Index. -actin, 128, 129, 131 -myosin heavy chain, 128, 129, 131 -globulin, intravenous, 200 1, fatty acids, 480

Transcription

1 Index -actin, 128, 129, 131 -myosin heavy chain, 128, 129, 131 -globulin, intravenous, 200 1, fatty acids, 480 A1AT ( 1 antitrypsin), 78 AAV see ANCA-associated vasculitis (AAV) abatacept, 59 abdominal obesity, 475, 476 abdominal pain Henoch Schönlein purpura, 207, 212, 213 polyarteritis nodosa, 220 sarcoidosis, 454 single organ vasculitis, 333, A -related angiitis (ABRA), ABMMNC (autologous bone marrow-derived mononuclear cells), 362, 363, 364 ABRA (A -related angiitis), Abra (actin-binding Rho activating protein) gene, 24 5 ACE inhibitors, 410 acne-like lesions, ACR see American College of Rheumatology (ACR) criteria ACTA2 gene, 132, 133 -actin, 128, 129, 131 actin cytoskeleton, 24 5 actin-binding Rho activating protein (Abra) gene, 24 5 activated protein C (apc), 107, activation leukocyte recruitment cascade, 30 lymphocytes, cytokine-mediated, 83 4 monocytes, 20 Tcells,52 acupuncture, 530 acute hemorrhagic edema of infancy (AHEI), acute necrotizing vasculitis, 413 acute phase reactants, acute renal failure (ARF), acute retinal necrosis (ARN), 375, 501 acyclovir, 375, 376 adalimumab, 397 adaptive immune system animal models of vasculitis, atherosclerosis, accelerated, 476 dendritic cells, 40 1 giant cell arteritis, innate immunity differences, 50 adhesion see also intercellular adhesion molecule 1 (ICAM-1) atherosclerosis, 475 endothelial leukocytes, 29 30, 87 8 neutrophils, 76 integrins, 29 30, 31, 36 intracellular matrix adhesion molecule, 9 LFA-1, 29, 30, 52 polyarteritis nodosa, 223 primary cutaneous vasculitis, 347 selectins, 28 9, 34 5, 72, 75, 223 adrenal insufficiency, 490 advanced glycation end-products (AGEs), 102, 112, 113 adventitial pattern, vasa vasoritis, 44 adverse drug reactions drug-induced vasculitis, GPA(WG) therapies, 250 reporting, 389 Takayasu s arteritis therapies, 285 vasculitis treatment side effects, AECA see antiendothelial cell antibodies (AECA) age atherosclerosis risk factor, 46 giant cell arteritis, 264 microscopic polyangiitis, 227, 228, 229 thymic decline, 51 AGEs (advanced glycation end-products), 102, 112, 113 AHA (American Heart Association), 194, 197, 198, 201 AHEI (acute hemorrhagic edema of infancy), airway disease see ear, nose and throat (ENT) symptoms; pulmonary disease albendazole, 260 allergic granuloma see Churg Strauss syndrome (CSS) alternative complement pathway, 65 alternative monocyte activation profile, 20 American College of Rheumatology (ACR) criteria drug-induced vasculitis, 381 EGPA(CSS), 253 giant cell arteritis, 266 glucocorticoid-induced osteoporosis, 489 Henoch Schönlein purpura, 205, 206 polyarteritis nodosa, 220, 222 single organ vasculitis, 333 systemic lupus erythematosus, 420 American Heart Association (AHA), 194, 197, 198, 201 amitryptiline, 530 amplification, cytokines, 86 8 amputation, 362, 364 amyloid deposition, cerebral, ANA (antinuclear antibodies), 404, 424, 425, 426, 428 anastomotic aneurysms, 548, 549, 551, 564 anatomy arteries, nerves, 521, 522 ANCA-associated vasculitis (AAV) see also granulomatosis with polyangiitis (Wegener s) (GPA(WG)); microscopic polyangiitis ANCA assays, 64 animal models, 119 autoantibody production, 61 2 characteristics, 71, 72, 77, 79 cytokines as therapeutic target, 91 drug-induced, 387, 388, 389 EGPA(CSS), , 524 neutrophil activation, 72, 77, 79 primary vasculitis, 62 renal failure dialysis, ESRD causes, 534 6, 541 transplantation, rheumatic disorders, atherosclerosis, 475 T cells, 55, 56, 57 Inflammatory Diseases of Blood Vessels, Second Edition. Edited by Gary S. Hoffman, Cornelia M. Weyand, Carol A. Langford and Jörg J. Goronzy Blackwell Publishing Ltd. Published 2012 by Blackwell Publishing Ltd. 567

2 568 Index aneurysms anastomotic, 548, 549, 551, 564 familial, 132, 133, giant cell arteritis, historical perspective, 162, 163 Kawasaki disease, 198, 199, 203 Marfan s syndrome, 173 rheumatoid vasculitis, 396 sarcoidosis, 454, 457 secondary aortitis, 172 syphilitic, 161 Takayasu s arteritis, 167, Ang-1, 11 angioblasts, 4, 9 angiocentric lymphomas, 465 angiogenesis characteristics, 16 cytokines/growth factors, 88 9 definition, 3, 5 6 intramedullary K-wires, 363 systemic sclerosis, 408 angioplasty, coronary, 545, 546 animal models, adaptive immunity-mediated vasculitis, ANCA-associated vasculitis, 119 atherosclerosis, 147 immune system role, autoimmune-mediated vasculitis, cytokine-mediated vasculitis, 119 general aspects, GPA(WG), 247 immune complex-associated vasculitis, infectious agent-associated vasculitis, MPO ANCA pathogenic role, 64, 65, 66 polygene vasculitis models, TLRs and atherosclerosis, antiadhesion molecule therapies, 36 antiangiogenic molecules, 7 antiarrhythmics, side effects, 381 anti-b-cell therapy, 213, 319, 410, 411 antibiotics, 381, 385, 516 anticardiolipin antibodies, 475 anticoagulants, 203, 381 anticonvulsant side effects, 381 anti-cpr3 (complementary proteinase 3) antibodies, 62, 66 anticytokine therapy, 156 see also anti-tumor necrosis factor (anti-tnf) therapy antiendothelial cell antibodies (AECA) adaptive immunity vasculitis animal models, 118, 119 characteristics, 67 9 primary vasculitis, 62 systemic sclerosis, 408, 409 antifibrotic agents, 410, 411 antigen presenting cells (APCs), 100, 150, 153, 272 see also dendritic cells antigen specificity, T cells, 51 anti-glomerular basement membrane disease, 118, 180, 233, 234, 535 anti-hepatitis B virus antibodies, 370 antihistamines, 348 anti-inflammatory therapy, 155, 156 see also immunosuppressants aspirin, 201, 202, 203, 274, 429 NSAIDs, 308, 448, 470 anti-jo-1 autoantibodies, 437 anti-lamp-2 antibodies, 66 7, 247 antilysosomal membrane protein-2 (Anti-LAMP-2), 62 antimetabolites see also azathioprine; methotrexate (MTX); mycophenolate mofetil ophthalmic side effects, antimicrobials, 381, 385, 516 antineutrophil cytoplasmic antibodies (ANCA) see ANCA-associated vasculitis (AAV) antinuclear antibodies (ANA), 404, 424, 425, 426, 428 antioxidants, 97, 103 antiphospholipid antibodies (APLA), 421, 427 antiphospholipid syndrome (APS), 176, 177 antiplatelet agents, 203, antirheumatic disease drugs, 381, 387 antispastic medications, 529 antithrombotic surfaces, 7 antithyroid agents, 381 anti-tumor necrosis factor (anti-tnf) therapy Behçet s syndrome, 296 lipid profiles, 479 monitoring side effects, 486 ocular side effects, 502 primary cutaneous vasculitis, 349 rheumatoid arthritis, drug-induced vasculitis, 397 rheumatoid vasculitis, 399 side effects, 486, 492, 502 Takayasu s arteritis, 284 antiviral therapy, 371, 372 3, 374, 377 anti-vsmc antibodies, 118, 119 aorta aneurysm aortitis, 172 atherosclerosis, 173 familial disorders diagnosis, giant cell arteritis, relapsing polychondritis, 443, 444, 446 sarcoidosis, 454, 457 Takayasu s arteritis, aortitis Cogan s syndrome, 301, 309 differential diagnosis, MRI studies, 187 relapsing polychondritis, 443, 444, 446 sarcoidosis, 454, 457 secondary, 172 single organ vasculitis, 332, 333, Cogan s syndrome, 301, 304, 309 congenital coarctation, congenital occlusion, 15 giant cell arteritis, , immunologic identity, dendritic cells, 41, 42 regurgitation, , 556 rheumatoid vasculitis, 394, 396 sarcoidosis, 454, 457 single organ vasculitis, 332, 333, stenting, 549, 562, 563 Takayasu s arteritis, 167, , valve replacement, VSMC mutations, aortic valve replacement (AVR), apc (activated protein C), 107, APCs (antigen presenting cells), 100, 150, 153, 272 see also dendritic cells APLA (antiphospholipid antibodies), 176, 177 apolipoprotein A/B, 477, 478 apolipoprotein E deletions, 147 apoptotic neutrophils, 32, 78 9 appendix vasculitis, 338 aquaporin 4 (AQP4), 11 ARF (acute renal failure), ARN (acute retinal necrosis), 375, 501 arteries see also arteriogenesis; arteriography; individual arteries anatomy, endothelial cells development, 8 9 polyarteritis nodosa, Takayasu s arteritis, vascular smooth muscle cells, arteriogenesis, 3, 5 characteristics, collaterals development, ephrin Efn family, 8 molecular pathways, 22 5 pre-existent arterioles, 17 shear stress, 8 9 arteriography, see also computed tomography angiography (CTA) cerebral, color Doppler flow studies, 354, 355 color-coded duplex sonography, 189, 192 digital subtraction angiography, 354, 357 postmortem angiography, 16, 18 arteriovenous anastomosis, 17, 18 arthralgia, 313 arthritis, 57, , 437, 475 aseptic vasculitis, 245, 246 aspirin, 201, 202, 203, 274, 429 asthma, , 387 astrocytes, 11 asymmetric blood pressures, 265, 280 asymptomatic aneurysms, atherogenesis treatments, 13 atherosclerosis accelerated, aortitis distinction, 173

3 Index 569 clinical aspects, 144, dendritic cells, 45 7 foreign pathogens role, immunity/host defense, 138 inhibition treatments, initiation/progression, 137 innate immune system, oxidative stress, rheumatic disorders, cholesterol, systemic lupus erythematosus, 424 Toll-like receptors, ATP-dependent cross-bridge cycling, 129, 131 atrial myxoma, 465, 466 atypical CNS vasculitis, 324 audiometry, 301, 304, 306, 308 see also ear, nose and throat symptoms auricular chondritis, 442, 443, 446, 447 Australia antigen, 218 autoantibody characteristics AECA antibodies, 67 9 ANCA antibodies, 62 6 anti-cpr3 antibodies, 66 anti-lamp-2 antibodies, 66 7 cryoglobulinemic vasculitis, 318 overview, 61 9 primary vasculitis, 61 2 systemic sclerosis, Th (T helper) cells, 55 autoantigen complementarity, 66 autoimmune-mediated vasculitis see also primary autoimmune vascular disease accelerated atherosclerosis, animal models, idiopathic inflammatory myopathies, lipid metabolism, 476, relapsing polychondritis, Sjögren s syndrome, systemic lupus erythematosus, systemic sclerosis, autologous bone marrow-derived mononuclear cells (ABMMNC), 362, 363, 364 avascular necrosis (AVN), 489, 490 AVR (aortic valve replacement), axonal degeneration, 523 azathioprine Cogan s syndrome, 309 EGPA(CSS), 259 GPA(WG), 248, 249, 250 microscopic polyangiitis, 236 PACNS, 329 side effects, 486, 492 Takayasu s arteritis, 283 B-lymphocytes atherosclerosis, 153, 154 cytokine-mediated activation, 83, 84 depletion therapy, 213, 319, 410, 411 HCV-associated vasculitis, 372 idiopathic cryoglobulinemic vasculitis, 317, 319 SLE secondary vasculitis, 419, 428, 430, 431 B19 parvovirus, 376 baclofen, 529 BACNS (benign angiopathy of the CNS), 322, 323, 326, 327, 328, 330 Baconian (induction) models, 115, 120 Behçet, Hulusi, 168, 289 Behçet s syndrome, clinical manifestations, diagnosis, epidemiology, historical perspective, 168 laboratory investigations, paraneoplastic syndrome, 464 pathogenesis, 295 treatment, benign angiopathy of the CNS (BACNS), 322, 323, 326, 327, 328, 330 Bentall procedure, 550, 555 biofeedback, 530 biologic immune modulatory agents see also anti-tumor necrosis factor (anti-tnf) therapy; infliximab; monoclonal antibodies (MABs) etanercept, 397 infection rates, 484 ocular side effects, 502 rheumatoid vasculitis, 399 vasculitis causation, 381, 387 biomarkers, 90 1, 430 biopsies drug-induced vasculitis, 383, 388 giant cell arteritis, 266 GPA(WG), 245 6, idiopathic inflammatory myopathies, 436 microscopic polyangiitis, muscle, EGPA(CSS), 257 nerve, 524, 527 PACNS, 327 polyarteritis nodosa, 222, 223 renal, 535, 536, 537 Henoch Schönlein purpura, 209 rheumatoid vasculitis, 398, 399 sarcoidosis, 453 single organ vasculitis, 334, 337 sinonasal, GPA(WG), skin, 346, 347 subglottic stenosis, bisphosphonates, blood clotting, blood pressure, 127, 265, 280 see also hypertension blood brain barrier, 11 Bobath method, 528 bone marrow-derived cells (BMCs), 19 20, 32, 121 bone morphogenetic protein-4 (BMP-4), 9 brachiocephalic disease, 565 brainstem auditory evoked potentials, 301, 304 branching capillary patterns, 5, 6, 9 breasts, 332, 333, 335 Brunnstrom method, 528 Buerger, Leo, 351 Buerger s disease, clinical features, demographic features, diagnostic imaging, differential diagnosis, etiology/pathogenesis, 358, 360 histopathology, 354, 358, 359, 360 laboratory investigations, 353 oral infection inflammatory pathway, 360 treatment, bull s eye maculopathy, 496 BXSB mouse strain, 120 c-anca (cytoplasmic ANCA), 62, 63, 64, 239, 424, 538 C-reactive protein (CRP), 268, 269 C57Black/6 mouse strain, 152 Ca ++ /calmodulin-dependent myosin light chain kinase (MLCK), 129 CAA (cerebral amyloid angiopathy), 323 CAD see coronary artery disease (CAD) calcification, CAB graft, 545 calcium channel blockers, 361 calcium signalling, 22 4, 25 calf muscles, 332, 333, 334 cancer-associated myositis (CAM), 433, 435 cardiopulmonary bypass (CPB), 547 cardiovascular system (CVS) see also coronary... Behçet s syndrome, cardiac disease, 220, 394, 396, 407, 477 cardiothoracic surgery, Cogan s syndrome, 301, 303, 304, 309 development, 3 6 rheumatoid vasculitis, 398 Takayasu s arteritis therapies, 285 carotid immunological identity, 41, 42 cartilaginous structures, 441 9, , cataracts, 496, 497, 499 CCDS (color-coded duplex sonography), 189, 192 CCP, rheumatoid vasculitis, 398, 399 CD4 + T cells, 52 4, 153, 452, 476 CD8 + T cells, 153, 437, 476 CD28, 52 CD31 (PECAM-1), 9, 87, 223 CD177 expression, 72 3 cell differentiation, 52 4, 83 4 phenotypic switch, 22, 25, 108, , 131 cell-based therapies, cell-mediated immunity, , 408, 437 central memory T cells (T cm ), 34 central nervous system (CNS) damage treatments/management, drug-induced vasculitis, 383 HIV-1-associated vasculitis, 374

4 570 Index central nervous system (CNS) (Continued ) idiopathic inflammatory myopathies, 437 primary angiitis, relapsing polychondritis, 444 rheumatoid vasculitis, 394, 395 Sjögren s syndrome, SLE secondary vasculitis, VZV-associated vasculitis, 375 central serous chorioretinopathy (CSCR), 498, 499 cerebral A -related angiitis, cerebral amyloid angiitis, 323 cerebral amyloid angiopathy (CAA), 323 cerebral angiography, cerebral spinal fluid (CSF), 324 5, 327 cerebral venous sinus thrombosis (CVST), 292 CFI (collateral flow index), 17 Chapel Hill Consensus Conference (CHCC) criteria, 217, 253, 333 chemerin, 111, 112 chemoattractants, 31, 37 chemokines, 34 6, 85 6, 475 chest X-ray, 256, 293, 445 children Henoch Schönlein purpura, juvenile dermatomyositis, 433, 434, 435, 436, 437 Kawasaki disease, polyarteritis nodosa, chimeric mouse model, 41 2 Chlamydophila pneumoniae, 140, 141 cholesterol, rheumatic disorders, chondritis, relapsing, chorioretinitis, fungal, 502 chromosomal mapping, chronic serous otitis media, Churg Strauss syndrome (CSS), 166 see also eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome) (EGPA(CSS)) CIMDL see cocaine-induced midline destructive lesions (CIMDL) CIMT (constraint-induced movement therapy), classification Behçet s syndrome, 294 Chapel Hill Consensus Conference criteria, 217, 253, 333 Crawford s classification, 547, 548 drug-induced vasculitis, 380 EGPA(CSS), 253 giant cell arteritis, 266 Ishikawa s prognostic classification system, 546 paraneoplastic syndrome, polyarteritis nodosa, 220, 222 systemic lupus erythematosus, 420 systemic vasculitis, , 117, Henoch Schönlein purpura, 205, 206 Takayasu s arteritis, 278 claudins, 11 clinical aspects ANCA antibodies, 66 atherosclerosis, 144 immune modulation, Behçet s syndrome, Buerger s disease, chemoattractants, 37 cytokines systemic vasculitis, 90 1 as therapeutic targets, 91 diagnostic imaging, EGPA(CSS), giant cell arteritis, GPA(WG), , complications, 244 Henoch Schönlein purpura, 206 8, idiopathic cryoglobulinemic vasculitis, inflammatory vasculopathy, 47 8 Kawasaki disease, leukocyte trafficking, 36 7 oxidative stress, polyarteritis nodosa, primary cutaneous vasculitis, Sjögren s syndrome, secondary vasculitis, systemic sclerosis, T cell-targeted therapy, 57 9 Takayasu s arteritis, vascular diversity, vascular repair, 25 C max (maximal conductances), 17, 18 CMV (cytomegalovirus), 119, 120, 329, 376 7, 500 CNSsee central nervous system (CNS) coagulation cascade, 106, 108, 181 cocaine-induced midline destructive lesions (CIMDL) cocaine-induced vasculitis, 386, 387, 388 paraneoplastic syndrome distinction, 465, 466 rhinosinusitis, Cogan s syndrome, cardiovascular manifestations, 301, 303, 304, 309 diagnosis, disease state assessment, ear involvement, 299, 300, 301, 303, 304 7, 308 9, 310 etiology, 307 eye involvement, 299, 300, 303 6, 307 8, 310 histopathology, 302, 303 treatment, colchicine, 295 6, 348 collagen, 441, 447, 449 collateral circulation, 15 25, 354, 355, 356, 357 color Doppler flow studies, 354, 355 color-coded duplex sonography (CCDS), 189, 192 combinatorial specificity model, 34 common variable immunodeficiency (CVID), 181 comparative genomics, 41, 42 compartmentalization, immune reactivity, 452 complement activation, 437 complications see adverse drug reactions computed tomography angiography (CTA), Buerger s disease, 356 giant cell arteritis, PACNS, 326, 327 Takayasu s arteritis, 281 computed tomography (CT), Behçet s syndrome, 293 Cogan s syndrome, 301 EGPA(CSS), 256, 257 giant cell arteritis, 268, 269 aorta, 554 GPA(WG), 241, 243 sinonasal manifestations, 513, 515 relapsing polychondritis, 445, 446 systemic lupus erythematosus, 424, 425, 426, 428 conductances, maximal, 17, 18 congenital coarctation of aorta, connective tissue disease associated myositis (CTM), 433, 434 connective tissue disorders, 173 4, , 433, 434 constraint-induced movement therapy (CIMT), continuous endothelial cells, 7 contractile elastin units, 127, 128, 129 corkscrew collaterals, 354, 355, 356, 357 corneal melt, 395, 397 coronary artery bypass graft (CABG), 545 coronary artery disease (CAD) angioplasty, 545, 546 arteritis, 45, 333, 334, 396, 444 autoimmune-mediated vasculitis, 477 gene expression, Kawasaki disease, aneurysms, 203 systemic lupus erythematosus, 424 Takayasu s arteritis, 278, coronary steal phenomenon, 544, 545, 546 corticosteroids Behçet s syndrome, 296 Cogan s syndrome, 307, 308, 309 EGPA(CSS), 259, 260 giant cell arteritis, 272, 273, 274 HCV-associated vasculitis, 373 Henoch Schönlein purpura, 212, 213 idiopathic cryoglobulinemic vasculitis, 319 inflammatory vasculopathy, 47, 48 microscopic polyangiitis, 236 nerve damage, 532 ophthalmic risks, 497 primary cutaneous vasculitis, 348 relapsing polychondritis, 448 rheumatoid vasculitis, 399

5 Index 571 side effects, Takayasu s arteritis, 547 cough see also pulmonary disease giant cell arteritis, 265 CPB (cardiopulmonary bypass), 547 cranial giant cell arteritis, 264 Crawford s classification, 547, 548 crescentic glomerulonephritis, 64, 66, 534, 535, 537, 538 cross-talk, TLRs, 142, 143 CRP (C-reactive protein), 268, 269 cryoglobulinemic vasculitis, , 371 3, 536 cryoglobulins, 412, 414, 417 cryoprecipitation, , 317 CSCR (central serous chorioretinopathy), 498, 499 CSS (Churg Strauss syndrome), 166 see also eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome) (EGPA(CSS)) CT see computed tomography (CT) CTA see computed tomography angiography (CTA) CTM (connective tissue disease associated myositis), 433, 434 Curio, Carlo, 403 cutaneous vasculitis see skin manifestations CVID (common variable immunodeficiency), 181 CVSsee cardiovascular system (CVS) CXC chemokines, 85 6 cyclophosphamide acute renal failure treatment, 536 ANCA associated vasculitis, 66 Behçet s syndrome, 296 Cogan s syndrome, 309 EGPA(CSS), 259, 260 GPA(WG), HCV-associated vasculitis, 373 Henoch Schönlein purpura, 213 microscopic polyangiitis, 235, 236 PACNS, 329 paraneoplastic syndrome, 463, 470 polyarteritis nodosa, 224 rheumatoid vasculitis, 399 side effects, 486, 490 1, malignancy, 487 monitoring, 486 ocular, Takayasu s arteritis, 283 cyclosporine, 58, 296 cysticercosis, 329 cystitis, cytokine sequence theory, 119 cytokine-mediated vasculitis, 119 cytokines see also individual cytokines blood vessel formation, 9 chemokines, 34 6, 85 6, 475 definition, 82 3 giant cell arteritis, 270, 271 Henoch Schönlein purpura, , 212 neutrophil priming, 73 pathogenesis of vascular inflammation, 83 8 primary cutaneous vasculitis, 347, 349 regeneration/remodeling involvement, 88 9 rheumatoid vasculitis, 398 sarcoidosis, 450, 452 systemic vasculitis treatments, 90 1 Tcells cytokine storms, 57 differentiation, 52 4, 55 6 neutralization, 59 signaling inhibition, 59 Th2/Th17 cells in atherosclerosis, cytomegalovirus (CMV), 119, 120, 329, 376 7, 500 cytoplasmic ANCA (c-anca), 62, 63, 64, 239, 424, 538 cytotoxic therapy, 283, 284, 381, 389, 532 see also individual cytotoxic drugs Dacron aortic grafts, 547, 548, danger-associated molecular patterns (DAMPs), 40, 41, 42, 263, 271 dantrolene, 529 dapsone, 348 Davson, J., 217 Davy, James, 167 deduction models, 115 demographic features Buerger s disease, idiopathic cryoglobulinemic vasculitis, Kawasaki disease, microscopic polyangiitis, PACNS, SLE secondary vasculitis, Takayasu s arteritis, 277 demyelination patterns, 523 dendritic cells (DC), accelerated atherosclerosis, 476 atherosclerosis, 45 7, functions in immune system, 40 1 giant cell arteritis, 270 hemostasis, immunologic identity definition of arteries, 41 3 imprinting phenomenon, 36 normal trafficking, 33 4 SLE secondary vasculitis, 419, 428, 430, 431 subsets, 33 therapeutic target role, 47 8 vasculitis, 43 5 dermatomyositis (DM), 433, 434, 436, 438 desmoplakin, 10 development vascular, 3 14 arterial/venous endothelial cell distinctions, 8 9 blood brain barrier, 11 cardiovascular system, 3 6 disease expression/therapy factors, endothelial cell heterogeneity/organ specificity, 7 8, 13 lymphatic capillaries, 9 11, 13 pre-existent arterioles, 15, 16, 17 repair, 16, 17 diabetes atherosclerosis, 99, 101 accelerated, 475, 476 oxidative stress, 102 treatments, vascular diversity, 13 diagnosis see diagnostic imaging; differential diagnosis diagnostic imaging see also computed tomography angiography (CTA); computed tomography (CT); fluorodeoxyglucose positron emission tomography (FDG-PET) aortic aneurysms, 548 Behçet s syndrome, 293 Buerger s disease, clinical aspects, EGPA(CSS), 256, 257 giant cell arteritis, Henoch Schönlein purpura, 209 Kawasaki disease, optical coherence tomography, 499 overview, PACNS, 326 7, 330 relapsing polychondritis, 443, SLE secondary vasculitis, 421, 425 6, 427, 428 systemic sclerosis, Takayasu s arteritis, 280, 281, 286 ultrasound, 189, 192, 354, 355 virus-associated vasculitides, 370 VZV-associated vasculitis, 375 dialysis, renal, diameter increase (arteries), 20, 22, diapedesis, 29, 30 1 diaschisis, 528 diazepam, 529 differential diagnosis aortitis, single organ vasculitis, atherosclerosis, 173 Behçet s syndrome, Buerger s disease, Cogan s syndrome, 304, congenital coarctation of aorta, end-stage renal disease, 535 fibromuscular dysplasia, 174, 176 giant cell arteritis, GPA(WG) microscopic polyangiitis/egpa(css), sinonasal manifestations, Grange syndrome, 178 9, 180 large vessel vasculitis, 171 6

6 572 Index differential diagnosis (Continued ) medium vessel vasculitis, arteriographic mimics, microscopic polyangiitis, 217 PACNS, 324, 325, paraneoplastic syndrome, polyarteritis nodosa, primary autoimmune vascular disease, primary cutaneous vasculitis, relapsing polychondritis, 444, 447 retinal vasculitis, 334 rheumatoid vasculitis, 398 sarcoidosis, 451 small vessel vasculitis, Takayasu s arteritis, temporal arteritis, differentiation of cells, 52 4, 83 4 phenotypic switch, 22, 25, 108, , 131 digital subtraction angiography, 354, 357 digital ulcers, 406 digital vessels, 358 dilatation, subglottic stenosis, 509, 510 discontinuous endothelial cells, 7 disease expression, vascular diversity, disease modifying antirheumatic drugs (DMARDs), 393, 400 diuretics, 381 DM (dermatomyositis), 433, 434, 436, 438 DMARDS (disease modifying antirheumatic drugs), 393, 400 dot sign, Buerger s disease, 354, 355 drug treatments see adverse drug reactions; medical treatments drug-induced vasculitis, clinical features, diagnosis, epidemiology, etiology, paraneoplastic syndrome, 470 rheumatoid arthritis, 397 treatment, duloxetine, 530 dyslipidemia, 475, 476 E-selectins, 28 9, 223 ear, nose and throat (ENT) symptoms auricular chondritis, 442, 443, 446, 447 Cogan s syndrome, 299, 300, 301, 303, 304 7, 308 9, 310 EGPA(CSS), 254 giant cell arteritis, 265 GPA(WG), 239, 240, 241, 242, microscopic polyangiitis, 231 nasal chondritis, 442, 443, 446 relapsing polychondritis, 441, 442, 443, 446, 447, 448 subglottic stenosis, early death, 416, 454, 457 EBV (Epstein Barr virus), 377 EC see endothelial cells (EC) echocardiography, 198, 199 efalizumab, 36 effector-memory T cells (T EM ), 34, 56 7 egfp-marked stem cell pools, vascular repair, 19 EGPA(CSS) see eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome) (EGPA(CSS)) Ehlers Danlos syndrome type IV (EDS IV), 173, 174 elastic arteries, anatomy, elastin units, 127, 128, 129 electrodiagnostic nerve testing, electromyography (EMG), 525, 526 elephant trunk procedure, 554 embryonic development see development EMG (electromyography), 525, 526 encephalopathy, 322 end-stage renal disease (ESRD), 228, 229, endarteritis obliterans, 413 endogenous TLR ligands, 142 endoscopy, , 513, 514 endothelial antigens, 67 9 endothelial cells (EC) atherosclerosis, rheumatic disorders, 475 Buerger s disease, 358, 360 hemostasis, immune complex-associated vasculitis animal models, 117, 118 leukocyte adhesion, 87 8 neutrophils interaction with, 73 7 organ specificity, 7 8, 13 systemic sclerosis, 408 tight junctions, BBB development, 11 tube formation, 3, 9 endothelial nitric oxide synthase (enos), 20 endothelin receptor antagonists, 410 endovascular stenting, 546, 549, 562, 563 endovascular surgery see surgery enos (endothelial nitric oxide synthase), 20 ENT see ear, nose and throat (ENT) symptoms environmental factors, 210, 409 enzymes see also matrix metalloproteinases (MMPs); myeloperoxidase; proteinase 3 (PR3) ACE inhibitors, 410 endothelial NO synthase, 20 human neutrophil elastase, 182 Janus kinase, 59 neutrophil serine proteases, 78 paraoxonase, 478, 479 plasma carboxypeptidases, 107, , 113 ROS production, NADPH oxidase, 73, 74, 76, 77, 94, 95, 96, 98 NO synthases, 95, 97, 98 uncoupled NO synthase, 97, 98, 100 xanthine oxidase, 95, 96 7, 98 thiopurine methyl transferase, 485, 486 tyrosine kinase inhibitors, 410, 411 eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome) (EGPA(CSS)), see also granulomatosis with polyangiitis (Wegener s) (GPA(WG)) clinical manifestations, diagnosis, differential diagnosis, 176, 232 eosinophils activation, 85 immunopathogenesis, nerve biopsy, 524 primary vasculitis, 62 pulmonary renal syndrome, 180 treatment, eosinophils, 85, 255 6, 258, 259 ephrin Efn family, 8 epidemiology Behçet s syndrome, drug-induced vasculitis, EGPA(CSS), giant cell arteritis, GPA(WG), Henoch Schönlein purpura, 205, 206 idiopathic cryoglobulinemic vasculitis, idiopathic inflammatory myopathies, 434 microscopic polyangiitis, paraneoplastic syndrome, polyarteritis nodosa, rheumatoid vasculitis, systemic sclerosis, 405, 406 Takayasu s arteritis, Eppinger, Hans, 162, 163 Epstein Barr virus (EBV), 377 etanercept, 397 ethnic variations, 277, 279 European League Against Rheumatism (EULAR), 205, 206, 296 European Vasculitis Study Group (EUVAS), 239, 248, Eustachian tube dysfunction, 513 exocrine dysfunction see Sjögren s syndrome extravascular giant cell arteritis, 266 eye involvement Behçet s syndrome, 291, 293 Cogan s syndrome, 299, 300, 303 6, 307 8, 310 giant cell arteritis, 264 5, 273 glaucoma, 496, 498, 499 GPA(WG), 240, 242 idiopathic inflammatory myopathies, 437 microscopic polyangiitis, 231 relapsing polychondritis, 443 retinal vasculitis, 333, 334 5, 375 6, 501 rheumatoid vasculitis, 394 Takayasu s arteritis, 278 vasculitis treatment risks, facial artery, 185 familial disorders see genetic risk factors FBN1 (fibrillin 1) gene, 132, 133, 174 Fc receptors, 73, 74, 75, 76, 398, 479

7 Index 573 FDG-PET see fluorodeoxyglucose positron emission tomography (FDG-PET) femoral occlusion, vascular repair, 19 fenestrated endothelial cells, 7 Ferrari, Enrico, 165 FESS (functional endoscopic sinus surgery), 516 fever, 196, 266 fibrates, 480 fibrillin 1 (FBN1) gene, 132, 133, 174 fibrinogen, 107 fibrinoid necrosis, 221, 225, 234, 413 fibroblast growth factor (FGF), 362 FGF-2, 9, 11 fibromuscular dysplasia (FMD), 174, 176 fibrosis, , 537 FimH antibodies, 66 7 fingolimod (FTY720), 37 firm adhesion, FK-506, 309 Fli1 transcription factor, 411 fluorodeoxyglucose positron emission tomography (FDG-PET) giant cell arteritis, 269 relapsing polychondritis, 445, 446 systemic lupus erythematosus, 424 Takayasu s arteritis, 281 fluorodeoxyglucose positron emission tomography (FDG PET), 190 1, 192 FMD (fibromuscular dysplasia), 174, 176 foam cells, 148, 149 focal single organ vasculitis, 333, foot drop, 394, 395 Foxp3 (Treg signature transcription factor), 56 FSS (fluid shear stress) arteriogenesis, 8 9, 22 4 atherosclerosis, 148, 149 hemostasis, 106 liver/lung, 35 neutrophils/ecs interaction, 74, 75 vascular repair, 16 18, 21 FTY720 (fingolimod), 37 functional endoscopic sinus surgery (FESS), 516 fungal chorioretinitis, 502 G-CSF (granulocyte colony-stimulating factor), 32 G-protein-coupled receptors (GPCR), 30, 31, 74, 76 gabapentin, 530 GACNS (granulomatous angiitis of the CNS), 323 GAG (glycosaminoglycans), 74, 76 gallbladder vasculitis, 338 GALT (gut-associated lymphoid tissue), 35, 36 gamma globulin, intravenous, 200 1, 202 gancyclovir, 377 gangrene, 265, gastrointestinal manifestations Behçet s syndrome, , 292 EGPA(CSS), 255, 257 Henoch Schönlein purpura, 207 idiopathic inflammatory myopathies, 436 microscopic polyangiitis, 231 polyarteritis nodosa, , 221 rheumatoid vasculitis, 394, 396 single organ vasculitis, 332, 333, systemic sclerosis, 410 GCA see giant cell arteritis (GCA) GDNF (glial-derived neurotrophic factor), 11 gene expression coronary artery disease, lymphatic capillary development, 10 phenotypic switch, 22, 25, 108, , 131 profiling arteriogenesis, 22 cytokines as biomarkers, 90 1 Toll-like receptors, 40, 41, 42 gene polymorphisms see also human leukocyte antigen (HLA) polymorphisms EGPA(CSS), 259 Henoch Schönlein purpura, 210 MRL mouse, systemic vasculitis, 90 TLRs, atherosclerosis, 139 genetic risk factors aneurysms, 132, 133, Buerger s disease, 360 connective tissue, GPA(WG), 247 Henoch Schönlein purpura, 210 Kawasaki disease, SLE secondary vasculitis, 428 thoracic aortic aneurysms, VSMC causative mutations, vascular inflammation, 54 5 genital ulcers, 290, 295 genitourinary structures, 332, 333 genome-wide association studies (GWAS), 54 GFAP (glial fibrillary acidic protein), 11 giant cell arteritis (GCA) cardiothoracic surgery, clinical features, cytokines, 84, 86 dendritic cells, 39, 41, 43, 44 5, 46 diagnosis, differential, 171, 172, 175 imaging, 185, 186, epidemiology, etiology, historical perspective, laboratory investigations, 268 MRI studies, 189 paraneoplastic syndrome, 462, 464, 465, 466 peripheral vascular surgery, Takayasu s arteritis distinction, 283 treatment, glaucoma, 496, 498, 499 glial fibrillary acidic protein (GFAP), 11 glial-derived neurotrohic factor (GDNF), 11 -globulin, intravenous, 200 1, 202 glomerulonephritis (GN) Goodpasture s syndrome, 118, 180, 233, 234, 535 GPA(WG), 243 4, 246 microscopic polyangiitis, 229, 230 pulmonary renal syndrome, rapidly progressive, 534, 535, 541 glomerulosclerosis, 537 glucocorticoids giant cell arteritis, 272, 273 infection risk, 484 lipid profiles, 479 PACNS, 329, 330 polyarteritis nodosa, 223, 224, 225 side effects, 389, 486, Takayasu s arteritis, 283, 284, 285 glucose metabolism, 190 1, 192 glycosaminoglycans (GAG), 74, 76 Goodpasture s syndrome, 118, 180, 233, 234, 535 Goto Kakizaki rat model, 13 GPA etanercept trial, 488, 489 GPA(WG) see granulomatosis with polyangiitis (Wegener s) (GPA(WG)) GPCR (G-protein-coupled receptors), 30, 31, 74, 76 gradients of chemoattractants, 31 grafts, arterial, 86, 545, 547, 548, Grange syndrome, 178 9, 180 granulocyte colony-stimulating factor (G-CSF), 32 granulocytes, 31 2 granulomatosis with polyangiitis (Wegener s) (GPA(WG)), see also eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome) (EGPA(CSS)) CIMDL/cocaine-induced vasculitis comparison, 386 clinical features, cytokines, 84 differential diagnosis, 232 epidemiology, histopathology, 242, 243, laboratory investigations, 245 PACNS distinction, 328 paraneoplastic syndrome, 462, 463, 464, 466 pathogenesis, primary cutaneous vasculitis distinction, 344, 345, 347 primary vasculitis, 62 pulmonary renal syndrome, 180 rhinosinusitis, sinonasal manifestations, subglottic stenosis, T cells, 51, 55, 56, 57, 59 treatment, granulomatous angiitis of the CNS (GACNS), 323 granulomatous reactions, 43 5,

8 574 Index growth factors Buerger s disease, 362 fibroblast growth factor, 362 FGF-2, 9, 11, 410, 411 hematopoietic, 381, 386 platelet-derived, 3, 89 transforming growth factor TGF-, 410, 411 TGF- 1, 3, 132, 133 vascular endothelial, 3, 4, 9, 10, 19, 362 vascular repair, 19, 88 9 gut-associated lymphoid tissue (GALT), 35, 36 gut-tropic T cells, 35 6 GWAS (genome-wide association studies), 54 gynecological structures, 332, 333, HAART (highly active retroviral therapy), 373, 374 hairy cell leukemia (HCL), half-lives, neutrophils, 32 HAV (hepatitis A virus), 140, 141 HCV see hepatitis C virus (HCV) HDLs (high density lipoproteins), 476, headache, 322, 327, 328 hearing loss, 299, 300, 301, 303, 304 7, 308 9, 310 heart see cardiovascular system (CVS) heat shock proteins (HSPs), 283, 476 Helicobacter pylori, 140, 141 hematologic tumors, 460, 461, hematopoietic growth factors, 381, 386 hematuria, hemodialysis, hemostasis, overview, plasma procarboxypeptidase, thrombin thrombomodulin, thrombomodulin, lectin domain, Henoch, Eduard Heinrich, 165 Henoch Schönlein purpura (HSP), classification criteria, 205, 206 clinical features, children, epidemiology, 205, 206 ESRD, 536 etiology, historical perspective, 165 laboratory investigations, paraneoplastic syndrome, 462, 464 pathogenesis, primary cutaneous vasculitis distinction, 343, 346, prognosis, treatment, heparin, 203 hepatitis A virus (HAV), 140, 141 hepatitis B virus (HBV) atherosclerosis progression, 141 immunosuppressed patients, 487 polyarteritis nodosa, 218, 223 secondary vasculitis, , 374 hepatitis C virus (HCV) cryoglobulinemic vasculitis, , 536 primary cutaneous vasculitis, 347, 348 secondary vasculitis, 371 3, 374 hepatotoxicity, methotrexate, 491 heritable disorders see genetic risk factors herpes simplex virus (HSV), 119, 140, 141, 377, 502 herpes zoster ophthalmicus (HZO), 50 2, 375, herpetic keratitis, 502 HES-1 (hairy and enhancer of split 1), 45 HGEC (human glomerular endothelial cells), 76 7 high density lipoproteins (HDLs), 476, high mobility group box-1 (HMGB1), , 142 highly active retroviral therapy (HAART), 373, 374 histopathology Behçet s syndrome, Buerger s disease, 354, 358, 359, 360 Cogan s syndrome, 302, 303 drug-induced vasculitis, 383 giant cell arteritis, GPA(WG), 242, 243, idiopathic cryoglobulinemic vasculitis, 361 Kawasaki disease, 199 microscopic polyangiitis, PACNS, 326 paraneoplastic syndrome, 469 polyarteritis nodosa, 222 primary cutaneous vasculitis, single organ vasculitis, 333, 335, 336, 340 Sjögren s syndrome, 413 systemic lupus erythematosus, systemic sclerosis, Takayasu s arteritis, 282 historical perspective immune complex-associated vasculitis animal models, 116, 117 oxidative stress new/old concepts, 94 5 PACNS, systemic sclerosis, 403 vascular repair, vasculitis diagnosis, Behçet s syndrome, 168 Churg Strauss disease, 166 giant cell arteritis, Henoch Schönlein purpura, 165 Kawasaki disease, 168 microscopic polyangiitis, necrotizing granulomatous (Wegener s) vasculitis, Takayasu s arteritis, HIV/AIDS, 328, 329, 373 4, HLA see human leukocyte antigen (HLA) hlamp-2 (human lysosomal membrane protein 2), 235 HMG-CoA reductase inhibitors, 285 HMGB1 (high mobility group box-1), , 142 HNE (human neutrophil elastase), 182 Hodgson, Joseph, 161 homeostasis, T cells, 51 homing receptors, 33, 34, 35 6 see also adhesion Horton, B.T., 166 HSP see Henoch Schönlein purpura (HSP) HSPs (heat shock proteins), 283, 476 HSV (herpes simplex virus), 119, 140, 141, 377, 502 HTLV-1 (human T lymphocytic virus type 1), human glomerular endothelial cells (HGEC), 76 7 human heat shock protein-60 (hhsp-60), 283 human immunodeficiency virus (HIV), 328, 329, 373 4, humanleukocyteantigen(hla) polymorphisms, 210 Behçet s syndrome, 289, 290, 295 EGPA(CSS), 259 giant cell arteritis, relapsing polychondritis, 441, 448 rheumatoid arthritis, 393 T cells, 54, 55 TCR specificity, 51 human lysosomal membrane protein 2 (hlamp-2), 235 human neutrophil elastase (HNE), 182 human T lymphocytic virus type 1 (HTLV-1), Hunter, John, 161 Hutchinson, Jonathan, 166 hydrogen peroxide, 94, 95 hydroxychloroquine, 429, 479, 496 hypercholesterolemia, 149, 150, 152 hypercoagulable states, 176 7, 360 hyperlipidemia, 480, 481 hypersensitivity reactions, 492 hypersensitivity vasculitis, 380 hypertension atherosclerosis, 99, 101 accelerated, 475, 476 pulmonary arterial, 406 Takayasu s arteritis, 279, 559, 564 hypnosis, 530 HZO (herpes zoster ophthalmicus), 50 2, 375, IBM (inclusion body myositis), 433, 435 ICAM-1 see intercellular adhesion molecule 1 (ICAM-1) ICM-AM (intracellular matrix adhesion molecule), 9 idiopathic autoimmune vascular disease see primary autoimmune vascular disease idiopathic cryoglobulinemic vasculitis, clinical manifestations, demographic features, epidemiology, etiology, histopathology, 361

9 Index 575 laboratory investigations, neural manifestations, treatment, idiopathic inflammatory myopathies (IIM), clinical aspects, 433 4, differential diagnosis, 433 epidemiology, 434 gastrointestinal manifestations, 436 subsets, 433, 435 IFN- (interferon- ), 55, 56, 91, 152, 153 IgA (immunoglobulin A), 211, 212, 213, 536 IgG (immunoglobulin G), 235, 425, 426 IIM see idiopathic inflammatory myopathies (IIM) IL-1 (interleukin 1 ), 86 IL-1Ra (interleukin 1 receptor antagonist), 119 IL-6 (interleukin 6), 101, 102 IL-8 (interleukin 8, CXCL8), 37 IL-12 (interleukin 12), 450, 452 IL-17 (interleukin 17), 56, 101, 102 ILD (interstitial lung disease), 406, 436, 437 iliac arteries, 41, 42 IMA (internal mammary artery), 545 IMG (intussusceptive microvascular growth), 5, 6, 9 immune complex-associated vasculitis, , , 398, 535 immune system, 39, 40 3, 148 9, 307 see also adaptive immune system; individual immune system cells; innate immune system; immunizations see vaccines immunofluorescence microscopy, 346 immunoglobulins (Ig) IgA, 211, 212, 213, 536 IgG, 235, 425, 426 immunohistochemistry, 356, 359 immunoreceptor tyrosine-based activation motif (ITAM), 52 immunostimulatory dendritic cell types, 40 immunosuppressants see also azathioprine; corticosteroids; cyclophosphamide; glucocorticoids; methotrexate (MTX); mycophenylate mofetil drug-induced vasculitis, 389 NSAIDs, 308, 448, 470 ophthalmic risks, paraneoplastic syndrome, 470 prednisone, 247, 272, 309 side effects, inclusion body myositis (IBM), 433, 435 induction models (Baconian), 115, 120 infection see also individual infective organisms ANCA-associated vasculitis, 227 animal models, antimicrobials, 381, 385, 516 atherosclerosis, Buerger s disease, 360, 361 Cogan s syndrome, 300, 303, 307 endocarditis, GPA(WG), 245 Group A streptococcus in HSP, 210 hemostasis, 105 idiopathic cryoglobulinemic vasculitis, 317 Kawasaki disease trigger, 199 medication side effects, 381, 385, 484, 487 medium vessel vasculitis, 176 paraneoplastic syndrome, 470 renal failure/dialysis, 539 secondary aortitis, 172 Staphylococcus aureus in GPA(WG), 238 virus-associated vasculitides, inflammatory arthritis, 437 see also rheumatoid arthritis (RA) infliximab Cogan s syndrome, 309 ocular side effects, 502 rheumatoid arthritis, drug-induced vasculitis, 397 rheumatoid vasculitis, 399 Takayasu s arteritis, 284 infrapopliteal artery, 351, 356, 357 inhaled agents, 238, 239, 246, 247 inherited factors see genetic risk factors innate immune system adaptive immunity differences, 50 atherosclerosis, , dendritic cells, 40 1 giant cell arteritis, sarcoidosis, 452 integrins, 29 30, 31, 36 intercellular adhesion molecule 1 (ICAM-1) leukocyte adhesion, 87 8 leukocyte recruitment cascade, 29, 30, 31 polyarteritis nodosa, 223 T cell activation, 52 interferon- -2a (IFN- -2a), 296, 381 interferon- (IFN- ) atherosclerosis, 152, 153, 479 drug-induced vasculitis, 381 sarcoidosis, 452, 458 T cell pathways, 55, 56 therapeutic target, 91 interleukins (IL) IL-1,86 IL-1Ra, 119 IL-6, 101, 102 IL-8 (CXCL8), 37 IL-12, 450, 452 IL-17, 56, 101, 102, 408 internal mammary artery (IMA), 545 interstitial keratitis (IK), 300, 304, 307 8, 310 interstitial lung disease (ILD), 406, 436, 437 interstitial renal fibrosis, 537 intracellular matrix adhesion molecule (ICM-AM), 9 intraluminal crawling, 29, 30 intramedullary K-wires, 363 intravascular lymphomas, 465 intussusceptive microvascular growth (IMG), 5, 6, 9 iodine-containing contrast agents, 184 IPEX (immunodysregulation polyendocrinopathy enteropathy X-linked) syndrome, 56 ischemic heart disease (IHD), 477 ischemic ulceration, Ishikawa s prognostic classification system, 546 ITAM (immunoreceptor tyrosine-based activation motif), 52 ivermectin, 260 IVIG (intravenous gamma globulin), 200 1, 202 Janus kinase (KNK3), 59 joint manifestations, 313 see also rheumatoid arthritis (RA) juvenile dermatomyositis (JDM), 433, 434, 435, 436, 437 Kawasaki disease, 45, animal models, infectious agents, 120 clinical aspects, 196 demographics, etiology, histopathology, 199 historical perspective, 168 imaging features, laboratory investigations, pathogenesis, 200 refractory, superantigens trigger, Tcells,54 treatments, keloid scar formation, 507 keratitis, herpetic, 50 2 kidney see renal manifestations Klinger, Heinz, 165 KNK3 (janus kinase), 59 Kussmaul, Adolf, 162, 164, 217 laboratory investigations Behçet s syndrome, Buerger s disease, 353 Cogan s syndrome, 303 differential diagnoses, 181, 182 drug-induced vasculitis, 383, EGPA(CSS), giant cell arteritis, 268 GPA(WG), 245 HCV-associated mixed cryoglobulinemia, 371 Henoch Schönlein purpura, idiopathic cryoglobulinemic vasculitis, Kawasaki disease, microscopic polyangiitis, PACNS, paraneoplastic syndrome, polyarteritis nodosa, 222 primary cutaneous vasculitis, 345, 346, 347 Raynaud s phenomenon, 404 relapsing polychondritis, 444 5

10 576 Index laboratory investigations (Continued ) rheumatoid vasculitis, Sjögren s syndrome, systemic sclerosis, lacrimal dysfunction see Sjögren s syndrome Lactobacillus casei (LCCWE), 120 large vessel vasculitis (LVV) autoantibodies, 67 8 Cogan s syndrome, 301 differential diagnosis, giant cell arteritis, peripheral vascular surgery, sarcoidosis, virus-associated, 370 larynx, subglottic stenosis, laser surgery, LDLs (low density lipoproteins), 99, 147 8, 149, 476, lectin domain, thrombomodulin, leflunomide, 309 left main coronary artery (LMCA), 545 leukemia cutis, 465 leukemic vasculitis (LV), 465 leukocyte cell-surface Fc receptors, 73, 74, 75, 76, 398, 479 leukocytes see also dendritic cells; neutrophils adhesion deficiency type 1, 30 endothelial cells, 87 8 granulocytes, 31 2 eosinophils, 85, 255 6, 258, 259 lymphocytes, 34, 83 4 monocytes, 20, 33 4 macrophages, 33 4, 84 5, 148, 149, 271 normal, 31 4 recruitment, 28 31, 85 6 trafficking, subsets, 31 4 leukocytoclastic vasculitis (LCV) EBV-associated vasculitis, 377 idiopathic cryoglobulinemic vasculitis, 318 paraneoplastic syndrome, 462, 463, 465, 467 parvovirus B19-associated, 376 primary cutaneous vasculitis, 344, 345, 346, 348 sarcoidosis, 453 Sjögren s syndrome, 413, 414 use of term, 380 leukopenia, 485, 491 leukotriene inhibitors, 387 levamisole, LFA-1 (lymphocyte function associated antigen 1), 29, 30, 52 LG (lymphatoid granulomatosis), 329 lidocaine, topical, 530 ligatures, vascular repair, 15 lipds, omega-3-fatty acids, 480 lipids HDLs, 476, LDLs, 99, 147 8, 149, 476, metabolism, 142, 143, mldl, 479 oxidized LDLs, 149, 150, 153, 154, 155, 479 peroxy-radicals, 95 recombinant anti-oxidized LDL (P45) IgG, 155 liver, 34 5, 491 see also hepatitis... liver X receptor (LXR), 142, 143 LMCA (left main coronary artery), 545 Loeys Dietz syndrome, 174, 175 low density lipoproteins (LDLs), 99, 147 8, 149, 476, lung see pulmonary disease lupus nephritis, 421 LV (leukemic vasculitis), 465 LVV see large vessel vasculitis (LVV) LXR (liver X receptor), 142, 143 lymphatic capillary development, 9 11, 13 lymphatic vessel endothelial hyaluronan receptor-1 (LYVE-1), 10, 11 lymphatoid granulomatosis (LG), 329 lymphocyte function associated antigen 1 (LFA-1), 29, 30, 52 lymphocytes, 34, 83 4 see also Blymphocytes;Tcells lymphocytic vasculitis, 413 lymphomas, angiocentric, 465 lymphomyeloproliferative disorders, 462 LYVE-1 (lymphatic vessel endothelial hyaluronan receptor-1), 10, 11 M1/M2 macrophages, 271 MABs see monoclonal antibodies (MABs) macrophages, 33 4, 84 5, 148, 149, 271 magnetic resonance imaging (MRI), Behçet s syndrome, 293 Cogan s syndrome, 301 EGPA(CSS), 257 giant cell arteritis, 268, 269 aorta, 554 PACNS, 326, 327, 330 relapsing polychondritis, 445, 446 SLE, 424, 426, 427 Takayasu s arteritis, 280, 281 renal artery stenosis, 564 Maier, Rudolf, 162, 164, 217 major histocompatibility complex (MHC), 51, 150 malignancies cancer-associated myositis, 433, 435 immunosuppressed patients, lymphangiogenesis, 10 paraneoplastic syndrome, tumor growth stage-specific efficacy of drugs, 12 Marfan s syndrome, MAS (mid-aortic syndrome), mass-like PACNS (ML-PACNS), 324 matrix metalloproteinases (MMPs) atherosclerosis, 99, 100, 101 collateral artery remodeling, 22 rheumatoid vasculitis, 398 systemic vasculitis, 89 maximal conductances, 17, 18 MC (mixed cryoglobulinemia), 371 MCP-1 (monocyte chemoattractant molecule 1), 19, 20, 99 MCP1 CCR-2 axis, vascular repair, 19, 20 MDCs (myeloid dendritic cells), 40 1, 46, 112, 113 medical treatments see also adverse drug reactions; immunosuppressives; individual medications Buerger s disease, CNS recovery enhancement, drug-induced vasculitis, GPA(WG) sinonasal manifestations, 516, 519 lipid profiles, 479 systemic sclerosis, 410 medium-sized vessel vasculitis autoantibodies, 67 8 cerebral, primary angiitis of the CNS, Cogan s syndrome, 301 differential diagnosis, giant cell arteritis, HBV-associated, HCV-associated, 373 HIV-associated, parvovirus B19-associated, 376 polyarteritis nodosa, sarcoidosis, segmental arterial mediolysis, 178 Sjögren s syndrome, 413, 415 membrane-bound PR3 (mpr3), 72 3 memory lymphocytes, 34 Ménière s disease-like symptoms, 299, 301, 304, 306 mepolizumab, 260, 261 mesenteric arteries, 41, 42 metabolic syndrome, 102, 475, 476 methotrexate (MTX) Cogan s syndrome, 309 giant cell arteritis, 273 GPA(WG), 248, 249 lipid profiles, 479 malignancy side effect, 487 microscopic polyangiitis, 236 monitoring side effects, 486 paraneoplastic syndrome, 470 rheumatoid arthritis, 397 rheumatoid vasculitis, 399 side effects, Takayasu s arteritis, 283 MHC (major histocompatibility complex), 51, 150 mhsp-65 (mycobacterial heat shock protein-65), 283 microscopic polyangiitis (MPA), clinical features, differential diagnosis, 176, 217

11 Index 577 epidemiology/demographics, histopathology, historical perspective, laboratory investigations, pathogenesis, primary vasculitis, 62 pulmonary renal syndrome, 180 microvascular beds heterogeneity/organ specificity, 7 8, 13 neurologic damage, 521, 522, 523 traffic control, 34 6 mid-aortic syndrome (MAS), mitochondrial electron transport, 94, 95, 96, 98 mitomycin-c, 509 MIVD (mononuclear inflammatory vascular disease), 413 mixed cryoglobulinemia (MC), 371 ML-PACNS (mass-like PACNS), 324 MLCK (Ca ++ /calmodulin-dependent myosin light chain kinase), 129 MMPs see matrix metalloproteinases (MMPs) modified LDL (mldl), 479 modified self-antigens, 147, 151 monoclocal antibodies (MABs) see also rituximab adalimumab, 397 efalizumab, 36 infliximab, 284, 309, 397, 399, 502 leukocyte trafficking therapies, 36, 37 mepolizumab, 260, 261 natalizumab, 36 omalizumab, 260 T cell-targeted therapy, 57 monocyte chemoattractant molecule 1 (MCP-1), 19, 20, 99 monocytes, 20, 33 4 mononeuritis multiplex, 218, 221, 394 5, 396, 416 mononuclear inflammatory vascular disease (MIVD), 413 motor recovery, mouse model, 64, 65, 66, 120 1, 152 see also animal models movement therapies, MPA see microscopic polyangiitis (MPA) MPOsee myeloperoxidase (MPO) mpr3 (membrane bound PR3), 72 3 MRI see magnetic resonance imaging (MRI) MRL/lpr mouse strain, 120, 121, 122, 123 MTXsee methotrexate (MTX) multidisciplinary management, 508, 510 mupirocin irrigations, 516 muscles biopsy, EGPA(CSS), 257 electromyography, 525, 526 muscular artery anatomy, myopathies, 433 9, polyarteritis nodosa, 222, 223 single organ vasculitis, 334 muskuloskeletal manifestations Behçet s syndrome, 291 drug-induced vasculitis, 383 GPA(WG), 241, 243 Henoch Schönlein purpura, 207 single organ vasculitis, 332, 333, 334 mutations, mycobacterial heat shock protein-65 (mhsp-65), 283 Mycobacterium tuberculosis, 283, 487, 502 mycophenolate mofetil lipid profiles, 479 microscopic polyangiitis, 236 PACNS, 329 side effects, 486, 492 Takayasu s arteritis, 283 MyD88-dependent/independent pathways, 142, 143, 150 myeloid dendritic cells (MDCs), 40 1, 46, 112, 113 myeloperoxidase (MPO) HDL/LDL production, 476 MPO-ANCA associated vasculitis, 64, 65, 227, 231, 232, 233, 234 5, 535, 537 neutrophils in ANCA-associated vasculitides, 72, 73, 74 primary vasculitis, 61, 62, 63 4, 65, 66 myeloproliferative disorders, 462 MYH11 gene, 132, 133 MYLK gene, 132, 133 myopathies, 433 9, myosin heavy chain, 128, 129, 131 NADPH oxidase cytokine priming of neutrophils, 73, 74, 76, 77 ROS production, 94, 95, 96, 98 nail-fold capillaries, 403, 405 naive lymphocytes, 34, 52, 56, 57 nasal disease, 442, 443, 446, see also ear, nose and throat (ENT) symptoms natalizumab, 36 natural killer T cells (NKT), natural regulatory T cells (ntregs), 151 NCGN (necrotizing and crescentic glomerulonephritis), 64, 66 necrosis acute necrotizing vasculitis, 413 acute retinal necrosis, 375, 501 GPA(WG), 245, 246 necrotizing and crescentic glomerulonephritis, 64, 66 necrotizing granulomatous (Wegener s) vasculitis, necrotizing sarcoid granulomatosis, 453 4, 457 pauci-immune necrotizing crescentic glomerulonephritis, 534 systemic necrotizing vasculitis, 347 VZV-associated retinal vasculitis, necrotizing and crescentic glomerulonephritis (NCGN), 64, 66 necrotizing granulomatous (Wegener s) vasculitis, necrotizing sarcoid granulomatosis (NSG), 453 4, 457 nephritis see also glomerulonephritis (GN); necrotizing and crescentic glomerulonephritis (NCGN) Henoch Schönlein purpura, 207, 208, 209, 210, 212, 213, 214, 215 lupus nephritis, 421 nerves see central nervous system (CNS); neural manifestations; peripheral neuropathy NETs (neutrophil extracellular traps), 32, 71, 77 neural manifestations see also central nervous system (CNS); peripheral neuropathy Behçet s syndrome, 292 EGPA(CSS), 254, 255, 261 GPA(WG), 241 HCV-associated vasculitis, 372 HIV-1-associated vasculitis, 374 idiopathic cryoglobulinemic vasculitis, microscopic polyangiitis, 231 nerve biopsy, 524, 527 pain, 527, paraneoplastic vasculitic neuropathy, 464 polyarteritis nodosa, 218 rheumatoid vasculitis, neuromyelitis optica (NMO), neuropathic pain medications, 530 neuropilin-1 (NRP-1), 8, 9 neutrophil extracellular traps (NETs), 32, 71, 77 neutrophil serine proteases (NSPs), 78 neutrophilic inflammatory vascular disease (NIVD) see leukocytoclastic vasculitis (LCV) neutrophils see also ANCA (antineutrophil cytoplasmic antibodies) apoptotic, 32, 78 9 cytokine-mediated activation/differentiation, 85 hematopoietic growth factor-associated vasculitis, 386 normal function, 31 2, 71 2 sarcoidosis, 452 vascular inflammation, 71 9 ANCA-associated vasculitides, 72 7 effector functions, 77 9 NFAT (nuclear factor of activated T cells) proteins, 58 NICD (Notch intracellular domain), 44 5 nicotinic acid, 480, 481 nitric oxide synthases, 95, 97, 98