Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis
This volume, written by well-known experts in the field, covers all aspects of Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis (AAV). The expression refers to a group of diseases, characterized by destruction and inflammation of small ve
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Renato Alberto Sinico Loïc Guillevin Editors
Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis
Rare Diseases of the Immune System Series Editors: Lorenzo Emmi Domenico Prisco Editoria Board: Systemic Vasculitis L. Emmi C. Salvarani R. A. Sinico
Primary Immunodeficiency A. Plebani C. T. Baldari M. M. D’Elios
Autoimmune Disease P. L. Meroni D. Roccatello M. Matucci Cerinic L. Emmi
Systemic Fibroinflammatory Disorders A. Vaglio
Autoinflammatory Syndromes M. Gattorno F. De Benedetti R. Cimaz
Nuvole sul mare, Laura Maddii Emmi (Private collection)
Renato Alberto Sinico • Loïc Guillevin Editors
Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis
Editors Renato Alberto Sinico Department of Medicine and Surgery University of Milano-Bicocca Monza Monza e Brianza Italy
Loïc Guillevin Department of Medicine Paris Descartes University Hospital Cochin Paris France
ISSN 2282-6505 ISSN 2283-6403 (electronic) Rare Diseases of the Immune System ISBN 978-3-030-02238-9 ISBN 978-3-030-02239-6 (eBook) https://doi.org/10.1007/978-3-030-02239-6 © Springer Nature Switzerland AG 2020 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors, and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland
Dedicated to my family.
Acknowledgements
We would like to thank all the prestigious colleagues and researches from around the world and from different disciplines for the invaluable contribution to this book. Without their help this book would not have been possible.
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Contents
Part I ANCA-Associated Vasculitis 1 Introduction: Nomenclature and Classification�������������������������������������� 3 J. Charles Jennette and Ronald J. Falk 2 Genetics of ANCA-Associated Vasculitis������������������������������������������
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