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I. Nervous System Vasculitis 1 Chapter 1 the Clinical Approach to Patients with Vasculitis 3 David S Complimentary Contributor Copy Complimentary Contributor Copy PUBLIC HEALTH IN THE 21ST CENTURY THE VASCULITIDES VOLUME 2 NERVOUS SYSTEM VASCULITIS AND TREATMENT (SECOND EDITION) No part of this digital document may be reproduced, stored in a retrieval system or transmitted in any form or by any means. The publisher has taken reasonable care in the preparation of this digital document, but makes no expressed or implied warranty of any kind and assumes no responsibility for any errors or omissions. No liability is assumed for incidental or consequential damages in connection with or arising out of information contained herein. This digital document is sold with the clear understanding that the publisher is not engaged in rendering legal, medical or any other professional services. Complimentary Contributor Copy PUBLIC HEALTH IN THE 21ST CENTURY Additional books and e-books in this series can be found on Nova’s website under the Series tab. Complimentary Contributor Copy PUBLIC HEALTH IN THE 21ST CENTURY THE VASCULITIDES VOLUME 2 NERVOUS SYSTEM VASCULITIS AND TREATMENT (SECOND EDITION) DAVID S. YOUNGER, MD, MPH, MS EDITOR Complimentary Contributor Copy Copyright © 2019 by Nova Science Publishers, Inc. All rights reserved. No part of this book may be reproduced, stored in a retrieval system or transmitted in any form or by any means: electronic, electrostatic, magnetic, tape, mechanical photocopying, recording or otherwise without the written permission of the Publisher. We have partnered with Copyright Clearance Center to make it easy for you to obtain permissions to reuse content from this publication. Simply navigate to this publication’s page on Nova’s website and locate the “Get Permission” button below the title description. This button is linked directly to the title’s permission page on copyright.com. Alternatively, you can visit copyright.com and search by title, ISBN, or ISSN. For further questions about using the service on copyright.com, please contact: Copyright Clearance Center Phone: +1-(978) 750-8400 Fax: +1-(978) 750-4470 E-mail: [email protected]. NOTICE TO THE READER The Publisher has taken reasonable care in the preparation of this book, but makes no expressed or implied warranty of any kind and assumes no responsibility for any errors or omissions. No liability is assumed for incidental or consequential damages in connection with or arising out of information contained in this book. The Publisher shall not be liable for any special, consequential, or exemplary damages resulting, in whole or in part, from the readers’ use of, or reliance upon, this material. Any parts of this book based on government reports are so indicated and copyright is claimed for those parts to the extent applicable to compilations of such works. Independent verification should be sought for any data, advice or recommendations contained in this book. In addition, no responsibility is assumed by the Publisher for any injury and/or damage to persons or property arising from any methods, products, instructions, ideas or otherwise contained in this publication. This publication is designed to provide accurate and authoritative information with regard to the subject matter covered herein. It is sold with the clear understanding that the Publisher is not engaged in rendering legal or any other professional services. If legal or any other expert assistance is required, the services of a competent person should be sought. FROM A DECLARATION OF PARTICIPANTS JOINTLY ADOPTED BY A COMMITTEE OF THE AMERICAN BAR ASSOCIATION AND A COMMITTEE OF PUBLISHERS. Additional color graphics may be available in the e-book version of this book. Library of Congress Cataloging-in-Publication Data ISBN: 978-1-53615-136-7 (eBook) Published by Nova Science Publishers, Inc. † New York Complimentary Contributor Copy CONTENTS Contents v Foreword to The Vasculitides vii Charles D. Pusey, MD and Richard A. Watts, MD, MPH Preface ix I. Nervous System Vasculitis 1 Chapter 1 The Clinical Approach to Patients with Vasculitis 3 David S. Younger, MD, MPH, MS Chapter 2 Polyarteritis Nodosa 19 Loïc Raffray, MD and Loïc Guillevin, MD Chapter 3 Giant Cell Arteritis 49 David S. Younger, MD, MPH, MS Chapter 4 Ophthalmologic and Neuro-Ophthalmologic Aspects of Vasculitides 73 David S. Younger, MD, MPH, MS Chapter 5 The Blood-Brain Barrier 105 David S. Younger, MD, MPH, MS Chapter 6 Neuroimaging of CNS Vasculitis 121 Eytan Raz, MD PhD, Adam J. Davis, MD and David S. Younger, MD, MPH, MD Chapter 7 Adult Primary Angiitis of the Central Nervous System and Related Syndromes 147 Rula A. Hajj-Ali, MD and Leonard H. Calabrese, DO Chapter 8 Primary CNS Vasculitis: Pathophysiological Diversity 159 Nicolò Pipitone, MD, PhD, Gene G. Hunder, MD and Carlo Salvarani, MD Chapter 9 Stroke Due to Vasculitis in Adults 171 David S. Younger, MD, MPH, MS Complimentary Contributor Copy vi Contents Chapter 10 Central Nervous System Vasculitis Due to Infection 189 David S. Younger, MD, MPH, MS and Patrica K. Coyle, MD Chapter 11 Central Nervous System Vasculitis Due to Drug Abuse 215 David S. Younger, MD, MPH, MS Chapter 12 Peripheral Nerve Vasculitis: Classification and Disease Associations 241 Kelly G. Gwathmey, MD, Jennifer A. Tracy, MD and P. James B. Dyck, MD Chapter 13 Autoimmune Encephalitides 269 David S. Younger, MD, MPH, MS II. Treatment 297 Chapter 14 Overview of the Treatment of Primary Systemic Vasculitis 299 Antoine Sreih, MD and Peter A. Merkel, MD, MPH About the Editor 323 List of Contributors 325 Index 329 Related Nova Publications 343 Complimentary Contributor Copy FOREWORD TO THE VASCULITIDES Charles D. Pusey and Richard A. Watts Clinical and basic research into the systemic vasculitides have continued to gather momentum over the last four years since publication of the first edition of this book. We have attended two more International Vasculitis and ANCA Workshops, in London in 2015 and Tokyo in 2017. Both of these excellent meetings have covered the area of vasculitis more broadly and extensively than in the past. The present edition of this book contains contributions from many of those participating in these meetings. Classification and nomenclature in vasculitis is increasingly harmonised and agreed across different specialties. There have been a number of genetic studies in ANCA-associated vasculitis (AAV) in which the genetic associations appear to be more closely related to ANCA specificity, i.e., MPO-ANCA or PR3-ANCA, than to clinical classification. The extent of overlap between the different vasculitic conditions is also becoming more apparent, in particular the co-existence of AAV and anti-GBM disease. In terms of pathogenesis, there is increasing evidence for the contribution of complement activation in AAV. This has been nicely demonstrated in animal models, and a phase 2 trial of a C5a receptor inhibitor has recently been reported. Another area of increasing interest is the role of neutrophil extracellular traps (NETs) which appear to play a part both in tissue inflammation and in the generation of autoimmunity. The number of clinical trials in vasculitis continues to expand. The use of rituximab for induction therapy in AAV is now well established, and different approaches to its use in maintenance therapy have been published. There are ongoing investigations of other novel agents, such as belimumab and abatacept. The results of the PEXIVAS study, which examines the use of additional plasma exchange, and of standard or reduced corticosteroid dose, are eagerly awaited. In the area of large vessel vasculitis, there have also been rapid developments. The genetic basis of Takayasu arteritis and giant cell arteritis is becoming clearer, with evidence of different genetic risk factors underlying the two conditions. Imaging, especially the role of PET-CT in disease assessment, is becoming better established. The treatment of giant cell arteritis has taken a large step forward with the introduction of IL-6 blockade as an established therapy. However, many questions remain, especially when to use IL-6 blockade and for how long. This Complimentary Contributor Copy viii Charles D. Pusey and Richard A. Watts treatment should permit many patients to avoid the deleterious consequences of long term high dose steroids. We believe that the breadth and depth of the contributions in the second edition of this book surpass the high standards set in the first edition. The publication of this new edition precedes the 19th International Vasculitis and ANCA Workshop to be held in April 2019 in Philadelphia. Complimentary Contributor Copy PREFACE Systemic and nervous system vasculitides are a heterogeneous group of related disorders, each characterized by vascular inflammation such that it has the potential to cause serious morbidity and mortality if unrecognized and therefore untreated. Systemic vasculitis affect all populations and every nationality and walk-of-life, from childhood to older age. The first edition of The Vasculitides published in 2014 to meet the urgent need for a clear, concise and reliable textbook regarding the epidemiology, pathogenesis, clinical presentation, laboratory evaluation and management of these disorders, assembled participants of the 16th International Vasculitis and ANCA Workshop in Paris, France. Five years later, two subsequent meetings have taken place, in London and Tokyo. The 19th International Vasculitis & ANCA Workshop in April 2019 at the University of Pennsylvania promises to be an exceptional venue to share translational scientific discoveries, data from clinical trials, and advances in the clinical assessment, pathophysiology, genetic biomarkers, and standard-of-care and novel therapies of vasculitis. The second edition, which is an update of the original two-volume book, remaining encyclopedic in content, adds six new chapters, incorporating the participation of investigators who did not have an opportunity to contribute the first volume, including some from the previous meeting in Japan. The new chapters are Health Related Quality of Life and Measurements, Neutrophilic Cell Pathology, Complement Factors in ANCA-Associated Vasculitis, Isolated Aortitis/IgG4 Disease, Anti-GBM Disease, and the Autoimmune Encephalitides.
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