments -- References -- CHAPTER 14 Adaptive Immunity in Atherosclerosis -- Immune cells at different stages of atherosclerosis -- Innate and adaptive immunity -- Innate immunity -- Adaptive immunity -- Regulatory T cells control autoimmunity -- Role of the immune system in animal models of atherosclerosis -- Animal models of atherosclerotic disease -- Atherosclerosis is accelerated by Th1 immunity -- Th2 and Th17 cells may have a multifaceted role in atherosclerosis -- CD4+ versus CD8+ T cells in atherosclerosis -- NKT cells respond to lipid antigens -- Tregs are atheroprotective -- B cells and antibodies -- Therapeutic potential of immune modulation in atherosclerosis.

Conclusions and clinical perspectives -- Disclosures -- References -- PART I I Primary Autoimmune Vascular Disease -- CHAPTER 15 Historical Perspectives of Vasculitis -- Polyarteritis nodosa -- Microscopic polyarteritis -- Henoch-Schönlein purpura -- Granulomatosis with polyangiitis (Wegener's) -- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) -- Giant cell arteritis -- Takayasu's arteritis -- Behçet's syndrome -- Kawasaki disease -- Classification of vasculitis -- References -- CHAPTER 16 Approach to the Differential Diagnosis of Vasculitis -- Introduction -- Overall approach to diagnosis of vasculitis -- Large vessel vasculitis -- Aortitis -- Temporal arteritis -- Medium vessel vasculitis -- Infectious mimics -- Hypercoagulable states -- Arteriographic mimics -- Segmental arterial mediolysis -- Grange syndrome -- Small vessel vasculitis -- Pulmonary-renal syndrome -- Rhinosinusitis -- Cutaneous vasculitis -- Conclusions -- References -- CHAPTER 17 Imaging of Medium and Large Vessels (CT/MR/PET) -- Computed tomography -- Magnetic resonance imaging -- Fluorodeoxyglucose positron emission tomography -- Key concepts supported by multiple studies -- Implications -- New opportunities for future investigation -- References -- CHAPTER 18 Kawasaki Disease -- Introduction -- Demographics -- Clinical features -- Laboratory features -- Imaging features -- Histopathology -- Etiology -- Pathogenesis -- Treatment -- Refractory disease -- Treatment options in those with coronary artery aneurysms -- Conclusions -- References -- CHAPTER 19 Henoch-Schönlein Purpura -- Introduction -- Classification criteria -- Epidemiology -- Clinical features -- Children -- Adult HSP -- Laboratory evaluation -- Imaging -- Pathology -- Etiology -- Genetic factors -- Environmental factors -- Pathogenesis (Figure 19.5).

Evidence for involvement of group A streptococcus.