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(BQ) Part 1 book Hospital for special surgery manual of rheumatology and outpatient orthopedic disorders - Diagnosis and therapy presents the following contents: Musculoskeletal database, the stat rheumatology and orthopedic consultation: your guide to acute care, clinical presentations.

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HOSPITAL FOR SPECIAL SURGERY MANUAL OF RHEUMATOLOGY AND OUTPATIENT ORTHOPEDIC

DISORDERS: DIAGNOSIS AND THERAPY

Fifth Edition

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Stephen A Paget, M D

Joseph P Routh Professor of Medicine

Weill Medical College of Cornell University

Professor of Medicine and Public Health

Weill Medical College of Cornell 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, 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

HOSPITAL FOR SPECIAL SURGERY MANUAL OF RHEUMATOLOGY AND OUTPATIENT ORTHOPEDIC

DISORDERS: DIAGNOSIS AND THERAPY

Fifth Edition

Associate Editor

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

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

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 omis- sions 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 responsi- bility 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 Administra- tion (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 The publisher has made every effort to trace copyright holders for borrowed material If they have inadvertently overlooked any, they will be pleased to make the necessary arrangements at the first opportunity.

To purchase additional copies of this book, call our customer service department at (800) 638-3030 or fax orders to (301) 223-2320 International customers should call (301) 223-2300 Lippincott Williams & Wilkins customer service representatives are available from 8:30 a.m to 6:30 p.m., EST, Monday through Friday, for telephone access Visit Lippincott Williams & Wilkins on the Internet: http://www.lww.com.

10 9 8 7 6 5 4 3 2 1

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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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Acknowledgments xiii

Forewords xv

Preface xvii

Contributing Authors xix

I: MUSCULOSKELETAL DATABASE 1 Musculoskeletal History and Physical Examination 1

Stephen A Paget, Charles N Cornell, and John F Beary, III 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

CONTENTS

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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 97

Stephen Ray Mitchell and John F Beary, III

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 122

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

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

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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

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

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D INFECTIOUS DISEASES INVOLVING THE MUSCULOSKELETAL SYSTEM

45 Human Immunodeficiency Virus 342

Stephen A Paget and Kristina Belostocki

E OSTEOARTHRITIS, METABOLIC BONE AND ENDOCRINE DISORDERS

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

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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We gratefully acknowledge our many friends, colleagues, and alumni of the tal for Special Surgery who have made helpful suggestions and contributions to this volumeover the past quarter-century We also gratefully appreciate the excellent assistance ofJoAnn Vega in the preparation of this volume.

Hospi-ACKNOWLEDGMENTS

xiii

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The composition and authorship of the Manual of Rheumatology and Outpatient Orthopedic Disorders continues to reflect the fact that rheumatology and orthopedic sur-

gery have a seamless interface in pursuit of education and patient care goals relative tomusculoskeletal disease The inter-relationship of these two disciplines is a special andunique 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 in-training Yet professionals of all ages (perhaps especially senior colleagues) find it usefulfor reviewing miscellaneous things not successfully committed to memory These include:American College of Rheumatology Criteria for Diagnosis and Classification of RheumaticDisease, neurologic dermatomes, molecular targets of autoantibodies, normal laboratoryvalues, details in the formulary, etc Between the fourth and fifth editions, there has been anexplosion of the rheumatologic formulary; new anti-inflammatory drugs and biologic dis-ease modifying antirheumatic drugs, some based on new insights relative to the pathogene-sis of rheumatoid arthritis

physicians-Over the span of our five editions, several new chapters have been added, reflectingour knowledge of recent advances: antiphospholipid syndrome, pregnancy, and connectivetissue diseases, rheumatic associations with HIV infection, diagnostic imaging, patient edu-cation, perioperative management, ethical and legal considerations, measuring functionalstatus, thinking like a rheumatologist, acute management of musculoskeletal and autoim-mune disorders, and so on In this edition, as in all previous ones, the emphasis remains thediscussion 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

This remarkable manual celebrates the publication of its fifth edition this year vations in diagnosis, therapeutics, and management strategies that have emerged in the

Inno-5 years since the last edition make this update timely The succinct, authoritative, and dactic style of presenting the rationale and practical information in this publication willdoubtless continue to assist and guide physicians in their clinical practice

di-The scope of the book is comprehensive, covering the full spectrum of therapy andpractice of rheumatology The broad church of the specialty covered extends to regionalpain syndromes, fibromyalgia, diseases of bone, sports injuries, and the principles andpractice of surgery and rehabilitation New chapters in the general sections cover not onlyimmunology, genomics, and proteomics but also ethical and legal issues and psychologicalaspects of rheumatic disease

Making knowledge-based therapeutic interventions that maximize benefit and mize risk has increasingly become part of rheumatological practice since publication of thelast edition The widespread use of anti-tumor necrosis factor (TNF) biologics added tomethotrexate therapy has profoundly altered the health outcomes for patients with moder-ate-to-severe rheumatoid arthritis whose disease is not controlled by more effective regi-mens employing standard drugs as monotherapy or in combination The recent emphasis

mini-FOREWORDS

xv

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on suppression of disease with judicious use of the available therapeutic armamentariumhas permitted control of signs, symptoms, and joint damage in most patients Not only hasthis permitted the maintenance of a good quality of life, but epidemiologic data alsodemonstrate that it has reduced cardiovascular complications and prolonged the life ex-pectancy of these patients.

The use of currently available biologics, and those in the development phase, has beenshown to be effective not only in rheumatoid arthritis but also in other inflammatory arthri-tides The repertoire of targeted drugs that is now being developed shows promising resultsfor systemic rheumatic disease and will enlarge the pharmacopoeia However, these advancescome 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 trol pain with an improved gastric tolerance profile has apparently led to an increase in car-diovascular occlusive events This has sparked a debate on the safety and indications of allnonsteroidal anti-inflammatory drugs The widespread public dissemination of this informa-tion 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

con-The rheumatological practice landscape has changed and will continue to change withthe advent of targeted biologic and chemical drugs and improvement in laboratory and im-aging technologies Managing this change will require a sharper focus and skill base inrheumatological practice The responsibility and role of thought leaders and educational-ists in this process has therefore become increasingly important It is worth recalling thatthe origins of research with Coley’s toxin that led to the discovery of TNF almost a centurylater originated at the Hospital for Special Surgery The cooperation between surgeons andphysicians and their allied health associates remains a hallmark of this institution in theirquest 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 re-gard to the community of rheumatological practitioners worldwide Their book deserves aplace 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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In the 5 years since the last edition of Manual of Rheumatology and Outpatient dic Disorders: Diagnosis and Therapy, the clinical and investigative tectonic plates of

Orthope-rheumatology have shifted in a profound manner, all for the good of our patients Through acombination of explosions in our knowledge about the basic mechanisms of disease, ad-vances in our appreciation of the clinical “personalities” of autoimmune and musculoskeletaldisorders, and the rapidity with which basic scientific wisdom is catapulted into therapeuticadvances at the bedside, our patients are living longer and better lives Nowhere is this seachange better seen than in the development of worldwide use of biologic drugs such as tumornecrosis factor (TNF)- blockers The three commercially available anti-TNF drugs have sig-nificantly and safely improved the lives of hundreds of thousands of patients with rheumatoidarthritis (RA), psoriatic arthritis, spondyloarthropathies, inflammatory bowel disease, andother systemic inflammatory disorders Despite these advances, we are still treating thepathogenesis of diseases (such as RA) and not their etiology; although we can now block acentrally important proinflammatory cytokine, we are still unable to identify and destroy theetiologic agents that initiate the process of RA However, just as systemic diseases such asrheumatic fever, polio, syphilis, and tuberculosis fell to the development of antibiotics in thelast 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, andwork limiting Rheumatologists have adopted the therapeutic approaches employed by ourcolleagues in the field of endocrinology and oncology We now employ induction and mainte-nance treatment regimens in many diseases in order to optimally balance disease control withdrug-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 hemoglobinlevels so as to avoid the development of neuropathy, nephropathy, and retinopathy, rheuma-tologists also “aim” at decreasing signs and symptoms of RA inflammation using sensitive andresponsive clinical research tools such as the Health Assessment Questionnaire and the DiseaseActivity Score Given the amazing effectiveness of anti-TNF medications, we have even resur-rected 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 weuse to treat them The former is possible through data obtained from randomized, con-trolled trials; observational studies; and use of and advances in clinical epidemiology andhealth 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 theUnited States, in an attempt to treat RA and other inflammatory disorders as close to theironset as possible Studies have recently shown that with self-limited, 1-year courses of anti-TNF 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 col-lateral damage in the form of premature atherosclerosis and osteoporosis Life span isshortened in RA by approximately 10 years primarily due to ischemic heart disease There-fore, treatment of these disorders demands a global approach, one that focuses not only onthe 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 treatdiabetes, with low-dose aspirin, aggressive lipid lowering, and smoking avoidance

PREFACE

xvii

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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 alwayskept 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 withearly disease recognition and the institution of the proper therapeutic approach that we canprolong 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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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

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

Joseph L Barker, M.D.

Orthopedic Resident

Weill Medical College of Cornell University

Hospital for Special Surgery

New York, New York

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

John F Beary, III, M.D.

Clinical Professor of MedicineUniversity of CincinnatiAttending PhysicianDivision of Rheumatology and Immunology

Veterans Administration Medical CenterCincinnati, Ohio

Kristina Belostocki, M.D.

Assistant Professor of MedicineWeill Medical College of Cornell UniversityAssistant Attending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Jessica R Berman, M.D.

Assistant Professor of MedicineWeill Medical College of Cornell UniversityAssistant Attending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

David A Bomback, M.D.

Orthopedic SurgeonConnecticut Neck and Back SpecialistsDanbury, Connecticut

Stefano Bombardieri, M.D.

Professor of RheumatologyChief, Rheumatic Diseases UnitUniversity of Pisa

Pisa, Italy

Barry D Brause, M.D.

Professor of Clinical MedicineWeill Medical College of Cornell UniversityAttending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

CONTRIBUTING AUTHORS

xix

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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

University of Michigan Medical School

Ann Arbor, Michigan

Charles N Cornell, M.D.

Professor of Orthopedic Surgery

Weill Medical College of Cornell University

Weill Medical College of Cornell University

Director, Autoimmunity and Inflammation

State University of New York at Albany

Albany, New York

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

Keith B Elkon, M.D.

Professor and Division HeadDivision of RheumatologyUniversity of WashingtonSeattle, Washington

Doruk Erkan, M.D.

Assistant Professor of MedicineWeill Medical College of Cornell UniversityAssociate Physician-Scientist

Barbara Volcker Center for Women and Rheumatic Disease

Assistant Attending PhysicianHospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

James C Farmer, M.D.

Assistant Professor of Orthopedic SurgeryWeill Medical College of Cornell UniversityAssistant Attending Physician

Hospital for Special SurgeryNew York, New York

Theodore R Fields, M.D.

Associate Professor of Clinical MedicineWeill Medical College of Cornell UniversityDirector, Rheumatology Faculty

Practice PlanAssociate Attending Physician Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Harry E Figgie, III, M.D.

Deceased

Mark Figgie, M.D.

Associate Professor of Orthopedic SurgeryWeill Medical College of Cornell UniversityChief, Surgical Arthritis Service

Associate Attending Physician Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

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Allan Gibofsky, M.D., J.D.

Professor of Medicine and Public Health

Weill Medical College of Cornell University

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

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

Alan T Kaell, M.D.

Professor of Clinical Medicine State University of New York at Stony Brook

Stony Brook, New YorkChief, Division of Rheumatology

St Charles Health SystemPort Jefferson, New York

Lawrence J Kagen, M.D.

Professor of MedicineWeill Medical College of Cornell UniversityAttending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Stuart S Kassan, M.D.

Clinical Professor of MedicineUniversity of Colorado Health SciencesCenter

Colorado Arthritis AssociatesDenver, Colorado

Gina Kearney, M.S.N., R.N., C.S., A.H.N.-B.C.

Holistic Nurse Practitioner Integrative Care Center Hospital for Special SurgeryNew York, New York

Robert P Kimberly, M.D.

Howard L Holley Professor

of MedicineUniversity of Alabama at BirminghamDirector, University of Alabama Arthritisand Musculoskeletal Center

Division of Clinical Immunology and Rheumatology

University HospitalBirmingham, Alabama

Kyriakos A Kirou, M.D.

Assistant Professor of MedicineWeill Medical College of Cornell UniversityAssistant Attending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

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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

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

Weill Medical College of Cornell University

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

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Steven K Magid, M.D.

Associate Professor of Clinical MedicineWeill Medical College of Cornell UniversityAssociate Attending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Lisa A Mandl, M.D., M.P.H.

Assistant Professor of Medicine Weill Medical College of Cornell UniversityAssistant Attending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Joseph A Markenson, M.D.

Professor of MedicineWeill Medical College of Cornell UniversityAttending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Richard R McCormack, M.D.

Orthopedic Surgeon EmeritusHospital for Special SurgeryWeill Medical College of Cornell UniversityNew York, New York

Stephen Ray Mitchell, M.D.

Director, Residency Program, Department

of MedicineGeorgetown UniversityWashington, District of Columbia

Stephen A Paget, M.D.

Joseph P Routh Professor of MedicineWeill Medical College of Cornell UniversityPhysician-in-Chief, Division of

RheumatologyHospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Edward Parrish, M.D.

Associate Professor of MedicineWeill Medical College of Cornell UniversityAttending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

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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

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

Paul Pellicci, M.D.

Professor of Orthopedic Surgery

Weill Medical College of Cornell University

Chief, Hip Service

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

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

C Michael Samson, M.D.

Co-Director, Uveitis ServiceNew York Eye and Ear InfirmaryNew York, New York

Eric S Schned, M.D.

Medical DirectorPark Nicollete ClinicMinneapolis, Minnesota

Robert Schneider, M.D.

Associate Professor of RadiologyWeill Medical College of Cornell UniversityAttending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Sergio Schwartzman, M.D.

Associate Professor of MedicineWeill Medical College of Cornell UniversityAssociate Attending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Department of OrthopedicsHospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Nigel Sharrock, M.B., Ch.B.

Clinical Professor of AnesthesiologyWeill Medical College of Cornell UniversityAttending Physician

Hospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

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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

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

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

Attending PhysicianManhattan Eye, Ear, and Throat Hospital

Attending Physician and DirectorUveitis Service, New York Eye and EarInfirmary

New York, New York

H Hallett Whitman, III, M.D.

Assistant Professor of Clinical MedicineClinical and Research Associate Cardiovascular Hypertension CenterWeill Medical College of Cornell UniversityChief of Rheumatology

Summit Medical GroupSummit, New JerseyPhysician to the Outpatient DepartmentHospital for Special Surgery

New York, New York

Thomas L Wickiewicz, M.D.

Associate Professor of Orthopedic Surgery Weill Medical College of Cornell UniversityChief, Sports Medicine and

Shoulder ServiceAssociate Attending PhysicianHospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Riley J Williams, M.D.

Associate Professor of Orthopedic Surgery Weill Medical College of Cornell UniversitySports Medicine and Shoulder ServiceAssociate Attending PhysicianHospital for Special Surgery-New YorkPresbyterian Hospital

New York, New York

Aviva Wolff, O.T.R., C.H.T.

Senior Hand SpecialistDepartment of RehabilitationHospital for Special SurgeryNew York, New York

Yusuf Yazici, M.D.

Assistant Professor of Clinical MedicineNew York University

Attending RheumatologistHospital for Joint DiseasesNew York, New York

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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

John B Zabriskie, M.D.

Associate Professor EmeritusClinical Microbiology and ImmunologyRockefeller University

Senior PhysicianRockefeller University HospitalNew York, New York

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Musculoskeletal Database I

MUSCULOSKELETAL HISTORY AND PHYSICAL EXAMINATION

Stephen A Paget, Charles N Cornell,

and John F Beary, III

1

The musculoskeletal or locomotor system,like other body systems, can be definedanatomically and assessed functionally Lowerextremities support the weight of the bodyand allow ambulation They require proper alignment and stability Upperextremitiesreach, grasp, and hold, thereby allowing self-care, feeding, and work They require mobil-ity and strength Diseases and disorders of the musculoskeletal system disturb anatomy andinterfere with function

MUSCULOSKELETAL HISTORY

A careful history is the most important and powerful of the information-gathering dures used to define a patient’s problems.In most musculoskeletal disorders, 80% of thediagnosis comes from this part of the clinical evaluation The history of patients with rheu-matic complaints should include the following: (a) reason for consultation and duration ofcomplaints; (b) present medical care and medications; (c) chronologic review of present ill-ness with emphasis on the locomotor system, consequences of time and disease, and pres-ent functional assessment; (d) past history—medical, surgical, and of trauma; (e) socialhistory, emotional and work impact of the disorder, and environmental and work site fac-tors; (f) family history, especially as it relates to the musculoskeletal system; and (g) review

proce-of systems These queries cover the spectrum proce-of rheumatic complaints: pain, stiffness, jointswelling, lack of mobility, physical handicap, and fear of future disability and handicap.The interviewer should be flexible and tactful and should avoid interrupting the patientwith too many questions and merely guiding the flow of information The objective of theinterview is to define the patient’s complaints and to identify patterns of disease and areas

of musculoskeletal involvement that can be further scrutinized on physical examination

I CHIEF COMPLAINT.Note duration

II PRIMARY PHYSICIAN.Note name, telephone number, fax number, and e-mail dress to assist in locating important data A discussion with that physician may add

ad-1

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greatly to your assessment, may avoid the need to repeat expensive tests already formed, and will better define the course and tempo of the disorder.

per-III HISTORY OF RHEUMATIC DISEASES

A.Determine the mode of onset, inciting events, duration, and pattern and sion of the musculoskeletal complaints

progres-1 Acute onsetis consistent with infectious, crystal-induced, or traumatic origin Itcan also occur in the setting of a connective tissue disorder Chronic complaintsare seen with rheumatoid arthritis (RA), spondyloarthropathies, and osteoarthri-tis, or the chronic sequelae of traumatic or degenerative back problems

2. The pattern of joint involvementis very important in defining the type of jointdisorder Symmetric polyarthritis of the small joints of the hands and feet is char-acteristic of RA, whereas asymmetric involvement of the large joints of the lowerextremities is most typical of spondyloarthropathies A migratory pattern ofjoint inflammation is seen in rheumatic fever and disseminated gonococcemia Amonarticular arthritis is consistent with osteoarthritis, infectious arthritis, crys-tal-induced synovitis, or one of the spondyloarthropathies (e.g., psoriatic arthri-tis, reactive arthritis) An intermittent joint inflammation of the knee withremissions and exacerbations is typical of the tertiary phase of Lyme disease

3 Location, pain characteristics, and associated findingsmay all be portant keys to the diagnosis First, metatarsophalangeal joint inflammation

im-of an acute and severe type is quite characteristic im-of gouty arthritis Suddenonset of low back pain in the setting of lifting or bending with associated painradiating down the lateral leg is a common presentation for a disk herniationwith sciatica

Pain in the superolateral shoulder or upper arm occurring in the setting

of playing tennis or painting a ceiling is typical of supraspinatus tendinitis orimpingement syndrome

B.Record the severity of disease,as revealed by a chronologic review of thefollowing:

1. Ability to workduring months or years

2. Need for hospitalization or home confinement

3. When applicable, ability to do household chores

4. Activities of daily living and personal care.

5. Landmarks or significant functional change,such as retirement from work,need for household help, assistance for personal care, and the use of a cane,crutches, or a wheelchair

C.Assess current functional ability.This can be done in a question-and-answer mat and quantified with the use of functional instruments such as the Health Assess-ment Questionnaire (HAQ) or the Arthritis Impact Measurement Scale (AIMS2), orfunctional ability can be measured with the use of a visual analog scale (0 represent-ing no impact on function and 10 being the worst possible limitation in function)

for-1. At home: independence or reliance on help from family members and others

2. At work: transportation and job requirements and limitations Have the tient collect an hour-by-hour log of work activities, with an attempt to defineactions that may cause or exacerbate musculoskeletal problems

pa-3. At recreational and social activities: limitations and extent to which patient ishouse-bound

4. Review of a typical 24-hour period, with focus on abilities to transfer, late, and perform personal care

ambu-D.Obtain an overview of managementfor rheumatic disease

1 Medicationsused in the past, with emphasis on dosages, duration of ments, efficacy response, and possible adverse reactions Record the presentdrug regimen and how well the patient complies with it, and also the pa-tient’s understanding of the reasons for and potential complications of themedication

treat-2. Instruction in and compliance with a therapeutic exerciseprogram

3 Surgical procedureson joints, including benefits and liabilities Record thename of the surgeon, date of the surgery, and the hospital Operative pathol-ogy reports may be helpful

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E.Determine the patient’s understanding of the disease, therapeutic goals, andexpectations.

F.Record psychosocialconsequences of disease

1 Anxiety, depression, insomnia. Obtain information about psychological/psychiatric intervention and a listing of psychotropic medications

2 Economic impactof handicap and present means of support

3. Family inter-relationships

4. Use of community resources

IV PAST HISTORY Follow traditional lines of questioning, with attention to trauma andjoint operations Also question the patient about those specific medical disorders thatcould have a significant impact on, or association with, the joint disorder

Specific associations include psoriasis with psoriatic arthritis; ulcerative colitis

or Crohn’s disease with inflammatory disease of the spine or peripheral or sacroiliacjoints; diabetes with neuropathic or septic joints, or osteomyelitis; hemochromatosis withsevere osteoarthritis; endocrinopathies such as hypothyroidism with carpal tunnel syn-drome or myopathy, hyperparathyroidism with pseudogout, and acromegaly with se-vere osteoarthritis A complete medication list of the patient is essential, as well as aninquiry into prior medications In this context, think about agents associated withdrug-induced lupus, Raynaud’s phenomenon associated with the use of

eosinophilia-myalgia syndrome associated with L-tryptophan, or myositis associatedwith the use of “statin” drugs for hypercholesterolemia

V SOCIAL HISTORY The physician must consider the following associations betweenthe social history and types of musculoskeletal disorders:

A Work activities,including the possibility of joint or back trauma, exposure totoxins, or overuse syndromes Specific examples include low-back syndromes, ex-posure to vinyl chloride leading to scleroderma-type skin changes, and carpal tun-nel syndrome resulting from typing at a computer terminal

B Sexual history,including sexual preference, sexual promiscuity, and the most cent sexual experience Musculoskeletal disorders related to acquired immunodefi-ciency syndrome (AIDS) and venereal disorders such as gonococcal disease should

re-be considered

C Living site and conditions,including overcrowding (e.g., rheumatic fever), living

in an area where Lyme disease is endemic, or a recent or distant history of tick bite

D Emotional or physical stress,which could have an impact on the development

or exacerbation of musculoskeletal disorders

E.The presence of medical problems within the family,including infectious ders in children (e.g., fifth disease caused by parvovirus B19, rubella) and adults(e.g., hepatitis B and C, Lyme disease, tuberculosis)

disor-F Recent travel,with specific emphasis on the development of dysentery caused by

Salmonella or Shigella (e.g., reactive arthritis), or travel to an area where Lyme

dis-ease is endemic

VI FAMILY HISTORY Inquiry about arthritis and rheumatic disease in parents and lings may elicit vague and unreliable statements, but they are nonetheless important.The presence of severely handicapped relatives with RA or other severe rheumatic dis-ease might result in a significant psychological impact on the patient and should bebrought out in the interview Such information may also be important in relation tothe genetic background of arthritis in the family The physician should inquire aboutthe following musculoskeletal disorders, which clearly have a tendency to run in fam-ilies: gout and uric acid kidney stones; RA and other connective tissue disorders;ankylosing spondylitis and other spondyloarthropathies; osteoarthritis, especiallynodal disease in the fingers; and classic, heritable connective tissue disorders, such asMarfan’s syndrome

sib-VII REVIEW OF SYSTEMS Emphasize diseases and systemic disorders related to matic complaints and diseases of connective tissue Especially inquire about eye dis-ease (iritis, uveitis, conjunctivitis, dryness), mouth disorders (dryness, mouth sores,tightness), gastrointestinal problems (problems with swallowing, reflux symptoms,abdominal pain, diarrhea with or without blood, constipation), genitourinary com-plaints (including dysuria, urethral discharge, hematuria), and skin disorders (rashwith or without sun sensitivity, nodules, ulcers, Raynaud’s phenomenon, ischemic

rheu--blockers,

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changes) The presence of constitutional symptoms is also important, including plaints of weight loss, fatigue, fever, chills, night sweats, and weakness.

com-PHYSICAL EXAMINATION WITH EMPHASIS

ON RHEUMATIC DISEASES

Five aspects of the physical examination that should be recorded are (a) gait, (b) spine, (c) muscles, (d) upper extremities, and (e) lower extremities.The patient should be prop-erly attired in a short gown, open at the back to allow examination of the entire spine Ex-amination should be methodic and start with observation of the patient’s attitude, comfortlevels, ease of undressing, method of rising from a chair and sitting down, and apparentstate of nutrition The patient is examined while standing, sitting, and supine The exam-iner should rely mainly on inspection When using palpation and manipulation, the exam-iner should be gentle and forewarn the patient of potentially painful maneuvers

I GAIT Describe the gait, and note a limp or use of a cane or crutches The normal gait

is divided into the phases of stance (60%) and swing (40%) Clinically important gaitsinclude the following:

A Antalgic gait,characterized by a short stance phase on the painful side

B Short-leg gait,with signs of pelvic obliquity and flexion deformity of the site knee

oppo-C Coxalgic gait,an antalgic gait with a lurch toward the painful hip

D Metatarsalgic gait,in which the patient tries to avoid weight bearing on theforefoot

II STANDING POSITION

A.Examining front and back, note posture (cervical lordosis, scoliosis, dorsalkyphosis, lumbar lordosis) Check if the pelvis is level by putting one finger oneach iliac crest and noting asymmetry Pelvic obliquity suggests unequal leglengths Note also if a tilt of the trunk to one side is present

B.Examine alignment of the lower extremitiesfor flexion deformity of the knees,genu varum (bowlegs), or genu valgum (knock-knees)

C.Observe position of the ankles and feet(varus or valgus heels, flat feet, sion or eversion of feet)

inver-D.Check back motionon forward bending (with rounding of the normal columbar spine), lateral flexion to each side, and hyperextension The extent ofoverall spinal flexioncan be assessed with a metal tape measure One end of thetape is placed at the C7 spinous process, and the other end is placed at S1 with thepatient standing erect The patient is then asked to bend forward, flexing the spinemaximally The measuring tape will reveal an increase of 10 cm with normal spineflexion; 7.5 cm of the total increase results from lumbar spine (measured fromspinous process T12–S1) mobility in normal adults The lumbar spine motion can

thora-be assessed by the Schothora-ber’s test in an erect patient, wherein the examiner makes

an ink mark at the lumbosacral junction and at a point 10 cm above The patient

is then instructed to maximally anterior flex, and the distance between the marks

is recorded Less than 5 cm of distraction is abnormal These measurements areuseful for the serial evaluation of patients with spondyloarthropathy

III SEATED POSITION

A.Observe head and neck motionin all planes (Fig 1-1)

B.Examine thoracolumbar spine motionwith the pelvis fixed Observe roundingand straightening of back, lateral flexion to each side, and rotation to right and left

C.Check temporomandibular joints. Palpate, examine lower jaw motion, andmeasure the aperture between upper and lower teeth with the mouth fully open

D.Proceed with the rest of the routine examination of the head and neck; describeeye, ear, nose, and throat findings

E Upper extremities

1 Shoulders

a. Note normal contour or “squaring” caused by deltoid atrophy Palpateanteriorly for soft-tissue swelling and laterally under the acromion for ten-don insertion tenderness

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b Function of the entire shoulder complex is evaluated by elevating botharms from 0 degrees along the sides of body to 180 degrees straight abovethe head Quantify internal rotation by having the patient reach with thedorsum of the hands, the highest possible level of the back (Fig 1-2); quan-tify external rotation by noting the position behind the neck or head thatthe hands can reach.

c. Isolate the glenohumeral joint motion from the scapulothoracic motion

by fixing the scapula.Holding both hands, assist the patient in ing arms to the normal maximum of 90 degrees, and note restriction of

C Figure 1-1.eral bending C:Neck motion.Rotation. A:Flexion and extension.B:

Lat-Figure 1-2.Internal rotation of shoulder, posterior view Record range of reach: dorsum of hand to specific vertebral bodies.

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motion on either side To determine internal and external rotation of theglenohumeral joint on each side, the examiner places one hand on the shoulder to prevent scapular motion and, with the other hand, assistseach arm to full external rotation of 90 degrees and full internal rotation

of 80 degrees (Fig 1-3)

2 Elbows

a. Inspect each elbow for maximum extension to 0 degrees and full flexion

to 150 degrees Less than full extension is reported in degrees as flexion formity or lack of extension

de-b. Inspection and palpation may reveal the presence of olecranon bursitis atthe elbow tip or the soft-tissue swelling of synovitis,which is felt in thefossae between the olecranon and lateral epicondyle or between the olecra-non and medial epicondyle

c Subcutaneous nodules and tophi should be sought in the olecranonbursa and over the extensor surface of the elbow and forearm

3 Wrist and hands

a. Inspect and palpate wrists; metacarpophalangeal (MCP), proximal phalangeal (PIP), and distal interphalangeal (DIP) joints of fingers; andcarpometacarpal (CMC), MCP, and interphalangeal (IP) joints of thumbs(Fig 1-4) Note shape and deformities: boutonniere, swan neck, and ulnardeviation

inter-b Soft-tissue swelling has a spongy consistency and should be sought onthe dorsum of the wrist, distal to the ulna and over the radiocarpal joint

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On the volar surface, the normal step-down from hand to forearmmay be obliterated by soft-tissue swelling Volar synovitis may be associ-ated with carpal tunnel syndrome Tapping on the volar aspect of the wristmay elicit paresthesiae radiating into the radial three fingers, or even theforearm This positive Tinel’s sign is consistent with carpal tunnel syn-drome Thenar atrophy would further support this diagnosis.

c. All finger joints should be examined by inspection and palpation for tissue swelling, capsular thickening, and bony enlargement

soft-d Average wrist motion is dorsiflexion to 75 degrees, palmar flexion to

70 degrees, ulnar deviation of 45 degrees, and radial deviation of 20 degrees(Table 1-1)

e. The fist is described as 100% when all fingers reach the palm of the handand the thumb closes over the fingers Halfway fist closing is recorded as50%; less than 50% and 75% are other possible intermediate measure-ments The distance from fingertips to palm can also be recorded

f Grip is quantified by noting the patient’s maximum strength in graspingtwo fingers of the examiner Pinch is assessed by the force necessary tobreak the patient’s pinch between index finger and thumb

g Pronation and supination are combined functions of the elbow and wristand are determined by having the patient hold the forearm horizontal andthe thumb up Pronation and supination are measured in degrees from theneutral position, with the hand turning palm up and palm down (Fig 1-5)

F.While the patient is sitting, customary physical examination of the neck and chest should be performed; it should include examination of sternoclavicularjoints and measurement of chest expansion, which should be greater than

5 cm in the nipple line.

IV SUPINE POSITION

A.Start with the standard physical examination of the abdomen,and then proceed

to the examination of the lower extremities.

B.Alignment of the kneesis compared with the alignment noted on weight bearing(see section II.B) Palpate pedal pulses

C Low back

1. Inspection, palpation, and assessment of range of motion (see section II.D)

2 Neurologic examination Look for radicular signs and root signatures (seesection I)

to the extended hip The maneuver exerts a traction force on the sacroiliac joint, which opens it up.

D Hips

1. Hip function is screened by gently log-rolling each lower extremity and notingthe freedom of motion of the ball-and-socket joint.Rolling also allows meas-urement of the internal and external rotationof the hip joint in extension

2. With one hand fixing the pelvis, the other hand moves each hip to the normal

60 degrees of full abductionand to the normal 30 degrees of adduction

while the hip is held in extension

3. Each hip joint is then examined in flexion;both lower extremities are flexed

at knees and hips and carried toward the chest, which gives the maximumangle (120 degrees) of flexion of each hip

4. Normal hip extensionis to 10 degrees To avoid overlooking a hip ion deformity for which accentuation of lumbar lordosis may compensate,the examiner keeps one lower extremity flexed over the chest, thereby flat-tening the lumbar spine, while instructing the patient to extend fully the op-posite leg

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flex-Average Joint Motion for Young Adults

Rotation (arm in abduction to 90º)

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5. With the hip in 90 degrees of flexion, the joint is evaluated for internal tion (25 degrees), external rotation (35 degrees), abduction (45 degrees), andadduction (25 degrees) (Fig 1-6).

rota-E Measurement of leg length(see Chapter 23) If the discrepancy is greater thanone-half inch, a heel lift for the shoe on the affected side should be ordered

F Knees

1. By inspection and palpation, note position and mobility of patellae.Kneeextension–flexion range is 0 to 130 degrees Also palpate for the presence ofosteophytes at the tibiofemoral joint margin, which may also be tender

2 Soft-tissue swelling is elicited by bimanual examination

a. Demonstrate intra-articular fluid by the patellar click sign.While pressing the suprapatellar pouch with one hand, push the patella againstunderlying fluid and the femoral condyle with the index finger of the otherhand to elicit a click

com-b. For detection of a small amount of effusion, use the bulge sign This neuver is best executed by placing both hands on the knee so that the indexfingers meet on the medial joint margin and the thumbs meet on the lateralaspect of the joint Through a firm stroking motion of the fingers above

Figure 1-6. Hip rotation in flexion.

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and below the patella, fluid is “milked” into the interior of the joint, andthe medial aspect of the joint becomes flat. The thumbs are thenpushed suddenly and firmly into the lateral joint margin, thereby produc-ing a bulge of fluid on the medial side of the joint.

3. The popliteal areais examined for the presence of a synovial cyst Standingmakes the cyst more prominent

4 Knee stabilityis evaluated by stressing medial and lateral collateral ments.Anteroposterior stability is assessed by holding the knee flexed withthe foot firmly anchored on the bed and using both hands to pull and push theleg (drawer sign) to test the cruciate ligaments.

liga-G Ankles and feet

1 Synovial soft-tissue swellingof the ankles at both malleoli should be tinguished from periarticular edema and fat pads

dis-2 Normal ankle motionis 15 degrees flexion and 35 degrees extension

3 Subtalar motion,which allows inversion and eversion of the foot, is best ported as a percentage of normal, with 100% meaning full mediolateral motion

re-H Toes.By inspection and palpation, note the following:

1 Alignmentand deformity: hammertoes, claw toes, and hallux valgus

2 Soft-tissue swelling and presence of inflammation, which are best documented

by mediolateral squeezing across the metatarsal joints; pain may be elicited

I Muscle examination Proximally and distally, note the following:

1. On inspection, muscle wastingand muscle atrophy.

2. On palpation, muscle tenderness.

3. On testing motion, muscle strength(Table 1-2)

J Neurologic examination

1. Standard evaluation of tendon reflexes.

2 Impairment of nerve root functionmust be sought with care, and motor andsensory deficits recorded (see Chapters 19 and 20)

3. Look for nerve entrapment,secondary to joint pathology (e.g., carpal nel syndrome)

tun-V SYSTEMATIC EXAMINATION AND JOINT CHART

A.Inspection, palpation, and movement of joints may reveal swelling, tenderness,temperature and color changes over the joint, crepitation, and deformity

1 Tenderness on direct pressure over the joint and stress painproduced whenthe joint, at the limit of its range of motion, is nudged a little farther are im-portant findings of inflammation The number of tender and swollen jointscan be recorded and compared with future joint counts after the institution oftherapy

2 Crepitation is a palpable or audible sensation with joint motion caused byroughened articular or extra-articular surfaces rubbing each other “Popping”sounds can also be heard and felt when tendons travel over bony prominences

3 Bony enlargement, subluxation, and ankylosis in abnormal positions cause deformity.

Gradations of Muscle Weakness

Grade Muscle involvement

0 No muscle contraction

1 Flicker or trace of contraction

2 Active movement possible with gravity eliminated

3 Active movement possible against gravity

4 Active movement possible against gravity and resistance

5 Normal muscle power

TABLE 1-2

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3. Other physical signs of joint abnormality include warmth and erythema over the jointand should be expressed as grades 1, 2, or 3 (mild, moderate,

or severe)

VI EXTRA-ARTICULAR FEATURES.Examination is completed by recording specificfindings important in rheumatic diseases, such as subcutaneous nodules, nail changes,rash, abnormal eye findings, sicca (dryness) signs of the eyes and mouth, lym-phadenopathy, leg ulcers, and visceral involvement such as splenomegaly, pleural orpericardial signs, and neurologic abnormalities

OVERALL ASSESSMENT OF JOINT STRUCTURE

insta-III FUNCTION.Assessment is based on the following:

A Joint range of motion.

B Muscle strength (grip strength, abduction of shoulders, straight leg raising, risingfrom squatting and sitting positions, and walking on toes) See Table 1-2

C Activities of daily living Mobility, personal care, special hand functions, andwork and play activities

D.Function can be reported in four classes based on the American College ofRheumatology classification:

Class 1 Normal function without or despite symptoms.

Class 2 Some disability but adequate for normal activity without special vices or assistance.

de-Class 3 Activities restricted; special devices or assistance required.

Class 4 Totally dependent.

Other, more quantitative instruments are available for the evaluation and prospectiveassessment of function, performance of social activities, and emotional status Specializedpain and function instruments are also available for clinical trials

In conclusion, a comprehensive clinical evaluation (history plus physical tion) focused on the musculoskeletal system and psychosocial consequences of disease, fol-lowed by a complete physical examination with a detailed musculoskeletal and jointevaluation, is the clinical basis for the diagnosis and individualized management of rheu-matic disease Such an approach allows the professional to distill large amounts of infor-mation rapidly to reach a specific diagnosis and formulate an appropriate, focused, andeffective therapeutic plan

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examina-■ KEY POINTS

The diagnosis of many rheumatologic disorders is made clinically, and so a detailed

med-ical history and thorough physmed-ical examination are unequivocally central to the initial evaluation of the patient A strong knowledge base of rheumatology streamlines the diagnostic process, en- abling quicker development of management plans.

Always treat the patient, not the laboratory results Although laboratory, radiologic, and

pathologic data can be very useful in the management of rheumatologic disorders, they should always be taken in the context of, and never supersede, the clinical picture Appreciating the limi- tations of diagnostic tests optimizes their clinical utility.

Uncertainty is rife in rheumatology and must be accepted Management decisions must

often be made even when the clinical picture is incomplete or atypical, or when clinical data is available or inaccessible.

un-■ Rheumatologic disorders are often variable in course and severity The aggressiveness of

therapy must be appropriate to the aggressiveness of disease, because both the treatment modalities and the illness carry potential dangers The chronic nature of many conditions necessi- tates ongoing vigilance, even during periods of disease quiescence.

Better education of the patient, especially with respect to the nature of illness and to

thera-peutic goals and expectations, and trust between the physician and patient optimize compliance and outcome.

Hanging in my examination room are reproductions of two French impressionist

paint-ings The first is the famous A Sunday on La Grande Jatte by Georges-Pierre Seurat who

pio-neered the technique of juxtaposing small dots of different colors to create images thatbecome apparent only when seen from a distance Even then, however, smaller details can re-main obscure and subtle I use this painting to illustrate to patients how I often approachrheumatologic conditions First, while I am generally called upon to evaluate a specific prob-lem, I do not focus solely on one single “dot” but rather view it in the context of all the “dots”

in order to see the whole clinical picture Second, even if the picture is spotted with areas offuzziness and uncertainty, it can still be fully appreciated and addressed with comfort

FRAMING THE CLINICAL INVESTIGATION

Many rheumatologic conditions are clinical diagnoses and are systemic in nature, so it not be overstated that the skills most important to the rheumatologist are those that arealso the most important to an astute internist These include the ability to obtain an accu-rate medical history and conduct a thorough physical examination and to be comfortablewith handling different organ systems The review of systems, in particular, often providescrucial pieces of information that may not be spontaneously volunteered by the patient andalso comprises a large part of my initial evaluations This process, although seemingly ex-hausting, can be made very efficient by attaining familiarity with potentially relevant con-ditions For example, an elderly man taking diuretics for hypertension who presents withrecurrent acute inflammation of the first metatarsophalangeal joint need not necessarily bequestioned for a history of sun sensitivity or a malar rash but should be questioned for ahistory of tophi or renal calculi A young woman with a history of multiple osteoporotic

can-THINKING LIKE A RHEUMATOLOGIST

Arthur M F Yee

2

12

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stress fractures should probably be asked about symptoms suggestive of malabsorptive

states A large fund of knowledge a priori improves the diagnostic process by generating

pertinent questions and discarding irrelevant ones

ADDRESSING THE PATIENT, NOT STUDY RESULTS

Laboratory, radiologic, and pathologic studies can be extremely useful to the gist, but they should only be obtained in the appropriate setting Inappropriate testing canoften increase diagnostic confusion as well as become a source of unnecessary anxiety forthe patient The utility of diagnostic testing is highly dependent on the pretest probability

rheumatolo-of a particular condition; therefore, an astute clinical evaluation beforehand remains tral For example, the presence of circulating antinuclear antibodies (ANA) is highly sensi-tive for the diagnosis of systemic lupus erythematosus (SLE) but is also notoriouslynonspecific Therefore, in considering the diagnosis of SLE, a negative test result can bevery useful in excluding this diagnosis, whereas a positive test result can best be used tosupport the clinical impression Conversely, the anti-dsDNA antibody is highly specific butonly moderately sensitive for SLE; therefore, it is less useful as a screening test and moreuseful (if positive) as a confirmatory test

cen-Tests such as the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)can provide useful information about the degree of activity of a systemic disease However,one should never be swayed blindly by the results of these tests and should always take theoverall clinical picture as the guide for developing the management plan A patient withpolymyalgia rheumatica (PMR) who has a slightly elevated ESR but who is feeling welldoes not need to have her corticosteroid dosage increased just to normalize the ESR Con-versely, a patient with PMR who complains of a recurrence of significant muscle stiffness inthe morning should probably increase her corticosteroid dosage no matter what the ESR is

LIVING COMFORTABLY WITH UNCERTAINTY

Because many rheumatologic diagnoses are made primarily on a clinical basis, one of thegreatest challenges in training rheumatology fellows is to teach them to become comfort-able with uncertainty This can only be accomplished by maximizing clinical experienceand maintaining a solid knowledge base

Many criteria and classification schemes for the diagnosis of rheumatologic disordershave been published, but for the most part, these were developed for the purpose of clinicaltrials and population studies and not as the sole basis for making diagnoses in specific pa-tients Therefore, a young woman with a malar rash, glomerulonephritis, and a positiveANA should be treated as a patient with SLE, even if she does not fulfill a fourth criterionfor diagnosis as established by the American College of Rheumatology

Not infrequently, a patient may present with an obvious but undiagnosed systemic flammatory condition which may be threatening a vital organ or even life, and awaiting adefinitive pathologic diagnosis may carry unacceptable risks An example of this may be anelderly woman presenting with fever, myalgias, and visual deficit of new vision loss, consis-tent with the diagnosis of giant cell arteritis (GCA) In this situation, therapy should not bedelayed pending a temporal artery biopsy because the risk of permanent blindness far out-weighs the risks of corticosteroid treatment Moreover, a strong argument can be made fortreating for presumptive GCA, even if the biopsy result is negative

in-On another occasion, a patient may have an inflammatory condition such as an stitial pneumonitis and show a steady decline in health status, but diagnostic testing has beenreasonably extensive to exclude infection or malignancy although no definitive diagnosis hasbeen arrived at Here again, systemic anti-inflammatory or immunosuppressive therapy may

inter-be appropriate, provided continued vigilance is maintained for new or progressive problems

TAILORING MANAGEMENT PLANS

Two important characteristics of many rheumatologic diseases are chronicity and widevariability in course and severity SLE may be quite mild for many years, with easily

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managed intermittent arthritic or dermatologic flares, or may be aggressive and fulminant

at any moment, with endangerment to vital organs or to life It is important to remember totreat the patient at hand, and not the diagnosis There is no one treatment regimen that isuniversally appropriate for any particular diagnosis, and the management plan needs to be

as potent as the severity of a particular case dictates Unlike oncologists who use very tent (and toxic) medications to treat a malignant neoplasm, that, if left untreated, wouldkill the patient, rheumatologists tend to walk a fine line between the threats imposed by thedisease and the toxicities carried by the therapies Close and vigilant monitoring for an in-definite period, to assess whether the patient requires medical treatment or not, is the rule

po-PARTNERING WITH THE PATIENT

Rheumatologic conditions are often difficult to understand for the medical professional, letalone the patient Therefore, every effort must be made to help the patient to the best ofhis/her ability to understand the nature of the illness and the goals of therapy and to estab-lish a trusting doctor–patient relationship Without understanding and rapport, complianceand, therefore, outcome are diminished

The second painting hanging on my wall is Two Young Girls at the Piano by Auguste

Renoir, who himself had severe arthritis and toward the end of his life required that hisbrushes be bound to his hands in order to paint It is said by some that Renoir sometimesportrayed his subjects as having the arthritic changes of his own hands, and I can make outthe synovitis on the hands of the two young girls in the painting I can only imagine whatmore he could have produced if he were not so disabled However, now with very effectivetreatments for our diseases, there is great promise to prevent their destructive conse-quences, and perhaps the full potential of all our patients can be protected and realized

IMMUNOLOGY FOR THE PRIMARY CARE PHYSICIAN

Mary K Crow

3

The function of the immune systemis to limit damage to the host by micro-organisms

The immune response to an infection has two components:

I The innate immune responseis the earliest phase of an immune response, triggered

by microbial components such as lipopolysaccharide (LPS), heat shock proteins, terial DNA, and viral double-stranded (ds) RNA It comprises phagocytes, includingmacrophages and neutrophils; natural killer (NK) cells; natural antibodies; solublemolecules, such as cytokines, chemokines, and immunomodulatory molecules (e.g.,prostaglandins); and the complement system Activation of the innate immune re-sponse is mediated by interaction of microbial components with members of the Toll-like receptor (TLR) family A close connection exists between the innate and adaptiveimmune systems via This is an important concept because it is possiblethat an infection triggers an autoimmune disorder through this mechanism

bac-II The adaptive immune response develops several days after the initiation of a mary immune response and is mediated by lymphocytes expressing cell membranereceptors specific to the invading pathogen Adaptive immunity is characterized by

pri-an increase in pri-antigen specificity over time pri-and development of immunologic memory

A Specificity.Individual T and B lymphocytes bear cell surface receptors that ognize a defined molecular structure (epitope) on an antigen (i.e., a molecule on

rec-or in a bacterium rec-or virus, rec-or a peptide, that elicits an immune response) Each phocyte expresses a single receptor of unique structure and antigenic specificity

lym-interferon-

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