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HOSPITAL FOR SPECIAL SURGERY MANUAL OF
RHEUMATOLOGY AND OUTPATIENT ORTHOPEDIC
DISORDERS: DIAGNOSIS AND THERAPY
Fifth Edition
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HOSPITAL FOR SPECIAL SURGERY MANUAL OF
RHEUMATOLOGY AND OUTPATIENT ORTHOPEDIC
DISORDERS: DIAGNOSIS AND THERAPY
Fifth Edition
Editors
Associate Editor
Stephen A. Paget,
M.D.
Joseph P. Routh Professor of Medicine
Weill Medical College of Cornell University
Physician-in-Chief
Division of Rheumatology
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Allan Gibofsky,
M.D., J.D.
Professor of Medicine and Public Health
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
Professor of Law
Fordham University
New York, New York
John F. Beary III,
M.D.
Clinical Professor of Medicine
University of Cincinnati
Attending Physician
Division of Rheumatology & Immunology
Veterans Administration Medical Center
Cincinnati, Ohio
Thomas P. Sculco,
Doruk Erkan,
M.D.
Assistant Professor of Medicine
Weill Medical College of Cornell University
Associate Physician-Scientist
Barbara Volcker Center for Women
and Rheumatic Disease
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
International Editors
Josef S. Smolen,
M.D.
Professor of Medicine
Chairman, Department of Rheumatology
Medical University of Vienna
Chairman, 2nd Department of Medicine
Rheumatic Disease Center, Lainz Hospital
Vienna, Austria
Stefano Bombardieri,
M.D.
Professor of Rheumatology
Chief, Rheumatic Diseases Unit
University of Pisa
Pisa, Italy
M.D.
Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Surgeon-in-Chief
Department of Orthopedics
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Coordinator
Cookie Reyes
Clinical and Research Administrator
Hospital for Special Surgery
New York, New York
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Acquisitions Editor: Sonya Seigafuse
Managing Editor: Nancy Winter
Project Manager: Nicole Walz
Senior Manufacturing Manager: Ben Rivera
Marketing Manager: Kathy Neely
Design Coordinator: Terry Mallon
Cover Designer: Becky Baxendell
Production Services: Laserwords Private Limited
Printer: RR Donnelley
Fifth Edition
© 2006 by Lippincott Williams & Wilkins
© 2000 by Lippincott Williams & Wilkins
530 Walnut Street
Philadelphia, PA 19106
www.LWW.com
All rights reserved. This book is protected by copyright. No part of this book may be reproduced in
any form or by any means, including photocopying, or utilizing by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews.
Printed in the United States
Library of Congress Cataloging-in-Publication Data
Hospital for Special Surgery manual of rheumatology and outpatient orthopedic disorders :
diagnosis and therapy / editors, Stephen A. Paget ... [et al.] ; associate editor, Doruk Erkan ;
coordinator, Cookie Reyes ; forewords, Sir Ravinder Maini, Charles L. Christian. — 5th ed.
p. ; cm. — (Spiral manual series)
Rev. ed. of: Manual of rheumatology and outpatient orthopedic disorders. 4th ed. c2000.
Includes bibliographical references and index.
ISBN 0-7817-6300-2
1. Rheumatology—Handbooks, manuals, etc. 2. Orthopedics—Handbooks, manuals, etc.
I. Paget, Stephen A. II. Hospital for Special Surgery. III. Manual of rheumatology and
outpatient orthopedic disorders. IV. Title: Manual of rheumatology and outpatient orthopedic
disorders. V. Series: Spiral manual.
[DNLM: 1. Rheumatic Diseases—diagnosis—Handbooks. 2. Ambulatory Care—Handbooks.
3. Bone Diseases—Handbooks. 4. Rheumatic Diseases—therapy—Handbooks. WE 39 H828
2006]
RC927.M346 2006
616.7'23—dc22
2005020653
Care has been taken to confirm the accuracy of the information presented and to describe generally
accepted practices. However, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information in this book and make no warranty,
expressed or implied, with respect to the currency, completeness, or accuracy of the contents of the
publication. Application of this information in a particular situation remains the professional responsibility of the practitioner.
The authors, editors, and publisher have exerted every effort to ensure that drug selection and
dosage set forth in this text are in accordance with current recommendations and practice at the time of
publication. However, in view of ongoing research, changes in government regulations, and the constant
flow of information relating to drug therapy and drug reactions, the reader is urged to check the package
insert for each drug for any change in indications and dosage and for added warnings and precautions.
This is particularly important when the recommended agent is a new or infrequently employed drug.
Some drugs and medical devices presented in this publication have Food and Drug Administration (FDA) clearance for limited use in restricted research settings. It is the responsibility of health care
providers to ascertain the FDA status of each drug or device planned for use in their clinical practice.
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With love, we dedicate this book to our families:
Sandra Paget, Daniel, Matthew, and Lauren
Karen Gibofsky, Lewis, Esther, and Laura
Bianca Beary, John Daniel, Vanessa, Webster, and Nina
Cynthia Sculco, Peter, and Sarah Jane
And to L. Robert Vermes, Jr.
“He who saves a single life saves the world entire.”
-Talmud
And to our colleague and friend
Mary (Peggy) K. Crow, M.D.
Professor of Medicine
Weill Medical College of Cornell University
Attending Physician, Hospital for Special Surgery
President of the American College of Rheumatology 2005–2006
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CONTENTS
Acknowledgments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xiii
Forewords . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
xv
Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xvii
Contributing Authors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xix
I: MUSCULOSKELETAL DATABASE
1
Musculoskeletal History and Physical Examination . . . . . . . . . . . . . . . . .
Stephen A. Paget, Charles N. Cornell, and John F. Beary, III
1
2
Thinking Like a Rheumatologist . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
Arthur M. F. Yee
3
Immunology for the Primary Care Physician . . . . . . . . . . . . . . . . . . . . . . . 14
Mary K. Crow
4
Rheumatologic Laboratory Tests . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
Dalit Ashany, Anne R. Bass, and Keith B. Elkon
5
Immunogenetic Aspects of Rheumatic Diseases . . . . . . . . . . . . . . . . . . . 27
Allan Gibofsky
6
Bone, Connective Tissue, Joint and Vascular Biology,
And Pathology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
Linda A. Russell and Edward F. DiCarlo
7
Diagnostic Imaging Techniques . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40
Robert Schneider and Helene Pavlov
8
Arthrocentesis, Intra-articular Injection, and Synovial
Fluid Analysis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
Jessica R. Berman, Theodore R. Fields, and Richard Stern
9
Measuring Clinical Outcomes in Rheumatic Disease . . . . . . . . . . . . . . . . 55
Melanie J. Harrison and Lisa A. Mandl
10
Ethical and Legal Considerations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 62
C. Ronald MacKenzie and Allan Gibofsky
11
Patient Education . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 67
Laura Robbins and John P. Allegrante
12
Psychosocial Aspects of the Rheumatic Diseases . . . . . . . . . . . . . . . . . . 70
Sharon Danoff-Burg and Tracey A. Revenson
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Contents
II: THE STAT RHEUMATOLOGY AND ORTHOPEDIC
CONSULTATION: YOUR GUIDE TO ACUTE CARE
13
Acute Management of Musculoskeletal
And Autoimmune Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 80
Arthur M. F. Yee and Edward Su
III: CLINICAL PRESENTATIONS
14
Monarthritis/Polyarthritis: Differential Diagnosis . . . . . . . . . . . . . . . . . . .
Stephen Ray Mitchell and John F. Beary, III
97
15
Muscle Pain and Weakness . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 105
Lawrence J. Kagen
16
Rash and Arthritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109
Henry Lee, Rachelle Scott, and Animesh A. Sinha
17
Raynaud’s Phenomenon
Kyriakos A. Kirou
18
Autoimmune and Inflammatory Ophthalmic Diseases . . . . . . . . . . . . . . 130
Sergio Schwartzman, C. Michael Samson, and Scott S. Weissman
19
Neck Pain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 139
James C. Farmer, David A. Bomback, and Thomas P. Sculco
20
Low Back Pain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 144
H. Hallett Whitman, III, Daniel J. Clauw, and John F. Beary, III
21
Shoulder Pain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 152
Andrew D. Pearle and Russell F. Warren
22
Elbow Pain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159
Robert N. Hotchkiss
23
Hip Pain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164
Thomas P. Sculco and Paul Lombardi
24
Knee Pain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169
Norman A. Johanson and Paul Pellicci
25
Ankle and Foot Pain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 173
David S. Levine
26
Sports Injuries . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182
Riley J. Williams and Thomas L. Wickiewicz
27
The Female Athlete . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 197
Lisa R. Callahan, Jo A. Hannafin, and Monique Sheridan
28
Bursitis and Tendinitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 203
Paul Pellicci and Richard R. McCormack
. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 122
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ix
IV: DIAGNOSIS AND THERAPY
A. CONNECTIVE TISSUE DISORDERS
29
Rheumatoid Arthritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 206
Ioannis Tassiulas and Stephen A. Paget
30
Systemic Lupus Erythematosus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 221
Jane E. Salmon and Robert P. Kimberly
31
Antiphospholipid Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 238
Doruk Erkan and Lisa R. Sammaritano
32
Dermatomyositis, Polymyositis, and Inclusion
Body Myositis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 245
Petros Efthimiou and Lawrence J. Kagen
33
Sjögren’s Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 252
Stuart S. Kassan
34
Systemic Sclerosis and Related Syndromes . . . . . . . . . . . . . . . . . . . . . . 259
Robert F. Spiera
35
Polymyalgia Rheumatica and Giant Cell Arteritis . . . . . . . . . . . . . . . . . . 268
Richard Stern
36
Childhood Rheumatic Diseases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 275
Thomas J. A. Lehman
37
The Vasculitides . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 284
Yusuf Yazici and Michael D. Lockshin
38
Pregnancy and Connective Tissue Disorders . . . . . . . . . . . . . . . . . . . . . 297
Doruk Erkan and Lisa R. Sammaritano
B. SPONDYLOARTHROPATHIES
39
Ankylosing Spondylitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 306
Eric S. Schned
40
Arthritis Associated with Ulcerative Colitis
And Crohn’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 312
Kyriakos A. Kirou and Allan Gibofsky
41
Psoriatic Arthritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 318
Petros Efthimiou and Joseph A. Markenson
42
Reactive Arthritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 324
Robert D. Inman
C. CRYSTAL ARTHROPATHIES
43
Gout . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Theodore R. Fields
328
44
Pseudogout (Calcium Pyrophosphate Dihydrate
Crystal Arthropathy) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 336
Theodore R. Fields
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Contents
D. INFECTIOUS DISEASES INVOLVING
THE MUSCULOSKELETAL SYSTEM
45
Human Immunodeficiency Virus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 342
Edward Parrish
46
Infectious Arthritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349
Barry D. Brause and Juliet Aizer
47
Lyme Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359
Anne R. Bass and Steven K. Magid
48
Osteomyelitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 367
Juliet Aizer and Barry D. Brause
49
Rheumatic Fever . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 374
Allan Gibofsky and John B. Zabriskie
50
Whipple’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379
Stephen A. Paget and Kristina Belostocki
E. OSTEOARTHRITIS, METABOLIC BONE
AND ENDOCRINE DISORDERS
51
Osteoarthritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 381
John F. Beary, III and Michael E. Luggen
52
Osteonecrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 393
John H. Healey and Andrea Piccioli
53
Osteoporosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 397
Alexander Krawiecki, Joseph M. Lane, and Joseph L. Barker
54
Paget’s Disease of Bone . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 402
John H. Healey and Andrea Piccioli
55
Endocrine Arthropathies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 406
Michael D. Lockshin
F. OTHER RHEUMATIC DISEASES
56
Fibromyalgia and Chronic Pain . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 409
Daniel J. Clauw and John F. Beary, III
57
Paraneoplastic Musculoskeletal Syndromes
And Hypertrophic Osteoarthropathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . 414
Alan T. Kaell
58
Miscellaneous Diseases with Rheumatic Manifestations . . . . . . . . . . . . 427
Diana A. Yens, Chiara Baldini, and Stefano Bombardieri
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V: ORTHOPEDIC SURGERY AND REHABILITATION:
PRINCIPLES AND PRACTICE
59
Prosthetic Joint Replacement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 443
Mark Figgie and Harry E. Figgie, III
60
Perioperative Care of the Patient with Rheumatic Disease . . . . . . . . . . 447
C. Ronald MacKenzie and Nigel Sharrock
61
Physical Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 462
Sandy B. Ganz and Louis L. Harris
62
Occupational Therapy: Therapist’s Management
Of Rheumatologic Disorders of the Hand . . . . . . . . . . . . . . . . . . . . . . . . 482
Aviva Wolff
VI: COMPLEMENTARY AND ALTERNATIVE MEDICINE
489
Gina Kearney and C. Ronald MacKenzie
VII: FORMULARY
497
Arthur M. F. Yee and Jane E. Salmon
VIII: APPENDICES
A
American College of Rheumatology Criteria for Diagnosis
And Classification of Rheumatic Diseases . . . . . . . . . . . . . . . . . . . . . . . . 557
Allan Gibofsky
B
Neurologic Dermatomes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 562
Allan Gibofsky
C
Functional Outcome Instruments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 564
Lisa A. Mandl and Melanie J. Harrison
D
Myositis Functional Assessment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 569
Sandy B. Ganz and Louis L. Harris
E
Normal Laboratory Values . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 570
Allan Gibofsky and Stephen A. Paget
F
Basic Rheumatology Library and Information Web Sites . . . . . . . . . . . . 572
Theodore R. Fields
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 575
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ACKNOWLEDGMENTS
W
e gratefully acknowledge our many friends, colleagues, and alumni of the Hospital for Special Surgery who have made helpful suggestions and contributions to this volume
over the past quarter-century. We also gratefully appreciate the excellent assistance of
JoAnn Vega in the preparation of this volume.
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FOREWORDS
T
he composition and authorship of the Manual of Rheumatology and Outpatient
Orthopedic Disorders continues to reflect the fact that rheumatology and orthopedic surgery have a seamless interface in pursuit of education and patient care goals relative to
musculoskeletal disease. The inter-relationship of these two disciplines is a special and
unique feature of the Hospital for Special Surgery, where many of the authors have trained
or practiced.
The primary goal of this manual has been to serve the needs of students and physiciansin-training. Yet professionals of all ages (perhaps especially senior colleagues) find it useful
for reviewing miscellaneous things not successfully committed to memory. These include:
American College of Rheumatology Criteria for Diagnosis and Classification of Rheumatic
Disease, neurologic dermatomes, molecular targets of autoantibodies, normal laboratory
values, details in the formulary, etc. Between the fourth and fifth editions, there has been an
explosion of the rheumatologic formulary; new anti-inflammatory drugs and biologic disease modifying antirheumatic drugs, some based on new insights relative to the pathogenesis of rheumatoid arthritis.
Over the span of our five editions, several new chapters have been added, reflecting
our knowledge of recent advances: antiphospholipid syndrome, pregnancy, and connective
tissue diseases, rheumatic associations with HIV infection, diagnostic imaging, patient education, perioperative management, ethical and legal considerations, measuring functional
status, thinking like a rheumatologist, acute management of musculoskeletal and autoimmune disorders, and so on. In this edition, as in all previous ones, the emphasis remains the
discussion of practical aspects of management of musculoskeletal disorders.
Charles L. Christian, M.D.
Physician-in-Chief Emeritus
Hospital for Special Surgery
New York, New York
T
his remarkable manual celebrates the publication of its fifth edition this year. Innovations in diagnosis, therapeutics, and management strategies that have emerged in the
5 years since the last edition make this update timely. The succinct, authoritative, and didactic style of presenting the rationale and practical information in this publication will
doubtless continue to assist and guide physicians in their clinical practice.
The scope of the book is comprehensive, covering the full spectrum of therapy and
practice of rheumatology. The broad church of the specialty covered extends to regional
pain syndromes, fibromyalgia, diseases of bone, sports injuries, and the principles and
practice of surgery and rehabilitation. New chapters in the general sections cover not only
immunology, genomics, and proteomics but also ethical and legal issues and psychological
aspects of rheumatic disease.
Making knowledge-based therapeutic interventions that maximize benefit and minimize risk has increasingly become part of rheumatological practice since publication of the
last edition. The widespread use of anti-tumor necrosis factor (TNF) biologics added to
methotrexate therapy has profoundly altered the health outcomes for patients with moderate-to-severe rheumatoid arthritis whose disease is not controlled by more effective regimens employing standard drugs as monotherapy or in combination. The recent emphasis
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Forewords
on suppression of disease with judicious use of the available therapeutic armamentarium
has permitted control of signs, symptoms, and joint damage in most patients. Not only has
this permitted the maintenance of a good quality of life, but epidemiologic data also
demonstrate that it has reduced cardiovascular complications and prolonged the life expectancy of these patients.
The use of currently available biologics, and those in the development phase, has been
shown to be effective not only in rheumatoid arthritis but also in other inflammatory arthritides. The repertoire of targeted drugs that is now being developed shows promising results
for systemic rheumatic disease and will enlarge the pharmacopoeia. However, these advances
come at a price of unwanted side effects, such as increasing infection rates, in some patients.
As another example, the widespread use of cyclo-oxygenase-2 (COX-2) inhibitors that control pain with an improved gastric tolerance profile has apparently led to an increase in cardiovascular occlusive events. This has sparked a debate on the safety and indications of all
nonsteroidal anti-inflammatory drugs. The widespread public dissemination of this information has alarmed and confused patients at a time when the potential for the good of patients,
resulting from technical and scientific developments, has never been greater.
The rheumatological practice landscape has changed and will continue to change with
the advent of targeted biologic and chemical drugs and improvement in laboratory and imaging technologies. Managing this change will require a sharper focus and skill base in
rheumatological practice. The responsibility and role of thought leaders and educationalists in this process has therefore become increasingly important. It is worth recalling that
the origins of research with Coley’s toxin that led to the discovery of TNF almost a century
later originated at the Hospital for Special Surgery. The cooperation between surgeons and
physicians and their allied health associates remains a hallmark of this institution in their
quest for a better future for the health of patients. The editors and authors of this book,
coming from this center of excellence in research and practice, have much to offer in this regard to the community of rheumatological practitioners worldwide. Their book deserves a
place on the desk of trainees and established practitioners.
Professor Sir Ravinder Maini, B.A., M.B., BChir, Hon DSc, FRCP FRCP(E) FMed Sci
Emeritus Professor of Rheumatology
The Kennedy Institute of Rheumatology Division
Imperial College
London, W6 8RF
United Kingdom
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PREFACE
I
n the 5 years since the last edition of Manual of Rheumatology and Outpatient Orthopedic Disorders: Diagnosis and Therapy, the clinical and investigative tectonic plates of
rheumatology have shifted in a profound manner, all for the good of our patients. Through a
combination of explosions in our knowledge about the basic mechanisms of disease, advances in our appreciation of the clinical “personalities” of autoimmune and musculoskeletal
disorders, and the rapidity with which basic scientific wisdom is catapulted into therapeutic
advances at the bedside, our patients are living longer and better lives. Nowhere is this sea
change better seen than in the development of worldwide use of biologic drugs such as tumor
necrosis factor (TNF)-␣ blockers. The three commercially available anti-TNF drugs have significantly and safely improved the lives of hundreds of thousands of patients with rheumatoid
arthritis (RA), psoriatic arthritis, spondyloarthropathies, inflammatory bowel disease, and
other systemic inflammatory disorders. Despite these advances, we are still treating the
pathogenesis of diseases (such as RA) and not their etiology; although we can now block a
centrally important proinflammatory cytokine, we are still unable to identify and destroy the
etiologic agents that initiate the process of RA. However, just as systemic diseases such as
rheumatic fever, polio, syphilis, and tuberculosis fell to the development of antibiotics in the
last century, similar paradigm shifts will likely occur in the field of rheumatology.
We have moved from a “wait and see” attitude with regard to so many disorders to a
“get tough and take no prisoners” approach, stimulated by the fact that illnesses such as RA,
if not countered early and aggressively, are intrinsically joint damaging, life shortening, and
work limiting. Rheumatologists have adopted the therapeutic approaches employed by our
colleagues in the field of endocrinology and oncology. We now employ induction and maintenance treatment regimens in many diseases in order to optimally balance disease control with
drug-related side effects, and we do so to achieve a “no evidence of disease” (NED) status. Just
as endocrinologists aim for “tight control” of diabetes by decreasing glycosylated hemoglobin
levels so as to avoid the development of neuropathy, nephropathy, and retinopathy, rheumatologists also “aim” at decreasing signs and symptoms of RA inflammation using sensitive and
responsive clinical research tools such as the Health Assessment Questionnaire and the Disease
Activity Score. Given the amazing effectiveness of anti-TNF medications, we have even resurrected the term disease remission and aim for it day by day in our care of our patients.
We have learned a great deal about both the diseases we treat and the medications we
use to treat them. The former is possible through data obtained from randomized, controlled trials; observational studies; and use of and advances in clinical epidemiology and
health services research. The latter has arisen from drug trials, postmarketing surveillance,
and robust registries. In view of our newfound ability to really make a difference in the lives
of our patients, early arthritis centers have risen, first in Europe and more recently in the
United States, in an attempt to treat RA and other inflammatory disorders as close to their
onset as possible. Studies have recently shown that with self-limited, 1-year courses of antiTNF drugs, sustained remissions can be achieved.
Systemic inflammatory disorders such as RA and systemic lupus erythematosus (SLE)
not only affect joints and kidneys, respectively, but are also associated with significant collateral damage in the form of premature atherosclerosis and osteoporosis. Life span is
shortened in RA by approximately 10 years primarily due to ischemic heart disease. Therefore, treatment of these disorders demands a global approach, one that focuses not only on
the characteristic disease manifestations themselves but also on those tissues affected by the
“spill over” effect of systemic inflammation. We now treat RA and SLE like we would treat
diabetes, with low-dose aspirin, aggressive lipid lowering, and smoking avoidance.
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Preface
We have changed the look of our Manual to make it more user-friendly, added new
chapters in order to keep the manual up-to-date in this rapidly changing field, and always
kept in mind the need to deliver information in its most edible and rapidly digested form.
We have carefully integrated the new science into each line of the Manual in an attempt to
easily bring our increasing knowledge of the basic science to your patient. It is only with
early disease recognition and the institution of the proper therapeutic approach that we can
prolong our patient’s lives and keep our patients diseasefree and damagefree, functional,
and productive.
Stephen A. Paget, M.D.
Allan Gibofsky, M.D., J.D.
John F. Beary, III, M.D.
Thomas P. Sculco, M.D.
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CONTRIBUTING AUTHORS
Juliet Aizer, M.D., M.P.H.
Rheumatology Fellow
Weill Medical College of Cornell University
Hospital for Special Surgery
New York, New York
John P. Allegrante, Ph.D.
Professor of Health Education
Department of Health and Behavior
Studies, Teachers College
Department of Sociomedical Sciences,
Mailman School of Public Health
Columbia University
Senior Scientist
Hospital for Special Surgery
New York, New York
John F. Beary, III, M.D.
Clinical Professor of Medicine
University of Cincinnati
Attending Physician
Division of Rheumatology and
Immunology
Veterans Administration Medical Center
Cincinnati, Ohio
Kristina Belostocki, M.D.
Assistant Professor of Medicine
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Dalit Ashany, M.D.
Assistant Professor of Medicine
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Jessica R. Berman, M.D.
Assistant Professor of Medicine
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Joseph L. Barker, M.D.
Orthopedic Resident
Weill Medical College of Cornell University
Hospital for Special Surgery
New York, New York
David A. Bomback, M.D.
Orthopedic Surgeon
Connecticut Neck and Back Specialists
Danbury, Connecticut
Chiara Baldini, M.D.
Rheumatology Fellow
Department of Internal Medicine
University of Pisa
Pisa, Italy
Stefano Bombardieri, M.D.
Professor of Rheumatology
Chief, Rheumatic Diseases Unit
University of Pisa
Pisa, Italy
Anne R. Bass, M.D.
Assistant Professor of Clinical Medicine
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Barry D. Brause, M.D.
Professor of Clinical Medicine
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
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Contributing Authors
Lisa R. Callahan, M.D.
Assistant Professor of Clinical Medicine
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Keith B. Elkon, M.D.
Professor and Division Head
Division of Rheumatology
University of Washington
Seattle, Washington
Daniel J. Clauw, M.D.
Professor of Medicine and Chief
Division of Rheumatology
Department of Medicine
Assistant Dean of Clinical and Transitional
Research
University of Michigan Medical School
Ann Arbor, Michigan
Doruk Erkan, M.D.
Assistant Professor of Medicine
Weill Medical College of Cornell University
Associate Physician-Scientist
Barbara Volcker Center for Women
and Rheumatic Disease
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Charles N. Cornell, M.D.
Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
James C. Farmer, M.D.
Assistant Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery
New York, New York
Mary K. Crow, M.D.
Professor of Medicine
Weill Medical College of Cornell University
Director, Autoimmunity and Inflammation
Program
Senior Scientist
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Theodore R. Fields, M.D.
Associate Professor of Clinical Medicine
Weill Medical College of Cornell University
Director, Rheumatology Faculty
Practice Plan
Associate Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Sharon Danoff-Burg, Ph.D.
Associate Professor
Department of Psychology
State University of New York at Albany
Albany, New York
Harry E. Figgie, III, M.D.
Deceased
Edward F. DiCarlo, M.D.
Associate Professor of Clinical Pathology
Weill Medical College of Cornell University
Chief Surgical Pathologist
Director, Histology Laboratory
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Petros Efthimiou, M.D.
Assistant Professor of Medicine
University of Medicine and Dentistry of
New Jersey
Attending Physician
The University Hospital
Newark, New Jersey
Mark Figgie, M.D.
Associate Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Chief, Surgical Arthritis Service
Associate Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Sandy B. Ganz, P.T., D.Sc., G.C.S.
Faculty, Division of Geriatrics and
Gerontology
Weill Medical College of Cornell University
Director of Rehabilitation
Amsterdam Nursing Home
Associate in Research
Hospital for Special Surgery
New York, New York
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Contributing Authors
Allan Gibofsky, M.D., J.D.
Professor of Medicine and Public Health
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
Professor of Law
Fordham University
New York, New York
Jo A. Hannafin, M.D., Ph.D.
Assistant Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Director, Orthopedic Research
Director, Women’s Sports Medicine Center
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Louis L. Harris, M.D.
Senior Administrator and Director
Network Development and Planning
Burke Rehabilitation Hospital
White Plains, New York
Melanie J. Harrison, M.D., M.S.
Assistant Research Professor of Medicine
and Public Health
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
John H. Healey, M.D.
Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Chief, Orthopedic Service
Memorial Sloan-Kettering Cancer Center
New York, New York
xxi
Norman A. Johanson, M.D.
Professor and Chairman of Orthopedic
Surgery
Drexel University College of Medicine
Hahnemann University Hospital
Philadelphia, Pennsylvania
Alan T. Kaell, M.D.
Professor of Clinical Medicine
State University of New York at
Stony Brook
Stony Brook, New York
Chief, Division of Rheumatology
St. Charles Health System
Port Jefferson, New York
Lawrence J. Kagen, M.D.
Professor of Medicine
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Stuart S. Kassan, M.D.
Clinical Professor of Medicine
University of Colorado Health Sciences
Center
Colorado Arthritis Associates
Denver, Colorado
Gina Kearney, M.S.N., R.N., C.S.,
A.H.N.-B.C.
Holistic Nurse Practitioner
Integrative Care Center
Hospital for Special Surgery
New York, New York
Robert N. Hotchkiss, M.D.
Associate Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Director of Clinical Research
Attending Physician
Hospital for Special Surgery
New York, New York
Robert P. Kimberly, M.D.
Howard L. Holley Professor
of Medicine
University of Alabama at Birmingham
Director, University of Alabama Arthritis
and Musculoskeletal Center
Division of Clinical Immunology
and Rheumatology
University Hospital
Birmingham, Alabama
Robert D. Inman, M.D.
Professor of Medicine and Immunology
University of Toronto
Director, Arthritis Center of Excellence
Division of Rheumatology
Toronto Western Hospital
Ontario, Canada
Kyriakos A. Kirou, M.D.
Assistant Professor of Medicine
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
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Contributing Authors
Alexander Krawiecki, M.D.
Hospital for Special Surgery
New York, New York
Joseph M. Lane, M.D.
Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Henry Lee, M.D.
Dermatology Resident
Weill Medical College of Cornell University
New York Presbyterian Hospital
New York, New York
Thomas J. A. Lehman, M.D.
Professor of Clinical Pediatrics
Weill Medical College of Cornell University
Chief, Division of Pediatric Rheumatology
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
David S. Levine, M.D.
Assistant Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Michael D. Lockshin, M.D.
Professor of Medicine and Obstetrics
Weill Medical College of Cornell University
Director, Barbara Volcker Center
for Women and Rheumatic Disease
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
C. Ronald MacKenzie, M.D.
Associate Professor of Clinical Medicine
Weill Medical College of Cornell University
Associate Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Steven K. Magid, M.D.
Associate Professor of Clinical Medicine
Weill Medical College of Cornell University
Associate Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Lisa A. Mandl, M.D., M.P.H.
Assistant Professor of Medicine
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Joseph A. Markenson, M.D.
Professor of Medicine
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Richard R. McCormack, M.D.
Orthopedic Surgeon Emeritus
Hospital for Special Surgery
Weill Medical College of Cornell University
New York, New York
Stephen Ray Mitchell, M.D.
Director, Residency Program, Department
of Medicine
Georgetown University
Washington, District of Columbia
Paul Lombardi, M.D.
Senior Clinical Associate in Orthopedic
Surgery
Weill Medical College of Cornell University
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Stephen A. Paget, M.D.
Joseph P. Routh Professor of Medicine
Weill Medical College of Cornell University
Physician-in-Chief, Division of
Rheumatology
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Michael E. Luggen, M.D.
Professor of Clinical Medicine
University of Cincinnati Medical Center
Rheumatology Consultants
Department of Internal Medicine
University Hospital
Cincinnati, Ohio
Edward Parrish, M.D.
Associate Professor of Medicine
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
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Contributing Authors
xxiii
Helene Pavlov, M.D.
Professor of Radiology
Weill Medical College of Cornell University
Chief, Department of Radiology and
Imaging
Hospital for Special Surgery
New York, New York
Lisa R. Sammaritano, M.D.
Associate Professor of Medicine
Weill Medical College of Cornell University
Associate Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Andrew D. Pearle, M.D.
Instructor in Orthopedic Surgery
Weill Medical College of Cornell University
Assistant Attending Orthopedic Surgeon
Hospital for Special Surgery
New York, New York
C. Michael Samson, M.D.
Co-Director, Uveitis Service
New York Eye and Ear Infirmary
New York, New York
Paul Pellicci, M.D.
Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Chief, Hip Service
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Andrea Piccioli, M.D.
Orthopedic Surgeon
Centro Traumatologico e Ortopedico
Rome, Italy
Tracey A. Revenson, Ph.D.
Professor of Psychology
The Graduate Center of the City
University of New York
New York, New York
Laura Robbins, D.S.W.
Associate Professor
Graduate School of Medical Sciences
Clinical Epidemiology and Health Sciences
Research
Weill Medical College at Cornell University
Vice President, Education and Academic
Affairs
Associate Scientist
Hospital for Special Surgery
New York, New York
Linda A. Russell, M.D.
Assistant Professor of Medicine
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Jane E. Salmon, M.D.
Professor of Medicine
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Eric S. Schned, M.D.
Medical Director
Park Nicollete Clinic
Minneapolis, Minnesota
Robert Schneider, M.D.
Associate Professor of Radiology
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Sergio Schwartzman, M.D.
Associate Professor of Medicine
Weill Medical College of Cornell University
Associate Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Rachelle Scott, M.D.
Associate Professor of Clinical
Dermatology
Weill Medical College of Cornell University
Assistant Attending Physician
New York Presbyterian Hospital
New York, New York
Thomas P. Sculco, M.D.
Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Surgeon-in-Chief, Chairman
Department of Orthopedics
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Nigel Sharrock, M.B., Ch.B.
Clinical Professor of Anesthesiology
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
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Contributing Authors
Monique Sheridan
Research Coordinator
Women’s Sports Medicine Center
Hospital for Special Surgery
New York, New York
Animesh A. Sinha, M.D., Ph.D.
Assistant Professor of Dermatology
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Robert F. Spiera, M.D.
Associate Professor of Medicine
Weill Medical College of Cornell University
Director, Vasculitis and Scleroderma
Programs
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Richard Stern, M.D.
Clinical Associate Professor
Weill Medical College of Cornell University
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Edward Su, M.D.
Clinical Instructor of Orthopedic Surgery
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Ioannis Tassiulas, M.D.
Assistant Professor of Medicine
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Russell F. Warren, M.D.
Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Surgeon-in-Chief Emeritus
Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Scott S. Weissman, M.D.
Adjunct Clinical Scientist
Columbia University College of Physicians
and Surgeons
Attending Physician
Manhattan Eye, Ear, and Throat
Hospital
Attending Physician and Director
Uveitis Service, New York Eye and Ear
Infirmary
New York, New York
H. Hallett Whitman, III, M.D.
Assistant Professor of Clinical Medicine
Clinical and Research Associate
Cardiovascular Hypertension Center
Weill Medical College of Cornell University
Chief of Rheumatology
Summit Medical Group
Summit, New Jersey
Physician to the Outpatient Department
Hospital for Special Surgery
New York, New York
Thomas L. Wickiewicz, M.D.
Associate Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Chief, Sports Medicine and
Shoulder Service
Associate Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Riley J. Williams, M.D.
Associate Professor of Orthopedic Surgery
Weill Medical College of Cornell University
Sports Medicine and Shoulder Service
Associate Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Aviva Wolff, O.T.R., C.H.T.
Senior Hand Specialist
Department of Rehabilitation
Hospital for Special Surgery
New York, New York
Yusuf Yazici, M.D.
Assistant Professor of Clinical Medicine
New York University
Attending Rheumatologist
Hospital for Joint Diseases
New York, New York
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Contributing Authors
Arthur M. F. Yee, M.D., Ph.D.
Assistant Professor of Medicine
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery-New York
Presbyterian Hospital
New York, New York
Diana A. Yens, M.D.
Assistant Professor of Clinical Medicine
Weill Medical College of Cornell University
Assistant Attending Physician
Hospital for Special Surgery
New York, New York
xxv
John B. Zabriskie, M.D.
Associate Professor Emeritus
Clinical Microbiology and Immunology
Rockefeller University
Senior Physician
Rockefeller University Hospital
New York, New York