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FOURTH EDITION RHEUMATOLOGY STERLING G. WEST, MD, MACP, MACR Professor of Medicine Department of Medicine Division of Rheumatology University of Colorado School of Medicine Aurora, Colorado JASON KOLFENBACH, MD, FACR Associate Professor of Medicine Department of Medicine Division of Rheumatology University of Colorado School of Medicine Aurora, Colorado Elsevier 1600 John F. Kennedy Blvd. Ste 1800 Philadelphia, PA 19103-2899 RHEUMATOLOGY SECRETS, FOURTH EDITION ISBN: 978-0-323-64186-9 Copyright © 2020 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). Notice Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information, methods, compounds or experiments described herein. Because of rapid advances in the medical sciences, in particular, independent verification of diagnoses and drug dos- ages should be made. To the fullest extent of the law, no responsibility is assumed by Elsevier, authors, editors or contributors for any injury and/or damage to persons or property as a matter of products liability, negligence or otherwise, or from any use or operation of any methods, products, instructions, or ideas contained in the material herein. Previous editions copyrighted 2015, 2002, and 1996. Library of Congress Control Number: 2019950664 Content Strategist: Marybeth Thiel Content Development Specialist: Angie Breckon Publishing Services Manager: Deepthi Unni Project Manager: Radjan Lourde Selvanadin Design Direction: Bridget Hoette Printed in the United States of America Last digit is the print number: 9 8 7 6 5 4 3 2 1 DEDICATION I wish to dedicate this book: To my wife, Brenda, my best friend. To my children, Dace and Matthew, the joys of my life. To my amazing grandchildren, Aidan, Eva, Owen, and Stella. Sterling West, MD To my wife, Lauren, a tireless mother of three, a patient wife to myself, and an amazing clinician whose advice on patient communication continues to shape my practice. Thank you for encouraging me to explore the rabbit-holes of Rheumatology, and for always pulling me out to enjoy the world outside of medicine. Jason Kolfenbach, MD PREFACE In the past year, we have been extensively authorized, approved, inspected, renovated, elevated, visited, consulted, circularized, informed; and have completed so many forms, orders, questionnaires, and reports that no medical progress has been made. Rudolf Virchow Berlin, 1865 With all the current regulatory changes, many physicians might agree with Virchow’s lament. Certainly, the regulations and documentation requirements continue to increase. Fortunately, there has been continued medical progress in Rheumatology over the past five years since the last edition of Rheumatology Secrets. I continue to be amazed with the advances that have occurred in both the science and treatment of the rheumatic diseases. Each chapter in the present edition has been extensively reviewed and updated to include those advancements through July 2018. As in the previous editions, Rheumatology Secrets, 4th Edition, is presented in the Socratic question and answer format that is the hallmark of The Secrets Series. The chapters are organized into 16 sections, most with a common theme emphasized by an introductory quotation. Common and uncommon rheumatic disease problems that we encounter in clinical practice, discuss during teaching rounds, and find on board examinations are covered. Each chapter reviews basic immunology and pathophysiology, important disease manifestations, and practical management issues. The book also contains a wealth of mnemonics, lists, tables, figures, and illustrations to emphasize important points. Chapter 2 lists some of the top Rheumatology Secrets and I encourage readers to send in their top secrets. A new section added to the electronic version of Rheumatology Secrets is the inclusion of 100 review questions. Although written in a Board Review format, the purpose of these questions is to be used as an application of the knowledge contained in this book. Therefore, an attempt has been made to include at least one question on subject matter from each chapter that may be useful in caring for patients with rheumatic diseases. Consequently, those who use this for “Board Review” will find some questions that seem too easy, some that may be too hard, and some that would not be included on a certification exam but may be useful knowledge to have in clinic. I hope that the reader will find Rheumatology Secrets, 4th Edition, and the accompanying review questions both enjoyable and useful in daily practice. This edition marks my last for Rheumatology Secrets. After four editions, it is time to turn over the position of editor to my colleague, Jason Kolfenbach, MD, who has done an excellent job co-editing this edition with me. I have been lucky enough to develop and expand this book over the years and I have no doubt that future editions will be even better. I want to close by thanking all my colleagues, fellows, students, and patients for what they have taught me and contributed to my career. Sterling West, M.D., MACP, MACR vi CONTRIBUTORS Venu Akuthota, MD, FAAPMR Richard Brasington, MD, FACP, FACR Chair and Professor of Physical Medicine and Professor of Medicine Rehabilitation Rheumatology and Opthalmology Spine Center Fellowship Program Director University of Colorado Hospital Rheumatology Aurora, Colorado Washington University in St. Louis St. Louis, Missouri Itziar Quinzaños Alonso, MD, FACR Department of Medicine Division of Rheumatology Amy C. Cannella, MD, MS, FACR University of Colorado School of Medicine Associate Professor Aurora, Colorado Internal Medicine and Rheumatology Department of Medicine Division of Rheumatology University of Nebraska Medical Center Denver Health and Hospital Veterans Affairs Medical Center Denver, Colorado Omaha, Nebraska Marsha S. Anderson, MD Liron Caplan, MD, PhD, FACR Associate Professor of Pediatrics Section Head, Rheumatology Assistant Dean for Longitudinal Curriculum Rocky Mountain Regional Veterans Affairs Medical Center University of Colorado School of Medicine Associate Professor of Medicine/Rheumatology Children’s Hospital Colorado University of Colorado School of Medicine Aurora, Colorado Denver, Colorado Ramon A. Arroyo, MD, FACR Courtney B. Crayne, MD, FACR Associate Professor of Medicine Pediatric Rheumatology Uniformed Services University of the Health Sciences University of Alabama—Birmingham Rheumatology Birmingham, Alabama Brooke Army Medical Center Randy Q. Cron, MD, PhD, FACR San Antonio, Texas Professor of Pediatrics & Medicine Wais Afzal, MD, FACR Pediatric Rheumatology Rheumatology University of Alabama—Birmingham University of Arkansas for Medical Sciences Birmingham, Alabama Little Rock, Arkansas Laura Damioli, MD Amrita Bath, MD, FACR Assistant Professor of Medicine Assistant Professor Division of Infectious Diseases Division of Rheumatology Director of Orthopedic Infectious Disease University of Kansas School of Medicine University of Colorado School of Medicine Kansas City, Kansas Aurora, Colorado Daniel F. Battafarano, DO, MACP, FACR Lisa Davis, MD, MSCS, FACR Professor of Medicine Associate Professor of Medicine Uniformed Services University of the Health Sciences Rheumatology Bethesda, Maryland Denver Health Division of Rheumatology Denver, Colorado San Antonio Military Medical Center Associate Program Director, Internal Medicine San Antonio, Texas University of Colorado School of Medicine Aurora, Colorado Seth Mark Berney, MD, FACR Professor of Medicine and Chief Kevin D. Deane, MD, PhD, FACR Division of Rheumatology Associate Professor of Medicine University of Arkansas for Medical Sciences Division of Rheumatology Little Rock, Arkansas University of Colorado School of Medicine Aurora, Colorado vii viii CONTRIBUTORS Kristen Demoruelle, MD, PhD, FACR Erica M. Hill, DO, FACP, FACR Assistant Professor of Medicine Assistant Professor of Medicine Division of Rheumatology Uniformed Services University of Health Science University of Colorado School of Medicine Bethesda, Maryland Aurora, Colorado Program Director, Rheumatology Fellowship Rheumatology Division Stacy Dixon, MD, PhD Department of Medicine Assistant Professor of Neurology San Antonio Military Medical Center University of Colorado School of Medicine San Antonio, Texas Aurora, Colorado Mary K. Jesse, MD Samuel R. Dominguez, MD, PhD Associate Professor of Radiology and Orthopaedics Associate Professor of Pediatrics Chief of Musculoskeletal Radiology Pediatric Infectious Diseases University of Colorado School of Medicine University of Colorado School of Medicine Denver, Colorado Children’s Hospital Colorado Aurora, Colorado Steven C. Johnson, MD, FACP, FIDSA Professor of Medicine Donald G. Eckhoff, MD, MS, FACS Division of Infectious Diseases Professor of Orthopaedics University of Colorado School of Medicine Department of Orthopaedics Aurora, Colorado University of Colorado School of Medicine Aurora, Colorado Shannon Kasperbauer, MD Assistant Professor of Medicine Alan R. Erickson, MD, FACR Division of Mycobacterial and Respiratory Assistant Professor of Medicine Infections Rheumatology National Jewish Health UNMC Physicians-Brentwood Denver, Colorado LaVista, Nebraska Lindsay Kelmenson, MD, MSCS, FACR Michael G. Feely, MD, FACR Instructor of Medicine Assistant Professor of Medicine Division of Rheumatology Division of Rheumatology and Immunology University of Colorado School of Medicine University of Nebraska Medical Center Aurora, Colorado Omaha, Nebraska Jason R. Kolfenbach, MD, FACR Aryeh Fischer, MD, FACR Associate Professor of Medicine Associate Professor of Medicine Division of Rheumatology University of Colorado School of Medicine University of Colorado School of Medicine Aurora, Colorado Aurora, Colorado Robert C. Fuhllbrigge, MD, PhD Vinisha Kota, MD, FACR Professor of Pediatrics Rheumatology University of Colorado School of Medicine University of Arkansas for Medical Sciences Aurora, Colorado Little Rock, Arkansas Chair, Rheumatology Children’s Hospital Colorado Kristine A. Kuhn, MD, PhD, FACR Aurora, Colorado Assistant Professor Department of Medicine Alexandra Garnett, MD Division of Rheumatology Department of Physical Medicine and Rehabilitation University of Colorado School of Medicine University of Colorado Hospital Aurora, Colorado Aurora, Colorado Bharat Kumar, MD, MMEd, FACP, FACR Alison M. Gizinski, MD, MS, FACR Clinical Assistant Professor of Medicine Emory University School of Medicine Division of Immunology/Rheumatology and Allergy Division of Rhematology University of Iowa Carver College of Medicine Atlanta, Georgia Iowa City, Iowa Melissa Grifﬁth, MD, FACR Jamie Lai, MD, FACR Instructor in Medicine Assistant Professor of Pediatrics University of Colorado School of Medicine Pediatric Rheumatology Aurora, Colorado Baylor College of Medicine Rebecca Grifﬁth, BS, MD Houston, Texas Orthopedic Surgery University of Colorado School of Medicine Aurora Colorado CONTRIBUTORS ix Katrina M. Lawrence-Wolff, DO, FACR Joel Brian Shirley, DO, FACR Associate Professor of Medicine Assistant Professor of Pediatrics Uniformed Services University of the Health Sciences Pediatrics—Rheumatology Bethesda, Maryland University of Colorado School of Medicine Rheumatology Children’s Hospital Colorado San Antonio Military Medical Center Aurora, Colorado San Antonio, Texas Namrata Singh, MD, MCSI, FACP Tiffany Lin, MD, FACR Clinical Assistant Professor Assistant Professor of Medicine Kelting Family Scholar in Rheumatology Associate Program Director, Rheumatology Fellowship Divsion of Immunology/Rheumatology and Allergy University of Wisconsin School of Medicine and University of Iowa Carver College of Medicine Public Health Iowa City, Iowa Madison, Wisconsin Marcus H. Snow, MD, FACR James S. Louie, MD, FACP, MACR Assistant Professor Professor of Medicine Division of Rheumatology UCLA School of Medicine University of Nebraska Medical Center Los Angeles, California Omaha, Nebraska Michael T. McDermott, MD, FACP Jennifer Stichman, MD FACP, FACR Professor of Medicine and Clinical Pharmacology Assistant Professor Division of Endocrinology, Metabolism, and Diabetes General Internal Medicine and Rheumatology University of Colorado School of Medicine Denver Health Medical Center Aurora, Colorado Denver, Colorado Kevin McKown, MD, FACR Tho Truong, MD, FACR Professor of Medicine Associate Professor of Medicine Head, Division of Rheumatology Allergy and Clinical Immunology University of Wisconsin School of Medicine and Division of Rheumatology Public Health National Jewish Health Madison, Wisconsin Denver, Colorado Ted R. Mikuls, MD, MSPH, FACR Kim Nguyen Tyler, MD, FACR Umbach Professor of Rheumatology Assistant Clinical Professor of Medicine Division of Rheumatology Division of Rheumatology University of Nebraska Medical Center University of Colorado School of Medicine Omaha, Nebraska Aurora, Colorado Katharine F. Moore, MD, FACR Colin Strickland, MD Assistant Professor of Pediatrics Associate Professor of Radiology University of Colorado School of Medicine University of Colorado School of Medicine Pediatric Rheumatology Aurora, Colorado Children’s Hospital Colorado Christopher C. Striebich, MD, PhD, FACR Aurora, Colorado Associate Professor Alan G. Palestine, MD Division of Rheumatology Professor of Ophthalmology University of Colorado School of Medicine Professor of Medicine-Rheumatology Aurora, Colorado Sue Anschutz-Rogers Eye Center Christine M. Swanson, MD, MCR Department of Ophthalmology Assistant Professor University of Colorado Division of Endocrinology, Metabolism and Diabetes Aurora, Colorado University of Colorado Duane Pearson, MD, FACR Aurora, Colorado Associate Professor Jeanne K. Tofferi, MD, MPH, FACP, FACR Rheumatology Assistant Professor of Medicine University of Colorado School of Medicine Uniformed Services University of Health Science Aurora, Colorado Bethesda, Maryland Dianna Quan, MD, FAAN, FANA Chief, Rheumatology Division Professor of Neurology Department of Medicine University of Colorado School of Medicine San Antonio Military Medical Center Aurora, Colorado San Antonio, Texas x CONTRIBUTORS Armando F. Vidal, MD Sterling G. West, MD, MACP, MACR Associate Professor Professor of Medicine Department of Orthopedics Department of Medicine University of Colorado School of Medicine Division of Rheumatology Aurora, Colorado University of Colorado School of Medicine Aurora, Colorado Scott Vogelgesang, MD, FACP, FACR Clinical Professor Matthew Wicklund, MD, FAAN Director, Division of Immunology/Rheumatology and Professor of Neurology Allergy Department of Neurology M. Paul Strottmann Family Chair of Medical Student University of Colorado Education and Physician Training Aurora, Colorado University of Iowa Carver College of Medicine Patrick R. Wood, MD, FACR Iowa City, Iowa Assistant Professor Matthew P. Wahl, MD University of Colorado School of Medicine Clinical Assistant Professor Rocky Mountain Regional Veterans Affairs Division of Endocrinology and Metabolism Medical Center University of Utah School of Medicine Aurora, Colorado Salt Lake City, Utah JoAnn Zell, MD, FACR Elena Weinstein, MD, FACR Assistant Professor of Medicine Assistant Professor University of Colorado School of Medicine Medicine, Division of Rheumatology Aurora, Colorado University of Colorado School of Medicine Aurora, Colorado Matthew S. West, MD Assistant Professor Department of Neurology University of Colorado School of Medicine Aurora, Colorado ACKNOWLEDGMENTS The editors want to thank: All the contributors for their time and effort in writing their chapters, the staff at Elsevier for their patience and help, and for giving us the opportunity to edit Rheumatology Secrets. To our patients, teachers, and students for what they have taught us. xi