ADVANCES IN THE ETIOLOGY, PATHOGENESIS AND PATHOLOGY OF VASCULITIS Edited by Luis M. Amezcua-Guerra Advances in the Etiology, Pathogenesis and Pathology of Vasculitis Edited by Luis M. Amezcua-Guerra Published by InTech Janeza Trdine 9, 51000 Rijeka, Croatia Copyright © 2011 InTech All chapters are Open Access articles distributed under the Creative Commons Non Commercial Share Alike Attribution 3.0 license, which permits to copy, distribute, transmit, and adapt the work in any medium, so long as the original work is properly cited. After this work has been published by InTech, authors have the right to republish it, in whole or part, in any publication of which they are the author, and to make other personal use of the work. Any republication, referencing or personal use of the work must explicitly identify the original source. Statements and opinions expressed in the chapters are these of the individual contributors and not necessarily those of the editors or publisher. No responsibility is accepted for the accuracy of information contained in the published articles. The publisher assumes no responsibility for any damage or injury to persons or property arising out of the use of any materials, instructions, methods or ideas contained in the book. Publishing Process Manager Dragana Manestar Technical Editor Teodora Smiljanic Cover Designer Jan Hyrat Image Copyright Lightspring, 2011. Used under license from Shutterstock.com First published September, 2011 Printed in Croatia A free online edition of this book is available at www.intechopen.com Additional hard copies can be obtained from orders@intechweb.org Advances in the Etiology, Pathogenesis and Pathology of Vasculitis, Edited by Luis M. Amezcua-Guerra p. cm. ISBN 978-953-307-651-5 free online editions of InTech Books and Journals can be found at www.intechopen.com Contents Preface IX Part 1 Contributions on the Etiology of Vasculitis 1 Chapter 1 Transcriptome Signature of Nipah Virus Infected Endothelial Cells 3 Mathieu Cyrille, Legras-Lachuer Catherine and Horvat Branka Chapter 2 Takayasu’s Arteritis and Its Potential Pathogenic Association with Mycobacterium tuberculosis 21 Luis M. Amezcua-Guerra and Diana Castillo-Martínez Chapter 3 Mycoplasma pneumoniae as an Under- Recognized Agent of Vasculitic Disorders 37 Mitsuo Narita Chapter 4 Vasculitis: Endothelial Dysfunction During Rickettsial Infection 57 Yassina Bechah, Christian Capo and Jean-Louis Mege Chapter 5 Responsible Genetic Factors for Vasculitis in Kawasaki Disease 71 Yoshihiro Onouchi and Akira Hata Chapter 6 The Role of Proteinase 3 and Neutrophils in ANCA-Associated Systemic Vasculitis 93 Mohamed Abdgawad Part 2 Pathogenesis and Pathology of Vasculitis 113 Chapter 7 Pathology of the Cutaneous Vasculitides: A Comprehensive Review 115 Adrienne C. Jordan, Stephen E. Mercer, and Robert G. Phelps Chapter 8 Endothelial Cells and Vasculitis 153 Vidosava B. Djordjević, Vladan Ćosić, Lilika Zvezdanović-Čelebić, Vladimir V. Djordjević and Predrag Vlahović VI Contents Chapter 9 Markers of Vascular Damage and Repair 179 Uta Erdbruegger, Ajay Dhaygude and Alexander Woywodt Chapter 10 Clinical Relevance of Cytokines, Chemokines and Adhesion Molecules in Systemic Vasculitis 195 Tsuyoshi Kasama, Ryo Takahashi, Kuninobu Wakabayashi and Yusuke Miwa Part 3 General Overviews in Vasculitis 223 Chapter 11 Wegener’s Granulomatosis 225 Lígia Peixoto, Patrício Aguiar, Filipe Veloso Gomes, João Espírito Santo, Nuno Marques, Ilídio Jesus and J. M. Braz Nogueira Chapter 12 The Etiology, Mechanisms, and Treatment of Churg-Strauss Syndrome 235 Tsurikisawa N., Saito H., Oshikata C., Tsuburai T. and Akiyama K. Chapter 13 Churg-Strauss Syndrome: Clinical and Immunological Features 255 Khrystyna Lishchuk-Yakymovych, Valentyna Chopyak and Roman Pukalyak Chapter 14 Drug-Induced Vasculitis 275 Mislav Radić Chapter 15 Drug Induced Small Vessel Vasculitis 287 Jorge Daza Barriga, Mónica Manga Conte and Arturo Valera Agámez Chapter 16 Hepatitis C Related Vasculitides 301 Reem H. A. Mohammed and Hesham I El-Makhzangy Part 4 Selected Issues in Vasculitis 333 Chapter 17 Audiovestibular Manifestations in Systemic Vasculitis: An Update 335 Juan Carlos Amor-Dorado and Miguel Angel Gonzalez-Gay Chapter 18 Vasculitis of the Central Nervous System – A Rare Cause of Stroke 349 Małgorzata Wiszniewska and Anna Członkowska Chapter 19 Vasculitis as a Cause of First-Ever Stroke 363 Malgorzata Wiszniewska and Julien Bogousslavsky Chapter 20 Acute Hemorrhagic Edema of Infancy 375 Hayrullah Alp Contents VII Chapter 21 The LAMP Story and What It Means for ANCA Positive Vasculitis in Nephrology 395 Hansjörg Rothe Chapter 22 Quality of Life Issues in Vasculitis 405 Delesha Carpenter and Robert F. DeVellis Chapter 23 Kawasaki Disease, Others Heart Injuries, Not Only Coronary Arteritis 421 Norberto Sotelo-Cruz Preface “But there were also other fevers, as will be described. Many had their mouths affected with aphthous ulcerations. There were also many defluxions about the genital parts, and ulcerations, boils (phymata), externally and internally, about the groins. Watery ophthalmies of a chronic character, with pains; fungous excrescences of the eyelids, externally and internally, called fig, which destroyed the sight of many persons. There were fungous growths, in many other instances, on ulcers, especially on those seated on the genital organs”. This archetypal description of the Adamantiades-Behçet´s disease remains as valid today as when it was detailed by Hippokrates of Kos (460-377 BC) in his Epidemion, book III, part 7 (Hipp. Epid. 3.3.7). Nevertheless, in these last 2500 years we have advanced a lot in the knowledge of vasculitis, a fascinating array of life-threatening and minor diseases caused by inflammatory conditions that affect the blood vessels. Indeed, research in immunology has invigorated the entire field of vasculitis, shaping a rational approach to its etiology, pathogenesis, diagnosis and treatment, which is the matter of the present book. This is not a textbook on vasculitis, since it was never intended as a compilation of comprehensive reviews. Rather, it represents the view of each author on selected topics related to vasculitis, verifying the scientific evidence with their own expertise. In other words, this book represents the amalgam between an evidence-based medicine to one based on eminence. Only outstanding experts within defined scientific fields of research in vasculitis from all over the world were invited to participate in this publication. This resulted in an exciting combination of original contributions, structured reviews, overviews, state-of the-art articles, and even the proposal of novel etiopathogenetic models of disease. Organizing this diversity of manuscripts has not been easy, and I am not certain how long will take readers to cover this book from beginning to end, but all the authors have endeavored to draw them into this volume by keeping both the text and the accompanying figures and tables lucid and memorable. This book has been intended to provide a broad base upon which one can build additional knowledge acquired from other sources. X Preface I invite you to read both consecutive but separable books on Vasculitis to better understand these fascinating but complex diseases. Advances in the Etiology, Pathogenesis and Pathology of Vasculitis begins with contributions on the etiology of vasculitis, how some pathogens may interact with the host’s immune system to induce autoimmune-mediated tissue injury, how different genes may confer risk for vasculitis and how some antibodies may become pathogenic. The following section deals on the pathology of vasculitis and the potential role of endothelial cells and cytokines in vascular damage and repair. We next find chapters summarizing the latest information on several primary and secondary vasculitis syndromes, to conclude with the coverage of selected topics such as organ-specific vasculitic involvement and quality of life issues in vasculitis. I am thankful to all the contributing authors. Their expert knowledge and experience has guaranteed a thoughtful and innovative approach for rheumatologists, nephrologists and other specialists interested in the fascinating field of vasculitis. Each author must be certain that their efforts will benefit to all patients suffering from these serious diseases. I am also grateful to Aleksandar Lazinica for this kind invitation to edit the present book; thank you for your confidence. Off note, this book could not have been edited without the dedicated technical assistance of the publishing process managers, Petra Zobic and Dragana Manestar; thank you for your patience and willingness. What began for Celsus as Rubor et tumor cum calore et dolore and led to Virchow’s Functio laesa has grown beyond the therapeutic targeting of cytokines. As editor, I hope that some of the enthusiasm and excitement of the contributing authors may be shared by each reader of this book. Dr. Luis M. Amezcua-Guerra, MD Department of Immunology The National Institute of Cardiology ʺIgnacio Chávezʺ Mexico City Mexico [...]... to severe 14 Advances in the Etiology, Pathogenesis and Pathology of Vasculitis damages on the endothelium, including systemic vasculitis in case of Lupus (Zimmer et al., 1998) and may be therefore involved in the pathogenesis of NiV induced vasculitis In addition, our results revealed NiV-induced up regulation of HERC5 (FC=5,37), which belongs to the E3 ubiquitin ligases family This protein has been... could be involved in the control of the vascular inflammation and disruption of endothelium, allowing the passage of the virus in the organs The early NiV infection of endothelial cells importantly upregulated the chemokines TNFSF13B, CXCL10, CXCL11 and CCL8 that are involved in many processes of autoimmune diseases as well as proteins belonging to the ubiquitination pathway More precisely, TAP1 and LMP2... possible link is not futile because the potential risk of using anti-tumour necrosis factor (TNF) therapies in the treatment of patients with Takayasus arteritis and the increasing use of Bacille CalmetteGuộrin (BCG) for vaccination purposes around the world 22 Advances in the Etiology, Pathogenesis and Pathology of Vasculitis In this chapter we will discuss the main epidemiological, immunological and genetic... pathway is the interferon signaling pathway (p=0,01) (Fig 4 B) The majority of the top 15 up-regulated genes are related to the Interferon pathway (Table 1B) The involvement of the Interferon pathway has been proposed in the development of the other types of vasculitis, including the postoperative vasculitis (Abe et al., 2008) Four other canonical pathways were significantly found modified during the infection... changes in the neurological diseases function (15 genes) and nervous dysfunctions (5 genes) This result could be correlated with the strong involvement of the endothelial cell-induced inflammatory reaction in the development of the encephalitis, as described in the introduction To refine the significance of these up-regulated genes, we next investigated the biological functions and interactions of these... socio-economical and health problems This virus induces a generalized vasculitis leading to the disruption of the Transcriptome Signature of Nipah Virus Infected Endothelial Cells 15 endothelial microvascular tissue in brain and inducing severe damages in the CNS Micorarray analysis of NiV infected primary endothelial cells allowed us to obtain a global overview of the host cell responses to NiV early during the infection... presence of the NiV in this tissue Virus may propagate initially within the lymphoid tissue, leading to the infection of the endothelial cells, recognized as the first primary targets of NiV Those cells allow the second cycle of replication of the virus and the viremia NiV infection is characterized by the formation of syncytia leading to the endothelial damages, which are thought to be the cause of thrombosis,... basal lamina plays a critical role in brain injury (Wang & Shuaib, 2007), the loss of basal lamina components may reflect the degradation of proteins by proteolitic enzymes 12 Advances in the Etiology, Pathogenesis and Pathology of Vasculitis Fig 5 Gene network identified in NiV infected HUVEC, compared to mock infected controls, reveals involvement of different genes within the pathways of Gene expression,... adenopathy, and Bazins erythema induratum; in sharply contrast, the prevalence of active tuberculosis was reported to be 0.028% in the general population from Mexico (Lupi-Herrera et al., 1977) 6 Bacille Calmette-Guộrin (BCG) vaccination and tuberculin skin tests in Takayasus arteritis Mantoux screening test is the main tuberculin reaction used in the world It consists of an intradermal injection of a standard... while the role of other genes rests to be demonstrated 10 Advances in the Etiology, Pathogenesis and Pathology of Vasculitis Table 1 Genes differentially expressed during NiV infection in the Immune Response (A), Interferon pathway (B), protein ubiquitination pathway (C) Transcriptome Signature of Nipah Virus Infected Endothelial Cells 11 Fig 4 Impact of NiV infection on biological functions (A) and . ADVANCES IN THE ETIOLOGY, PATHOGENESIS AND PATHOLOGY OF VASCULITIS Edited by Luis M. Amezcua-Guerra Advances in the Etiology, Pathogenesis and Pathology of Vasculitis. who survived the encephalitis had relapsed during the year following their infection without any reexposure to virus. In Advances in the Etiology, Pathogenesis and Pathology of Vasculitis . risk for vasculitis and how some antibodies may become pathogenic. The following section deals on the pathology of vasculitis and the potential role of endothelial cells and cytokines in vascular