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The Vasculitides The Vasculitides Science and practice Edited by B.M. Ansell Consultant Rheumatologist and Former Head of Division of Rheumatology, Clinical Research Centre, Northwick Park Hospital, London, UK P.A. Bacon Professor of Rheumatology and Head of the Rheumatology Department, University of Birmingham, Birmingham, UK J.T. Lie Professor and Director, Division of Anatomic Pathology, University of California Davis School of Medicine and University of California Davis Medical Center, Sacramento, California, USA and H. Yazlcl Professor and Chief, Division of Rheumatology, Department of Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Turkey I~!'I Springer-Science+Business Media, B.Y. FFiirrsstt eeddiittiioonn 11999966 ©© 11999966 SSpprriinnggeerr SScciieennccee++BBuussiinneessss MMeeddiiaa DDoorrddrreecchhtt OOrriiggiinnaallllyy ppuubblliisshheedd bbyy CChhaappmmaann && HHaallll iinn 11999966.. SSooffttccoovveerr rreepprriinntt oofft thhee hhaarrddccoovveerr 1Isstt eeddiittiioonn 11999966 TTyyppeesseett iinn 1100//1122 PPaallaattiinnoo bbyy JJ&&LL CCoommppoossiittiioonn LLttdd,, FFiilleeyy,, NNoorrtthh YYoorrkksshhiirree IISSBBNN 997788--00--441122--6644114400--44 IISSBBNN 997788--11--44889999--22888899--44 ((eeBBooookk)) DDOOII 1100..11000077//997788--11--44889999--22888899--44 AAppaarrtt ffrroomm aannyy ffaaiirr ddeeaalliinngg ffoorr tthhee ppuurrppoosseess ooff rreesseeaarrcchh oorr pprriivvaattee ssttuuddyy,, oorr ccrriittiicciissmm oorr rreevviieeww,, aass ppeerrmmiitttteedd uunnddeerr tthhee UUKK CCooppyyrriigghhtt DDeessiiggnnss aanndd PPaatteennttss AAcctt,, 11998888,, tthhiiss ppuubblliiccaattiioonn mmaayy nnoott bbee rreepprroodduucceedd,, ssttoorreedd,, oorr ttrraannssrmniitttteedd,, iinn aannyy ffoorrmm oorr bbyy aannyy mmeeaannss,, wwiitthhoouutt tthhee pprriioorr ppeerrrmniissssiioonn iinn wwrriittiinngg ooff tthhee ppuubblliisshheerrss,, oorr iinn tthhee ccaassee ooff rreepprrooggrraapphhiice rreepprroodduuccttiioonn oonnllyy iinn aaccccoorrddaannccee wwiitthh tthhee tteerrmmss ooff tthhee lliicceenncceess iissssuueedd bbyy tthhee CCooppyyrriigghhtt LLiicceennssiinngg AAggeennccyy iinn tthhee UUKK,, oorr iinn aaccccoorrddaannccee wwiitthh tthhee tteerrmmss ooff lliicceenncceess iissssuueedd bbyy tthhee aapppprroopprriiaattee RReepprroodduuccttiioonn RRiigghhttss OOrrggaanniizzaattiioonn oouuttssiiddee tthhee UUKK.. EEnnqquuiirriieess ccoonncceerrnniinngg rreepprroodduuccttiioonn oouuttssiiddee tthhee tteerrmmss ssttaatteedd hheerree sshhoouulldd bbee sseenntt ttoo tthhee ppuubblliisshheerrss aatt tthhee LLoonnddoonn aaddddrreessss pprriinntteedd oonn tthhiiss ppaaggee.. TThhee ppuubblliisshheerr mmaakkeess nnoo rreepprreesseennttaattiioonn,, eexxpprreessss oorr iimmpplliieedd,, wwiitthh rreeggaarrdd ttoo tthhee aaccccuurraaccyy ooff tthhee iinnffoorrmmaattiioonn ccoonnttaaiinneedd iinn tthhiiss bbooookk aanndd ccaannnnoott aacccceepptt aannyy lleeggaall rreessppoonnssiibbiilliittyy oorr lliiaabbiilliittyy ffoorr aannyy eerrrroorrss oorr oormniissssiioonnss tthhaatt mmaayybbee mmaaddee.. AA ccaattaalioogguuee rreeccoorrdd ffoorr tthhiiss bbooookk iiss aavvaaiillaabbllee ffrroomm tthhee BBrriittiisshh LLiibbrraarryy LLiibbrraarryy ooff CCoonnggrreessss CCaattaalloogg CCaarrdd NNuummbbeerr:: 9955--7788884488 @@ PPrriinntteedd oonn aacciidd ffrreeee tteexxtt ppaappeerr,, mmaannuuffaaccttuurreedd iinn aaccccoorrddaannccee wwiitthh AANNSSII//NNIISSOO ZZ3399..4488--11999922 ((PPeerrmmaanneennccee ooff PPaappeerr)).. Contents Color plates appear hetween pages 232 and 233 List of contributors viii Foreword xxi E.G.L. Bywaters Preface XXlll Part One Classification and Histology 1 1 Vaseulitis - then and now 3 P.A. Bacon 2 Classification and histopathologie specificity of systemie vaseulitis 21 J.T. Lie Part Two Disease Mechanisms and Investigations 37 3 Vasculitis: mechanisms of injury 39 F.c. Breedveld and M.R. Daha 4 Antineutrophil cytoplasmie antibodies (ANCA): current perspectives 48 C.G.M. Kallenberg 5 Antiendothelial cell antibodies, antiphospholipid antibodies and vascular disease 65 D. D'Cruz and G. Hughes Contents VI 6 Adhesion molecules in disease: vascular cell adhesion molecule-1 as a target for therapy 83 J.M. Clements, R Pigott, L.A Needham and AJ.H. Gearing 7 MRI and CT in vasculitis 99 M. Heller, C. Muhle, M. Reuter and E Schubert 8 Angiography in vasculitis: its role in diagnosis and in the management of complications 109 I.E. Jackson Part Three Primary and Systemic Vasculitides 119 9 Polyarteritis nodosa: clinical aspects 121 L. Guillevin, E Lhote and P. Casassus 10 Microscopic polyarteritis (MPA) 135 P.A Bacon, C. Savage and D. Adu 11 New concepts in Wegener's granulomatosis 145 W.L. Gross 12 Giant cell arteritis 171 B.-A. Bengtsson 13 Takayasu arteritis: a current update 181 I.T. Lie 14 Beh~et's syndrome with emphasis on clinical genetics and mortality 199 H. Yazlcl, B. C;irek, G. Ba~aran et al. 15 Endothelial cell dysfunction in Beh~et's disease 207 E. Kansu 16 Pulmonary vasculitides 222 L. Mouthon, E Lhote and L. Guillevin 17 Angiitis of the central nervous system 246 J.T. Lie Part Four Connective Tissue and Dermal Vasculitides 265 18 Rheumatoid vasculitis 267 P.A Bacon, RA Luqmani and D.G.I. Scott 19 Vasculitis in primary Sjogren's syndrome 277 AG. Tzioujas, EN. Skopouli, D. Boumba and H.M. Moutsopoulos 20 Mixed cryoglobulinemia: the role of HCVand organ-specific antibodies 283 S. Bombardieri, A Tavoni, M. Mosca et al. 21 Cutaneous vasculitis 294 J.J. Cream Contents vii Part Five ~anagernent 307 22 Treatment of severe vasculitis: general principles and the use of corticosteroids 309 J.S. Cameron 23 Therapy of vasculitis 315 R.A. Luqmani, A. Pall, D. Adu et al. 24 Treatment of polyarteritis nodosa and Churg-Strauss syndrome 332 L. Guillevin and F. Lhote 25 Relapses of Wegener's granulomatosis: role of micro-organisms and prophylaxis with trimethoprim/ sul£amethoxazole 356 CG.M. Kallenberg 26 Alternative treatments for systemic vasculitis 362 CM. Lockwood Part Six Vasculitis in Childhood 375 27 Juvenile dermatomyositis 377 B.M. Ansell 28 Kawasaki syndrome 384 M.J. Dillon 29 Kawasaki syndrome: the Florentine experience 392 F. Falcini, M. Ermini, S. Trapani et al. 30 Vasculitis and its relation to the streptococcus 397 J. David 31 Schonlein-Henoch purpura nephritis 403 P. Niaudet 32 Vasculitis in familial Mediterranean fever 412 M. Pras, P. Langevitz, A. Livneh and D. Zemer 33 Behc;et's syndrome in children 417 H. 6zdogan Index 425 Contribut ors D.Adu Birmingham Vasculitis Group Departments of Rheumatology and Nephrology Queen Elizabeth Medical Centre University of Birmingham Edgbaston Birmingham UK B.M. Ansell Stoke Poges Berkshire UK P.A. Bacon Department of Rheumatology University of Birmingham Edgbaston Birmingham B15 2TT UK G. Bartolozzi Department of Pediatrics Rheumatology Unit University of Florence Florence Italy G. Ba~aran Cerrahpasa Medical Faculty Division of Rheumatology University of Istanbul Istanbul Turkey Contributors ix B.-A. Bengtsson Department of Medicine Sahlgrenska University Hospital Gothenburg Sweden S. Bombardieri Clinical Immunology and Rheumatology Units Istituto di Patologia Medica University of Pisa Via Roma 67 56126 Pisa Italy D. Boumba Department of Internal Medicine School of Medicine University of Ioannina Greece Ee. Breedveld Department of Rheumatology Building I, C4-R PO Box 9600 2300 RC Leiden The Netherlands E.G.L. Bywaters Beaconsfield Buckinghamshire UK N. <;akir Cerrahpasa Medical Faculty Division of Rheumatology University of Istanbul Istanbul Turkey J.S. Cameron Clinical Science Laboratories 17th Floor Guy's Hospital Guy's Campus UMDS London SEI 9RT UK x Contributors P. Casassus Service de Medecine Interne H6pital Avicenne 125 rue de Stalingrad F-/93009 Bobigny France B. <;i.;ek Cerrahpasa Medical Faculty Division of Rheumatology University of Istanbul Istanbul Turkey L. La Civita Clinical Immunology and Rheumatology Units Istituto di Patologia Medica University of Pisa Via Roma 67 56126 Pisa Italy J.M. Clements British Biotech Watlington Road Cowley Oxford OX4 5LY UK J.J. Cream Charing Cross Hospital Fulham Palace Road London W6 8RF UK M.B. Daha Department of Nephrology Building 1, C3-P PO Box 9600 2300 RC Leiden The Netherlands

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Professor E. G. L. Bywaters CBE, MO, FRCp, FACP This book provides a summary - a distillation - of most of the presenta­ tion at the XIIIth Symposium of EULAR (The European League Against Rheumatism) on Vasculitis organized by Professor Hasan Yazici and Professor Paul Bacon. It was indeed a symposi
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Professor E. G. L. Bywaters CBE, MO, FRCp, FACP This book provides a summary - a distillation - of most of the presenta­ tion at the XIIIth Symposium of EULAR (The European League Against Rheumatism) on Vasculitis organized by Professor Hasan Yazici and Professor Paul Bacon. It was indeed a symposi

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