Textbook of PEDIATRIC RHEUMATOLOGY SEVENTH EDITION Textbook of PEDIATRIC RHEUMATOLOGY Ross E. Petty Ronald M. Laxer Carol B. Lindsley Lucy R. Wedderburn 1600 John F. Kennedy Blvd. Ste 1800 Philadelphia, PA 19103-2899 TEXTBOOK OF PEDIATRIC RHEUMATOLOGY, ISBN: 978-0-323-24145-8 SEVENTH EDITION Copyright © 2016 by Elsevier, Inc. All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Details on how to seek permission, further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency, can be found at our website: www.elsevier.com/permissions. This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may be noted herein). Notices Knowledge and best practice in this field are constantly changing. As new research and experience broaden our understanding, changes in research methods, professional practices, or medical treatment may become necessary. Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds, or experiments described herein. In using such information or methods they should be mindful of their own safety and the safety of others, including parties for whom they have a professional responsibility. 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Library of Congress Cataloging-in-Publication Data Textbook of pediatric rheumatology. Textbook of pediatric rheumatology / [edited by] Ross E. Petty, Ronald M. Laxer, Carol B. Lindsley, Lucy Wedderburn. – 7th edition. p. ; cm. Textbook of pediatric rheumatology Pediatric rheumatology Preceded by Textbook of pediatric rheumatology / [edited by] James T. Cassidy … [et al.]. 6th ed. c2011. Includes bibliographical references and index. ISBN 978-0-323-24145-8 (hardcover : alk. paper) I. Petty, Ross E., editor. II. Lindsley, Carol B., editor. III. Laxer, Ronald M., editor. IV. Wedderburn, Lucy, editor. V. Title. VI. Title: Textbook of pediatric rheumatology. VII. Title: Pediatric rheumatology. [DNLM: 1. Rheumatic Diseases. 2. Arthritis. 3. Child. 4. Connective Tissue Diseases. 5. Vasculitis. WE 544] RJ482.R48 618.92′723–dc23 2015004728 Content Strategist: Michael Houston Content Development Specialist: Laura Schmidt Publishing Services Manager: Patricia Tannian Project Manager: Kate Mannix Design Direction: Paula Catalano Printed in China Last digit is the print number: 9 8 7 6 5 4 3 2 1 To the memory of James Thomas Cassidy: scholar, mentor, colleague, and friend. To Beryl, Edda, Bart, and Jerry. And to our patients, who continue to inspire us. P R E FAC E The publication of the seventh edition of the Textbook of Pediatric contributions to previous editions of the book have been so important: Rheumatology marks 33 years since the publication of the first edition. Dr. Balu Athreya, Dr. Michael Dillon, Dr. Allison Eddy, Dr. Fernanda The changes in the discipline of pediatric rheumatology during that Falcini, Dr. Edward Giannini, Dr. Wietse Kuis, Dr. Deborah Wenkert, time have been profound, and the Textbook has attempted to keep up and the late Dr. David Glass. The editors are indebted to the current with important developments in our understanding of rheumatic authors for their willingness to share their expertise and for the time diseases in childhood and their diagnosis and management. The Text- and effort put into their contributions. book will provide an up-to-date synthesis of the state of knowledge of The death of Jim Cassidy, the founding editor, is noted with sadness rheumatic diseases of childhood for students, physicians, and health and a sense of loss, and the editors have endeavored to maintain and care professionals involved in the management of these children and enhance the intellectual standard and clarity of writing he espoused in adolescents. the first six editions. To that end, this edition incorporates new chapters and contribu- The editors and authors are grateful to the thousands of readers of tions from new authors. The challenge of maintaining a book of rea- the Textbook who have made it the standard reference in the field of sonable size while incorporating new information has necessitated pediatric rheumatology. deleting some old information. Some chapters have been shortened, and all chapters have been thoroughly revised, updated, and, in some Ross E. Petty instances, rewritten. The globalization of the specialty of pediatric Ronald M. Laxer rheumatology is recognized by the inclusion of information about Carol B. Lindsley diagnosis and management of childhood rheumatic diseases in the Lucy R. Wedderburn southern hemisphere. We wish to acknowledge senior authors whose vi C O N T R I B U TO R S Jonathan Akikusa, MBBS Mara L. Becker, MD, MSCE Paediatric Rheumatologist Director, Division of Rheumatology Rheumatology Service, Department of General Medicine Department of Pediatrics Paediatrician Children’s Mercy Kansas City Department of General Medicine Kansas City, Missouri Royal Children’s Hospital Melbourne, Victoria, Australia Susanne M. Benseler, MD, PhD Section Chief, Rheumatology Salvatore Albani, MD, PhD Department of Pediatrics Director Alberta Children’s Hospital; Translational Research for Infectious and Inflammatory Disease Associate Professor Center Department of Pediatrics Adjunct Professor University of Calgary Sanford Burnham Medical Research Institute Calgary, Alberta, Canada La Jolla, California Timothy Beukelman, MD, MSCE Roger Allen, MBBS, FRACP Associate Professor Department of Rheumatology Department of Pediatrics Royal Children’s Hospital Division of Rheumatology Melbourne, Victoria, Australia University of Alabama at Birmingham Birmingham, Alabama Khaled Alsaeid, MD Professor of Pediatrics Paul Brogan, PhD, FRCPCH Department of Pediatrics Reader in Vasculitis Kuwait University Honorary Consultant Paediatric Rheumatologist Jabryah, Kuwait Infection Immunity and Rheumatology Section University College London Institute of Child Health Tadej Avčin, MD, PhD London, Great Britain Professor of Pediatrics Department of Pediatrics Hermine I. Brunner, MD, MSc, MBA University of Ljubljana; Professor of Pediatrics Head Division of Rheumatology Department of Allergology, Rheumatology, and Clinical Immunology Cincinnati Children’s Hospital Medical Center Children’s Hospital, University Medical Center University of Cincinnati Ljubljana, Slovenia Cincinnati, Ohio Paul S. Babyn, MDCM, FRCPC Rubèn Burgos-Vargas, MD Professor and Head of Medical Imaging Investigator in Medical Sciences Saskatoon Health Region Department of Rheumatology University of Saskatchewan General Hospital of Mexico; Saskatoon, Saskatchewan, Canada Professor of Medicine National Autonomous University of Mexico Arvind Bagga, MD Mexico City, Mexico Professor Department of Pediatrics Jill Buyon, MD All India Insitute of Medical Sciences Director New Delhi, India Division of Rheumatology Director Karyl S. Barron, MD Lupus Center Deputy Director, Division of Intramural Research Departments of Joint Disease and Medicine National Institute of Allergy and Infectious Diseases Center for Musculoskeletal Care National Institutes of Health New York University Langone Medical Center Bethesda, Maryland New York, New York vii viii CONTRIBUTORS David A. Cabral, MBBS Ciarán M. Duffy, MBBCh, MSc, FRCPC, FRCPI Ross Petty Chair and Clinical Professor Professor and Chairman Department of Pediatrics Department of Paediatrics Division of Rheumatology University of Ottawa; British Columbia Children’s Hospital Chief of Paediatrics University of British Columbia Department of Paediatrics Vancouver, British Columbia, Canada Children’s Hospital of Eastern Ontario Ottawa, Ontario, Canada Sharon Choo, MBBS, FRACP, FRCPA Departments of Allergy and Immunology and Laboratory Services Despina Eleftheriou, MBBS,PhD Royal Children’s Hospital Senior Lecturer Parkville, Australia Paediatric and Adolescent Rheumatology Department Institute of Child Health and Great Ormond Street Hospital Rolando Cimaz, MD London, United Kingdom Associate Professor Department of Pediatrics Brian M. Feldman, MD, MSc, FRCPC University of Florence Division Head, Rheumatology Florence, Italy Department of Rheumatology The Hospital for Sick Children; Robert Allen Colbert, MD, PhD Professor Senior Investigator Departments of Pediatrics & Medicine and Health Policy National Institute of Arthritis, Musculoskeletal, and Skin Diseases Management & Evaluation National Institutes of Health Dalia Lana School of Public Health Bethesda, Maryland University of Toronto Toronto, Ontario, Canada William G. Cole, MBBS, MSc, PhD, FRCSC Director Polly J. Ferguson, BS, MD Pediatric Surgery Director University of Alberta Pediatric Rheumatology Edmonton, Alberta, Canada Associate Professor of Pediatrics—Rheumatology University of Iowa Carver College of Medicine Iris Davidson, BSR Iowa City, Iowa Children’s Program Mary Pack Arthritis Center Robert Fuhlbrigge, MD, PhD Pediatric Rheumatology Division Associate Professor of Pediatrics and Dermatology British Columbia Children’s Hospital Harvard Medical School; Vancouver, British Columbia, Canada Attending in Rheumatology Division of Immunology Fabrizio De Benedetti, MD, PhD Boston Children’s Hospital Division of Rheumatology Boston, Massachusetts Bambino Gesù Children’s Hospital Rome, Italy Marco Gattorno, MD Pediatria II Andrea S. Doria, MD, PhD, MSc G. Gaslini Institute Radiologist University of Genoa Clinician-Scientist Genoa, Italy Research Director Diagnostic Imaging Alexei A. Grom, MD The Hospital for Sick Children; Professor Associate Professor Department of Pediatrics Medical Imaging University of Cincinnati College of Medicine; University of Toronto Attending Physician Toronto, Ontario, Canada Department of Rheumatology Cincinnati Children’s Hospital Medical Center Frank Dressler, Dr. Med Cincinnati, Ohio Pediatric Rheumatologist Pediatric Pneumology, Allergology, and Neonatology The Children’s Hospital of MHH Hannover, Germany CONTRIBUTORS ix Philip J. Hashkes, MD, MSc Marisa Klein-Gitelman, MD, MPH Head, Pediatric Rheumatology Unit Professor of Pediatrics Department of Pediatrics Northwestern University Feinberg School of Medicine; Shaare Zedek Medical Center; Head, Division of Rheumatology Associate Professor of Pediatrics Ann & Robert H. Lurie Children’s Hospital of Chicago Hebrew University Medical School Chicago, Illinois Jerusalem, Israel; Associate Professor of Medicine and Pediatrics Gay Kuchta, OT Cleveland Clinic Lerner School of Medicine Children’s Program Case Western Reserve University Mary Pack Arthritis Center Cleveland, Ohio Pediatric Rheumatology Division British Columbia Children’s Hospital Kristin Houghton, MD, MSc, FRCPC, Dip Sports Med, FAAP Vancouver, British Columbia, Canada Clinical Associate Professor Department of Paediatrics Jerome Charles Lane, MD British Columbia Children’s Hospital Associate Professor of Pediatrics University of British Columbia, Northwestern University Feinberg School of Medicine Vancouver, British Columbia, Canada Division of Kidney Diseases Ann & Robert H. Lurie Children’s Hospital of Chicago Hans-Iko Huppertz, MD Chicago, Illinois Professor of Pediatrics Head and Director Ronald M. Laxer, MDCM, FRCPC Children’s Hospital (Prof.-Hess-Kinderklinik) Professor Bremen, Germany; Departments of Pediatrics and Medicine Children’s Hospital University of Toronto; Georg-August-University Division of Rheumatology Göttingen, Germany The Hospital for Sick Children Toronto, Ontario, Canada Norman T. Ilowite, MD Professor Claire LeBlanc, MD, Dip Sports Med, FRCPC Department of Pediatrics Associate Professor Albert Einstein College of Medicine; Department of Pediatrics Division Chief McGill University Pediatric Rheumatology Montreal, Quebec, Canada Children’s Hospital at Montefiore New York, New York Steven J. Leeder, PharmD, PhD Marion Merrell Dow Endowed Chair in Pediatric Clinical Edgar Jaeggi, MD Pharmacology Staff Cardiologist Director, Division of Clinical Pharmacology, Toxicology, Department of Cardiology & Therapeutic Innovation Head Department of Pediatrics Fetal Cardiac Program Children’s Mercy Kansas City The Hospital for Sick Children; Kansas City, Missouri Professor Department of Paediatrics G. Elizabeth Legger, MD University of Toronto Consultant Pediatric Rheumatologist Toronto, Ontario, Canada Department of Pediatric Rheumatology University Medical Center Groningen Daniel L. Kastner, MD, PhD Beatrix Children’s Hospital Scientific Director University of Groningen Division of Intramural Research Groningen, The Netherlands National Human Genome Research Institute Bethesda, Maryland Suzanne C. Li, MD, PhD Senior Attending Adam Kirton, MD, MSc, FRCPC Department of Pediatrics Associate Professor Joseph M. Sanzari Children’s Hospital Pediatrics & Clinical Neurosciences Hackensack University Medical Center University of Calgary Hackensack, New Jersey; Calgary, Alberta, Canada Associate Professor Department of Pediatrics Rutgers-University of Medicine and Dentistry of New Jersey, Newark, New Jersey