Renato Alberto Sinico - Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis

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Rare Diseases of the Immune System

Series Editors

Lorenzo Emmi and Domenico Prisco

Editorial Board

C. Salvarani , R. A. Sinico , P. L. Meroni , D. Roccatello , M. Matucci Cerinic , M. Gattorno , F. de Benedetti , R. Cimaz , A. Plebani , C. T. Baldari , M. M. DElios and A. Vaglio

Editors

Renato Alberto Sinico and Loc Guillevin

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

Loc Guillevin

Department of Medicine, Paris Descartes University Hospital Cochin, Paris, France

ISSN 2282-6505 e-ISSN 2283-6403

Rare Diseases of the Immune System

ISBN 978-3-030-02238-9 e-ISBN 978-3-030-02239-6

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.

Contents

Part IANCA-Associated Vasculitis

J. Charles Jennette and Ronald J. Falk

Federico Alberici , Paul Anthony Lyons and Davide Martorana

Delphine Sterlin , Alexis Mathian and Makoto Miyara

Elena Csernok and Antonella Radice

Raashid A. Luqmani

Yann Nguyen and Loc Guillevin

Christian Pagnoux

Renato Alberto Sinico , Filippo Maria Sala , Maria Rosa Pozzi , Paolo Fabbrini and Federico Pieruzzi

Part IIVasculitis/Organs Involvement

Trimarchi Matteo , Galli Andrea and Roberto Teggi

Marta Casal Moura and Ulrich Specks

Renato Alberto Sinico , Fabio Pagni , Vincenzo LImperio , Valentina Binda , Paolo Fabbrini , Federico Pieruzzi and Gabriella Moroni

Michael P. Collins and P. James B. Dyck

Hubert de Boysson

Angelo Valerio Marzano , Simona Tavecchio and Emilio Berti

Giorgio Trivioli and Augusto Vaglio

David Jayne

Loc Guillevin , Loc Raffray , Yann Nguyen , Benjamin Chaigne and Benjamin Terrier

Part I ANCA-Associated Vasculitis

Springer Nature Switzerland AG 2020

R. A. Sinico, L. Guillevin (eds.) Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Rare Diseases of the Immune System https://doi.org/10.1007/978-3-030-02239-6_1

1. Introduction: Nomenclature and Classification

J. Charles Jennette

(1)

Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA

(2)

Department of Medicine, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA

J. Charles Jennette (Corresponding author)

Email:

Ronald J. Falk

Email:

Keywords

Antineutrophil cytoplasmic antibody (ANCA) Vasculitis Microscopic polyangiitis Granulomatosis with polyangiitis Eosinophilic granulomatosis with polyangiitis

1.1 AAV Pathologic Features

Vasculitis is inflammation in blood vessel walls. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is vasculitis accompanied by circulating ANCAs, or phenotypically identical disease without detectable ANCA (ANCA-negative AAV). AAV is a necrotizing vasculitis with few or no immune deposits that affects predominantly small vessels (i.e., capillaries, venules, arterioles, and small arteries) (Table ].

Table 1.1

Vasculitis names adopted by the 2012 International Chapel Hill Consensus Conference on the Nomenclature of Vasculitides (2012 CHCC) []

Large-vessel vasculitis (LVV)

Takayasu arteritis (TAK)

Giant cell arteritis (GCA)

Medium-vessel vasculitis (MVV)

Polyarteritis nodosa (PAN)

Kawasaki disease (KD)

Small-vessel vasculitis (SVV)

Antineutrophil cytoplasmic antibody (ANCA)associated vasculitis (AAV)

Microscopic polyangiitis (MPA)

Granulomatosis with polyangiitis (Wegeners) (GPA)

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA)

Immune complex SVV

Anti-glomerular basement membrane (anti-GBM) disease

Cryoglobulinemic vasculitis (CV)

IgA vasculitis (Henoch-Schnlein) (IgAV)

Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis)

Variable vessel vasculitis (VVV)

Behcets disease (BD)

Cogans syndrome (CS)

Single-organ vasculitis (SOV)

Cutaneous leukocytoclastic angiitis

Cutaneous arteritis

Primary central nervous system vasculitis - Isolated aortitis

Others

Vasculitis associated with systemic disease

Lupus vasculitis

Rheumatoid vasculitis

Sarcoid vasculitis

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