Systemic Sclerosis
This book provides a comprehensive review of the recent advances in the research of systemic sclerosis (scleroderma). Systemic sclerosis is a complex autoimmune disease characterized by vasculopathy, fibrosis, and immune activation. This disease is also
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Systemic Sclerosis Basic and Translational Research
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Systemic Sclerosis
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Kazuhiko Takehara • Manabu Fujimoto • Masataka Kuwana Editors
Systemic Sclerosis Basic and Translational Research
Editors Kazuhiko Takehara Department of Dermatology Kanazawa University Kanazawa, Ishikawa Japan
Manabu Fujimoto Department of Dermatology University of Tsukuba Tsukuba, Ibaraki Japan
Masataka Kuwana Department of Allergy and Rheumatology Nippon Medical School Tokyo, Tokyo Japan
ISBN 978-4-431-55707-4 ISBN 978-4-431-55708-1 DOI 10.1007/978-4-431-55708-1
(eBook)
Library of Congress Control Number: 2015958785 Springer Tokyo Heidelberg New York Dordrecht London © Springer Japan 2016 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, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. Printed on acid-free paper Springer Japan KK is part of Springer Science+Business Media (www.springer.com)
Preface
Systemic sclerosis (scleroderma) is a most challenging disease to treat. Great progress has been made in the treatment of specific symptoms, including renal crisis with angiotensin-converting enzyme inhibitors reflux esophagitis with proton-pump inhibitors and pulmonary arterial hypertension with prostanoids, endothelin receptor antagonists, and phosphodiesterase type 5 inhibitors; however, there are still no disease-modifying drugs that are able to control the overall disease activity satisfactorily. This is partly because, whereas other connective tissue diseases appear primarily mediated by immune dysregulation, systemic sclerosis has a tremendously complex pathogenesis comprising several distinct components of microvasculopathy, excessive fibrosis, and immune activation and autoimmunity. Thus, to develop a new concept of therapy based on the pathogenesis of the disease is needed. Recent studies nonetheless have revealed a variety of new evidence that suggests clues to the pathogenesis of the disease. This book provides a comprehensive review of the recent advances in the research of systemic sclerosis. First,
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