Oxford American Handbook of Rheumatology Second Edition Published and upcoming Oxford American Handbooks Oxford American Handbook of Clinical Medicine Oxford American Handbook of Anesthesiology Ox ford American Handbook of Cardiology Oxford American Handbook of Clinical Dentistry Oxford American Handbook of Clinical Diagnosis Oxford American Handbook of Clinical Drugstore Oxfo rd American Handbook of Critical Care Oxford American Handbook of Disaster Medicine Oxford American Handbook of Emergency Medicine Oxford American Handbook of Endocrinology and Diabe tes Oxford American Handbook of Geriatric Medicine Oxford American Handbook of Hospice and Palliative Medicine Oxford American Handbook of Infectious Diseases Oxford American Handboo k of Nephrology and Hypertension Oxford American Handbook of Neurology Oxford American Hand book of Obstetrics and Gynecology Oxford American Handbook of Oncology Oxford American Hand book of Otolaryngology Oxford American Handbook of Pediatrics Oxford American Handbook of P hysical Medicine and Rehabilitation Oxford American Handbook of Psychiatry Oxford American Handbook of Pulmonary Medicine Oxford American Handbook of Radiology Oxford American Handbo ok of Reproductive Medicine Oxford American Handbook of Rheumatology Oxford American Handbo ok of Sports Medicine Oxford American Handbook of Surgery Oxford American Handbook of Urolo gy Oxford American Handbook of Rheumatology Second Edition Philip Seo Director, The Johns Hopkins Vasculitis Center Director, Johns Hopkins Rheumatolo gy Fellowship Associate Professor of Medicine Division of Rheumatology The Johns Hopkins Un iversity School of Medicine with Alan J. Hakim Gavin P.R. Clunie Inam Haq 1 3 Oxford University Press is a department of the University of Oxford. It furthers the Univer sity \200\231s objective of excellence in research, scholarship, and education by publishin g worldwide. Oxford New York Auckland Cape Town Dar es Salaam Hong Kong Karachi Kuala Lumpur Madrid Melb ourne Mexico City Nairobi New Delhi Shanghai Taipei Toronto With of \201 ces in Chile Czech Republic France Greece Argentina Austria Brazil Japan Pola nd Portugal Singapore Italy Guatemala Hungary South Korea Switzerland Thailand Turkey Ukrai ne Vietnam Oxford is a registered trademark of Oxford University Press in the UK and certain other cou ntries. Published in the United States of America by Oxford University Press 198 Madison Avenue, Ne w York, NY 10016 Oxford University Press 2013 All rights reserved. No part of this publication may be reproduced, stored in a retrieval s ystem, or transmitted, in any form or by any means, without the prior permission in writing of Oxford University Press, or as expressly permitted by law, by license, or under terms a greed with the appropriate reproduction rights organization. Inquiries concerning reproduct ion outside the scope of the above should be sent to the Rights Department, Oxford Universi ty Press, at the address above. You must not circulate this work in any other form and you must impose this same condition on any acquirer. Library of Congress Cataloging-in-Publication Data Seo, Philip. Oxford American handbook of rheumatology / Philip Seo. \200\2242nd ed. p. ; cm. \200\224(Oxford American handbooks) Ha ndbook of rheumatology Adapted from: Oxford handbook of rheumatology / Alan J. Hakim, Gavin P.R. Clunie, Inam Haq. 3rd ed. 2011. Includes bibliographical references and index. ISBN 9 78 \200\2230 \200\22319 \200\223990799 \200\2231 (alk. paper) I. Hakim, Alan. Oxford handbo ok of rheumatology. Handbook of rheumatology. [DNLM: 1. Rheumatic Diseases \200\224Handbook s. WE 39] LCClassi \201 cation not assigned 616.7 \200 23 \200\224dc23 II. Title. III. Title: IV. Series: Oxford American handbooks. 2012035653 9 8 7 6 5 4 3 2 1 Printed in the United States of America on acid-free paper This material is not intended to be, and should not be considered, a sub- stitute for medic al or other professional advice. Treatment for the con- ditions described in this material is highly dependent on the individual circumstances. Although this material is designed to offer accurate infor- mation with respect to the subject matter covered and to be current a s of the time it was written, research and knowledge about medical and health issues are co nstantly evolving and dose schedules for medica- tions are being revised continually, with new side effects recognized and accounted for regularly. Readers must therefore always chec k the product information and clinical procedures with the most up-to-date published produc t information and data sheets provided by the manufacturers and the most recent codes of co nduct and safety regulation. Oxford University Press and the authors make no representation s or warranties to readers, express or implied, about the accuracy or completeness of this material, including, without limitation, that they make no representation or war- ranties a bout the accuracy or ef \201 cacy of the drug dosages mentioned in the material. The autho rs and the publishers do not accept, and expressly disclaim, any responsibility for any lia bility, loss, or risk that may be claimed or incurred as a consequence of the use and/or ap plication of any of the contents of this material. This page intentionally left blank vii Foreword Patients with rheumatic disorders present so many different challenges that almost every pr acticing physician needs some familiarity with rheu- matology. An abbreviated litany of com mon rheumatic symptoms and signs \200\223arthralgias, myalgias, weakness, back pain, and ne ck pain \200\223reinforces the observation that rheumatic disorders are among the most freq uent reasons patients present to family physicians, general internists, geriatri- cians, an d orthopedists. Although many rheumatic diseases are temporary nuisances, others can cause life-threatening, multisystem diseases that can be encountered by hospitalists, critical ca re specialists, nephrologists, car- diologists, neurologists, and a myriad of other consult ants. Distinguishing among the more than 150 different forms of arthritis, although often c hal- lenging, is important, given the recent dramatic increase in speci \201 c and effecti ve therapies that are now available. Fortunately, this handbook will offer any physician substantial help in meeting the challen ges posed by rheumatic diseases. The liberal use of bulleted summary points and tables make s this handbook both concise and thorough. Physicians eager to improve their clinical skill s will enjoy the excellent photographs that illustrate important aspects of the muscu- losk eletal physical examination. The chapter on rheumatic emergencies will be especially useful to hospitalists. I believe this handbook will allow many physicians to experience the joy and sense of accom plishment that can come from diagnosing and treating patients with rheumatic diseases. David B. Hellmann, M.D., M.A.C.P. Aliki Perroti Professor of Medicine Vise Dean, Johns Hopk ins Bayview Medical Center Chairman, Department of Medicine Johns Hopkins Bayview The Johns Hopkins University School of Medicine This page intentionally left blank ix Preface Rheumatology is one of the last great frontiers in medicine. In this modern era, in which w e hear less about neck veins and more about BNP levels, rheumatology remains the exception to the rule. Despite the growing number of diagnostic tests we have at our disposal, they r emain a mere adjunct to the patient evaluation. This is one of the last subspecialties in w hich it is not only common, but appropriate, to ignore a positive ANA or joint space narrow ing, and instead listen to what the patient has to say. That said, rheumatology is a \201 eld in transition. Long behind us are the days in which clinical trials compared the ef \201 cacy of one NSAID to another. The advent of biologic agents in particular has led to a new era in thera- peutics in which largely nonspeci \201 c immunosuppressive drugs are being replaced by progressively more nuanced agents. The day that molecular phenotyping of patients joins clinical phenotyping as a way of selectin g treatment strategies cannot be far behind. During the last few years, the rate of transitions has occurred at break- neck speed. Since the publication of the \201 rst edition, new classi \201 cation criteria have been publ ished for systemic lupus erythematosus, Sj gren \200\231s syndrome, polymyalgia rheumati ca, and rheumatoid arthritis. For the \201 rst time, we have FDA-approved agents for the treatment of both systemic lupus erythematosus and ANCA-associated vasculitis. Finally, we are start- ing to see oral biologic agents hit the market, allowing patients to take advant age of our most advanced therapeutics without subjecting them to the needle. For the present, rheumatology encompasses a dizzying amount of infor- mation; no book can b e expected to replace years of clinical experience. I believe and hope, however, that this book will \201 nd a home in many white-coat pockets, from the attending who just needs to refresh his or her memory on a few details, to the trainee who is looking for some way of getting a toehold in this daunting \201 eld. More than anything else, this book is meant to provide you with an approach to the patient, and a framework on which you can build. This book represents just the beginning of a conti nually evolving, continuously changing story that never fails to fascinate. Philip Seo, MD, MHS Director, the Johns Hopkins Vasculitis Center Director, Johns Hopkins R heumatology Fellowship Associate Professor of Medicine Johns Hopkins University School of M edicine This page intentionally left blank xi Acknowledgments Last year, I became Director of the Rheumatology Fellowship at Johns Hopkins, and with that new position came a deep sense of obligation to improve my general understanding of rheuma tology \200\224no small feat after many years of seeing only patients with systemic vasculi tis. My new position has allowed me to work even more closely with my col- leagues, and has left me awestruck by the depths of both their knowledge and their generosity. As I have said before, the greatest gift that a fellow academic can give is his or her time . I am therefore pleased to thank publicly my friends and colleagues who graciously reviewe d these chapters during airport layo- vers and other moments stolen from far more deserving obligations: Alan Baer, Clifton (Bing) Bingham, Lisa-Christopher-Stine, Fred Wigley, Grant Louie, Laura Hummers, Khalil Ghanem, Michelle Petri, Geeta Sood, and Sangeeta Sule. This motley crew gently (or not so) informed me when I was woefully misinformed \200\224whi ch was more often than I would like to admit \200\224and credit for many of the improvement s to this second edition really belongs to them. I also wish to thank Antony Rosen and David Hellmann, whose avuncular support has provided my career with a second act. I continue to be grateful to Alan Hakim, Gavin Clunie, and Inam Haq, who wrote the original Oxford Handbook of Rheumatology, and provided the backbone (and much of the meat) for this current edition. Also, I would be remiss if I did not thank Andrea Seils, Senior Editor of Clinical Medicine at Oxford University Press, whose encouragement and support have been in valuable. Finally, I dedicate this work to my family, who mean more to me with each passing year: Kyu ng Hwa Seo, Hae Ja Yoon Seo, Susan Seo, and Ellen Seo. I also dedicate this work to my niec e and nephew, Avery Pusey and Jackson Pusey, who \200\224as they pointed out to me \200\224 I had neglected to name in the \201 rst edition, but are never far from my heart. Philip Seo Baltimore February 2013 This page intentionally left blank xiii Contents Detailed contents xv List of color plates xxi Symbols and abbreviations xxiii Part 1: The presentation of rheumatic disease 1 Evaluating musculoskeletal pain 2 Regional musculoskeletal conditions: making a 3 working diagnosis 11 3 Patterns of disease presentation: making a working diagnosis 149 4 The spectrum of presentation of rheumatic disease 195 Part 2: The clinical features and management of rheumatic diseases 5 Rheumatoid arthritis (RA) 6 Osteoarthritis (OA) 7 The crystal arthropathies 8 The spondyl oarthropathies 9 Juvenile idiopathic arthiritis (JIA) 10 Systemic lupus erythematosus (SLE) 11 The antiphospholipid (antibody) 235 261 271 283 309 329 syndrome (APS) 351 361 373 12 Sj gren \200\231s syndrome (SS) 13 Systemic sclerosis and related disorders 14 Idiopa thic in \202 ammatory myopathies \200\224 polymyositis (PM) and dermatomyositis (DM) 397 417 15 Primary vasculitides CONTENTS xiv CONTENTS 16 Metabolic bone diseases and disorders of collagen 443 481 499 529 537 17 Infection and rheumatic disease 18 Miscellaneous conditions 19 Common upper limb musculo skeletal lesions 20 Back pain 21 Complementary and alternative medicine in rheumatology 553 561 22 Rheumatologic emergencies Index 577 xv Detailed contents List of color plates xxi Symbols and abbreviations xxiii Part 1 The presentation of rheumatic disease 1 Evaluating musculoskeletal pain 3 Introduction 4 Localization of pain and pain patterns 6 Changes in pain on examination 8 The assessment of pain in young children 9 2 Regional musculoskeletal conditions: making a working diagnosis 11 Introduction 12 Neck pain 14 Shoulder pain 22 Pain around the elbow 34 Wrist pain 40 Symptoms in the hand 46 Upper limb peripheral nerve lesions 58 Thoracic back and chest pain 64 Low back pain and disorders in adults 70 Spinal disorders in children and adolescents 84 Pelvic, groin, and thigh pain 92 Knee pain 104 Lower leg and foot disorders (adults) 118 Child and adolescent foot disorders 132 Corticosteroid injection therapy 142 Principles of rehabilitation 146 3 Patterns of disease presentation: making a working diagnosis 149 Monoarticular pains in adults 150 Oligoarticular pains in adults 152 xvi DETAILED CONTENTS Oligoarticular pains in children and adolescents 162 Widespread pain in adults 170 Widespread pain in children and adolescents 186 4 The spectrum of presentation of rheumatic disease 195 Skin disorders and rheumatic disease 196 Skin vasculitis in adults 202 Skin vasculitis in children and adolescents 206 Cardiac manifestations 210 Pulmonary manifestations 214 Renal manifestations 216 Endocrine manifestations 220 Gut and hepatobiliary manifestations 224 Malignancy 230 Part 2 The clinical features and management of rheumatic diseases 5 Rheumatoid arthritis (RA) 235 Disease criteria and epidemiology 236 Incidence, prevalence, and morbidity 238 The clinical features of rheumatoid arthritis 239 Organ disease in rheumatoid arthritis 240 The evaluation and treatment of rheumatoid arthritis 244 Rheumatoid factor positive polyarthritis in childhood 259 6 Osteoarthritis (OA) 261 Introduction 262 Clinical features of OA 264 The investigation of OA 266 The management of OA 268 7 The crystal arthropathies 271 Gout and hyperuricemia 272 Calcium pyrophosphate dihydrate (CPPD) disease 278 Basic calcium phosphate (BCP) associated disease 281 Calcium oxalate arthritis 282 DETAILED CONTENTS xvii 8 The spondyloarthridities 283 Introduction 284 Diagnostic criteria and clinical subsets 286 Ankylosing spondylitis 290 Psoriatic arthritis 296 Reactive arthropathy 300 Enteric arthropathy 302 Undifferentiated spondyloarthritis 303 SAPHO 304 Spondyloarthridities in childhood 306 9 Juvenile idiopathic arthritis (JIA) 309 Introduction 330 Oligoarthritis 312 Systemic arthritis 316 Rheumatoid factor negative polyarthritis in childhood 321 Rheumatoid factor negative polyarticular JIA 322 Rheumatoid factor positive polyarticular JIA 324 Juvenile psoriatic arthritis 325 Enthesitis related arthritis 326 Chronic, infantile, neurological, cutaneous, & articular syndrome (CINCA) 327 Still \200\231s disease 328 10 Systemic lupus erythematosus (SLE) 329 Introduction 330 The clinical features of SLE 334 Antiphospholipid (antibody) syndrome and SLE 338 Pregnancy and SLE 339 Diagnosis and investigation of SLE 340 Drug-induced lupus erythematosus (DILE) 342 The treatment of SLE 344 Prognosis and survival in SLE 348 Childhood SLE 349 Neonatal SLE 350 xviii DETAILED CONTENTS 11 The antiphospholipid (antibody) syndrome (APS) 351 Introduction 352 Epidemiology and pathology 353 Clinical features of APS 354 Treatment of APS 356 Catastrophic APS (CAPS) 358 12 Sj gren \200\231s syndrome (SS) 361 Epidemiology and pathology 362 Clinical manifestations of SS 364 Investigation of SS 368 Treatment of SS 370 13 Systemic sclerosis and related disorders 373 Epidemiology and diagnostic criteria 374 Cutaneous features of scleroderma and their treatment 380 Systemic features of the disease, investigation, and treatment 384 Anti \201 brotic and immunosuppressive therapies for systemic sclerosis 390 Summary \200\224the approach to systemic sclerosis 392 Scleroderma-like \201 brosing diso rders 394 14 Idiopathic in \202 ammatory myopathies \200\224 polymyositis (PM) and dermatomyositis (DM) 397 Epidemiology and diagnosis 398 Clinical features of PM and DM 400 Investigation of PM and DM 404 Autoantibodies in myositis 406 Treatment of PM and DM 410 Inclusion-body myositis (IBM) 413 PM and DM in children 414 15 Primary vasculitides 417 Introduction 418 Large-vessel vasculitis 421 DETAILED CONTENTS xix Takayasu \200\231s arteritis 422 Polymyalgia rheumatica and giant cell arteritis 424 Polyarteritis nodosa 429 Granulomatosis with polyangiitis (Wegener \200\231s granulomatosis) 430 Other forms of AAV: microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA) 434 Small-vessel and single organ vasculitis 438 Kawasaki disease 442 16 Metabolic bone diseases and disorders of collagen 443 Osteoporosis 444 Osteomalacia and rickets 454 Parathyroid disease and related disorders 458 Paget \200\231s disease of bone 466 Miscellaneous diseases of bone 470 Molecular abnormalities of collagen and \201 brillin 474 17 Infection and rheumatic disease 481 Introduction 482 Pyogenic nongonococcal and gonococcal arthritides 486 Mycobacterium tuberculosis 490 Osteomyelitis 492 Lyme disease 494 Rheumatic fever 496 18 Miscellaneous conditions 499 Beh et \200\231s disease 500 Sarcoidosis 504 Miscellaneous skin conditions associated with arthritis 508 Chronic regional pain syndrome 514 Relapsing polychondritis 516 Miscellaneous disorders of synovium 518 Amyloidosis 520 Fibromyalgia and chronic widespread pain 524 xx DETAILED CONTENTS 19 Common upper limb musculoskeletal lesions 529 Shoulder (subacromial) impingement syndrome 530 Adhesive capsulitis (AC) 533 Lateral epicondylitis (tennis elbow) 534 20 Back pain 537 Conditions causing acute or subacute back pain in adults 538 Management of chronic back pain 544 Management of back pain in children and adolescents 546 21 Complementary and alternative medicine in rheumatology 553 Introduction 554 Herbal remedies (phytotherapy) 555 Physical and hands-on therapies 556 Homeopathy 557 Other CAMs 558 22 Rheumatologic emergencies 561 Septic arthritis 562 Infections in patients taking anti-TNF -A drugs 566 Acute SLE 568 Syst emic vasculitis 574 Scleroderma (Scl) crises 575 Methotrexate-induced pneumonitis 576 Index 577 xxi List of color plates Plate 1 MR scan of the neck showing loss of height and signal affecting several disks with multisegmental spondylotic bars, compression of the cord from protrusion of the C5/6 disc a nd myelopathic changes (high sig- nal) in the cord. Plate 2 Patterns of radiographic abnormality in chronic SAI: sclerosis and cystic changes i n greater tuberosity. Plate 3 Dactylitis, nail changes, and DIP joint arthritis in psoriatic arthritis. Plate 4 (a) Normal nailfold capillaries. (b) Nailfold capillaries in sclero- derma showing avascular areas and dilated capillaries in an irregular orien- tation (original magni \201 cation 65x). Plate 5 Diffuse arm and hand swelling in chronic regional pain syndrome (re \202 ex sympat hetic dystrophy). Plate 6 Slight \202 exion of fourth and \201 fth \201 ngers as a result of an ulnar n erve lesion at the elbow. The area of sensory loss is indicated by the dotted line. Plate 7 Psoriatic spondylitis: Nonmarginal and \200\234 \202 oating \200\235 (nonattached ) syndesmophytes. Plate 8 Spondylolysis. The defect in the pars interarticularis (black arrows) may only be n oted on an oblique view. The patient has had a spinal fusion (open arrows). Plate 9 Testing passive hip \202 exion and rotational movements (a) and hip abduction (b) .The pelvis should be \201 xed when testing abduction and adduction. Plate 10 Bone scintigraphy showing osteonecrosis of the left femoral head (on the right-han d side as this is an anterior view). Photopenia (an early sign) corresponds to ischemia. Plate 11 The \200\234patellar tap \200\235 test. Any \202 uid in the suprapatellar pouch is squeezed distally by the left hand. The patella is depressed by the right hand. It will normally tap the underlying femur immediately. Any delay in eliciting the tap or a feeling of damping as the patella is depressed suggests a joint effusion. Plate 12 Injection of the glenohumeral joint via the anterior route. Plate 13 Injection of the subacromial space. Plate 14 Injection of tennis elbow (lateral humeral epicondylitis/enthesitis). LIST OF COLOR PLATES xxii Plate 15 Injection of the carpal tunnel to the ulnar side of the palmaris longus tendon. Plate 16 Nodules associated with joint diseases. (a) RA: typically over extensor surfaces a nd pressure areas. (b) Chronic tophaceous gout: tophi can be indistinguishable clinically f rom RA nodules though may appear as eccentric swellings around joints (image provided court esy of Dr. R. A. Watts). (c) Multicentric reticulohistiocytosis: nodules are in the skin, a re small, yellowish-brown, and are often around nails. (d) Nodal OA: swelling is bony, typi cally at PIPs and DIPs. Plate 17 Bone scintigraphy ( 99m Tc MDP) of a 65-year-old man with wide- spread bone pain a nd weakness suspected to have metastatic malignancy. Undecalci \201 ed transiliac bone bio psy con \201 rmed severe osteomalacia. There was coincidental Paget \200\231s disease (arr owed lesions). Plate 18 Increased growth of the left lower limb due to chronic knee in \202 ammation in ( RF-) JIA. Plate 19 Lupus pernio presenting as a bluish-red or violaceous swelling of the nose extendi ng onto the cheek. Plate 20 Nailfold capillaries, demonstrating normal capillary loops. Magni \201 ed 300x. P hotograph used with the kind permission of Graham Dinsdale, Tonia Moore, and Ariane Herrick . Plate 21 Nailfold capillaries, demonstrating abnormal capillary loops, including capillary loop dilatation and dropout. Magni \201 ed 300x. Photograph used with the kind permission of Graham Dinsdale, Tonia Moore, and Ariane Herrick. xxiii Symbols and abbreviations 1 alert/warning p Primary s Secondary i increase/raise d decrease/reduce 4 male(s) 5 female(s) 9 plus or minus A Alpha B Beta ACA Anticentromere antibody AC(J) Acromioclavicular (joint) ACR American College of Rheumatology ADM Abductor digiti minimi ALP Alkaline phosphatase ALT Alanine transaminases ANA Anti-nuclear antibody ANCA Antineutrophil cytoplasmic antibody AP Anteroposterior APB Abductor pollicis brevis APL Abductor pollicis longus APS Antiphospholipid (antibody) syndrome ARA American Rheumatism Association AS Ankylosing spondylitis AST Aspartate transaminase ASOT Antistreptolysin O titer ASU Avocado/soybean unsaponi \201 able AZA Azathioprine BCP Basic calcium phosphate (crystals) bid Twice daily BJHS Benign joint hypermobility syndrome SYMBOLS AND ABBREVIATIONS xxiv BMC Bone mineral content BMD Bone mineral density BSR British Society of Rheumatology C Cervical (e.g., C6 is the sixth cervical vertebra) CA Coracoacromial CBC Complete blood count CINCA Chronic, infantile, neurological, cutaneous, and articular syndrome CK Creatine phosphokinase CMC(J) Carpometacarpal (joint) CMP Comprehensive metabolic panel CMV Cytomegalovirus CPPD Calcium pyrophosphate deposition (arthritis) CREST Calcinosis, Raynaud \200\231s, Esophageal dysmotility, Sclerodactyly, Telangiectasia (syndr ome) CRP C-reactive protein CS Congenital scoliosis CSS Churg \200\223Strauss syndrome CT Computed tomography CTS Carpal tunnel syndrome CXR Chest radiograph dcSScl Diffuse cutaneous systemic sclerosis DEXA/ DXA Dual-energy X-ray absorptiometry DIP(J) Distal interphalangeal (joint) DISH Diffuse idiopathic skeletal hyperostosis DLCO Diffusion capacity for carbon monoxide DM Dermatomyositis DMARD Disease-modifying antirheumatic drug DVT Deep vein thrombosis EA Enteropathic arthritis EBV Epstein \200\223Barr virus ECG (EKG) Electrocardiogram ECM Erythema chronicum migrans SYMBOLS AND ABBREVIATIONS xxv ECRB Extensor carpi radialis brevis ECRL Extensor carpi radialis longus ECU Extensor carpi ulnaris ED Extensor digitorum EDL Extensor digitorum longus EDM Extensor digiti minimi EDS Ehlers \200\223Danlos syndrome EHL Extensor hallucis longus EI Extensor indicis ELMS Eaton \200\223Lambert myasthenic syndrome EMG Electromyography EN Erythema nodosum ENA(S) Extractable nuclear antigen(s) EPB Extensor pollicis brevis EPL Extensor pollicis longus ERA Enthesitis-related arthritis ESR Erythrocyte sedimentation rate ESSG European Spondyloarthropathy Study Group EULAR European League Against Rheumatism FCR Flexor carpi radialis FCU Flexor carpi ulnaris FDP Flexor digitorum profundus FDS Flexor digitorum super \201 cialis FHB Flexor hallucis brevis FM Fibromyalgia FMF Familial Mediterranean fever FPL Flexor pollicis longus FR Flexor retinaculum GARA Gut-associated reactive arthritis GBS Guillain Barr syndrome GCA Giant cell arteritis GFR Glomerular \201 ltration rate GI Gastrointestinal SYMBOLS AND ABBREVIATIONS xxvi GOA Generalized osteoarthritis HA Hydroxyapatite HIV Human immunode \201 ciency virus HLA Human leukocyte antigen HO Hypertrophic osteoarthropathy HSP Henoch \200\223Sch nlein purpura HTLV Human T-cell leukemia virus IL Interleukin ILAR International League of Associations for Rheumatology IM Intramuscular(ly) INR International normalized ratio ITB Iliotibial band ITP Idiopathic thrombocytopenic purpura JCA Juvenile chronic arthritis JIA Juvenile idiopathic arthritis JIO Juvenile idiopathic osteoporosis KD Kawasaki disease L Lumbar (e.g., L5 is the \201 fth lumbar vertebra) LCL Lateral collateral ligament LDH Lactate dehydrogenase LFTS Liver function tests lcSScl Limited cutaneous systemic sclerosis MCP(J) Metacarpophalangeal (joint) MCL Medial collateral ligament MCTD Mixed connective tissue disease MG Myasthenia gravis MND Motor neuron disease MPA Microscopic polyangiitis MR Magnetic resonance MTP(J) Metatarsophalangeal (joint) MTX Methotrexate NMS Neuromuscular scoliosis NSAID Nonsteroidal anti-in \202 ammatory drug OA Osteoarthritis SYMBOLS AND ABBREVIATIONS xxvii OI Osteogenesis imperfecta PAN Polyarteritis nodosa PBC Primary biliary cirrhosis PCR Polymerase chain reaction PIN Posterior interosseous nerve PIP(J) Proximal interphalangeal (joint) PL Palmaris longus PLM Polarized light microscopy PM Polymyositis PMN Polymorphonuclear neutrophil PMR Polymyalgia rheumatica PSA Psoriatic arthritis PSA Prostatic speci \201 c antigen PTH Parathyroid hormone PV Plasma viscosity PVNS Pigmented villonodular synovitis qhs Every night, at bedtime qid Four times daily RA Rheumatoid arthritis RF Rheumatoid factor RNP Ribonucleoprotein RP Raynaud \200\231s phenomenon REA Reactive arthritis RSD Re \202 ex sympathetic dystrophy (algo/osteodystrophy) RSI RS3PE Repetitive strain injury Remitting seronegative symmetrical synovitis with pitting edema RTA Renal tubular acidosis sACE Serum angiotensin converting enzyme SAI Subacromial impingement SAPHO Synovitis, acne, palmoplantar pustulosis, hyperostosis, aseptic osteomyelitis (syndrome) SARA Sexually transmitted reactive arthritis sq Subcutaneous(ly) SC(J) Sternoclavicular (joint) SYMBOLS AND ABBREVIATIONS xxviii SI(J) Sacroiliac (joint) SLE Systemic lupus erythematosus SpA Spondyloarthritis SS Sj gren \200\231s syndrome SScl/Scl Systemic sclerosis/Scleroderma T Thoracic (e.g. T5 is the \201 fth thoracic vertebra) TB Tuberculosis Tid Three times daily TENS Transcutaneous electrical nerve stimulation TFTs Thyroid function tests TIA Transient ischemic attack TM(J) TNF( ) TPMT Temporomandibular (joint) Tumor necrosis factor (alpha) Thiopurine S-methyltransferase TRAPS Tumor necrosis factor-associated periodic syndrome TSH Thyroid stimulating hormone UC Ulcerative colitis US Ultrasound UV Ultraviolet WG Wegener \200\231s granulomatosis WHO World Health Organization Part I The presentation of rheumatic disease 1 Evaluating musculoskeletal pain 2 Regional musculoskeletal conditions: 3 making a working diagnosis 11 3 Patterns of disease presentation: making a working diagnosis 149 4 The spectrum of presentation of rheumatic disease 195 This page intentionally left blank 3 Chapter 1 Evaluating musculoskeletal pain Introduction 4 Localization of pain and pain patterns 6 Changes in pain on examination 8 Th e assessment of pain in young children 9 CHAPTER 1 Evaluating musculoskeletal pain 4 Introduction Pain is the most common musculoskeletal symptom. It is de \201 ned by its sub jective description, which may vary depending on its physical (or bio- logical) cause, the patient \200\231s understanding of it, its impact on function, and the emotional and behavi oral response it invokes. Pain is also often colored by cultural, linguistic, and religious differences. Therefore, pain is not merely an unpleasant sensation; it is in effect an emo tional change. The experience is different for every individual. Patients who think of them - selves as having a high pain threshold may have the hardest time coping. In children and adolescents, the evaluation of pain is sometimes compli- cated further by the interacting i n \202 uences of the experience of pain within the family, school, and peer group. This page intentionally left blank CHAPTER 1 Evaluating musculoskeletal pain 6 Localization of pain and pain patterns \200 Adults usually localize pain accurately, alth ough there are some situations worth noting in rheumatic disease where pain may be poorly localized (see Table 1.1). Adults may not clearly differentiate between periarticular and articular pain, referring to bursitis, tendonitis, and other forms of soft tissue injury as \200\234joint pain. \200 \235 Therefore, it is important to con \201 rm the precise location of the pain on physica l examination. Pain may be well localized but caused by a distant lesion, e.g., interscapular pain caused by mechanical problems in the cervical spine, or right shoulder p ain caused by acute cholecystitis. Pain caused by neurological abnormalities, ischemic pain, and pain referred from viscera are harder for the patient to visualize or express, and the history m ay be more dif \201 cult to interpret. Bone pain is generally constant despite movement or change in posture \200\224unlike muscular, synovial, ligament, or tendon pain \200\224and often distu rbs sleep. Fracture, tumor, and metabolic bone disease are all possible causes. Such consta nt, localized, sleep-disturbing pain should always be investigated. Patterns of pain distribution are associated with certain musculoskeletal conditions. For example, polymyalgia rheumatica (PMR) typically affects the shoulder girdle and hips, whereas rheumatoid arthritis (RA) affects the joints symmetrical ly, with a predilection for the hands and feet. Patterns of pain distribution may overlap, especially in the elderly, who may have several conditions simultaneously. For example, hip and/or knee osteoarthritis (OA), peripheral vas cular disease, and degenerative lumbar spine all may cause lower extremity discomfort. The quality of pain Some individuals \201 nd it hard to describe pain or use descriptors of sever- ity. A description of the quality of pain can often help determine the cause. Cer tain pain descriptors are associated with nonorganic pain syndromes (see Table 1.2): \200 Burning pain, hyperpathia (i.e., an exaggerated response to painful stimuli), and allodyni a (i.e., pain from stimuli that are normally not painful) suggest a neurological cause. A change in the description of pain in a patient with a long-standing condition is worth no ting, since it may denote the presence of a second condition, e.g., a fracture or septic ar thritis in a patient with established RA. Repeated, embellished, or elaborate description (i.e., \200\234catastrophizing \200\235) m ay suggest nonorganic pain, but be aware that such a presentation may be cultural. LOCALIZATION OF PAIN AND PAIN PATTERNS 7 Table 1.1 Clinical pointers in conditions where pain is poorly localized Diagnosis Clinical pointer Periarticular shoulder pain Referred to deltoid insertion Carpal tunnel syndrome Nocturnal paresthesias and/or pain, often diffuse Trochanteric bursitis Nocturnal pain lying on affected side Hip Synovitis Groin/outer thigh pain radiating to the knee Table 1.2 Terms from the McGill pain scale that help distinguish between organic and nonorg anic pain syndromes Organic Nonorganic Pounding Flickering Jumping Shooting Pricking Lancinating ( \200\234Shooting \200\235) Sharp Lacerating Pinching Crushing Hot Searing Tender Splitting Nagging Torturing Spreading Piercing Annoying Unbearable Tiring Exhausting Fearful Terrifying Tight Tearing CHAPTER 1 Evaluating musculoskeletal pain 8 Changes in pain on examination Eliciting changes in pain by the use of different examinatio n techniques may be used to provide clues to the diagnosis: \200 Palpation and comparison of active and passive range of motion can be used to reproduce pain and localize pathology . This requires practice and a good knowledge of anatomy. Many of the classic physical exam signs and maneuvers have a high degree of interobserver v ariability. Interpretation should take into account the context in which the examination is done and the effects of suggestibility. Palpation and passive range of motion exercises are performed while the patient is relaxed. The concept of passive movement is the assumption that when t he patient is completely relaxed, the muscles and tendons around the joint are removed as p otential sources of pain; in theory, passive range of motion is limited only by pain at the true joint. This assumption has its own limitations, however, especially since passive mov ements of the joint will still cause some movement of the soft tissues. In some cases, e.g. , shoulder rotator cuff disease, the joint may be painful to move passively because of subl uxation or impingement due to a musculotendinous lesion. The clinician should be aware of myofascial pain when palpating musculotendinous structures, especially around the neck and shoulder regions. Myofascial pa in is said to occur when there is activation of a trigger point that elicits pain in a zone stereotypical for the individual muscle. It is often aching in nature. Trigger points are associated with palpable, tender bands of skeletal muscle that are hyper irritable. Trigger points are tender to palpation, and pressure may induce a stereotyped pa ttern of referred pain. This is different from the tender points characteristic of \201 b romyalgia, which tend to be present symmetrically throughout the body and do not induce ref erred pain. Local anesthetic in \201 ltration at the site of a painful structure is sometimes used to help localize pathology, e.g., injection under the acromion may provide s ubstantial relief from a \200\234shoulder impingement syndrome. \200\235 However, the tech nique is reliable only if localization of the injected anesthetic can be guaranteed. Few, i f any, rigorously controlled trials have shown it to give speci \201 c results for any con dition. THE ASSESSMENT OF PAIN IN YOUNG CHILDREN 9 The assessment of pain in young children The assessment of pain in young children is often dif \201 cult: \200 Young children often localize pain poorly. Careful identi \201 cati on of the painful area is necessary through observation and palpation. A child may not admit to pain but will withdraw the limb or appear anxious when the painful area is examined. Observing a child \200\231s facial expression during an examination is very important, as is the parent \200\231s response. Quanti \201 cation of pain often requires nonverbal clues, such as the child \200\231s behavior. Pain rating scales are often helpful (see Figure 1.1). \200 Tur ning the examination into a form of play may put the child at ease and assist with the exam ination. For example, asking the child to imitate your own movements may help you gauge ran ge of motion. \200 The trappings of a clinic setting may make young children nervous, and removing a white coat or stethoscope from sight may also help place the patient at ease. 0 1 2 3 4 5 Fig. 1.1 Pain assessment in children \200\224the faces rating scale. This page intentionally left blank 11 Chapter 2 Regional musculoskeletal conditions: making a working diagnosis Introduction 12 Neck pain 14 Shoulder pain 22 Pain around the elbow 34 Wrist pain 40 Sympto ms in the hand 46 Upper limb peripheral nerve lesions 58 Thoracic back and chest pain 64 Lo w back pain and disorders in adults 70 Spinal disorders in children and adolescents 84 Pelv ic, groin, and thigh pain 92 Knee pain 104 Lower leg and foot disorders (adults) 118 Child and adolescent foot disorders 132 Corticosteroid injection therapy 142 Principles of rehabi litation 146 CHAPTER 2 Regional musculoskeletal conditions 12 Introduction This chapter provides a guide to constructing an appropriate differential diag nosis for the patient who presents with regional musculoskeletal symp- toms. It does not ma ke reference to all possible diagnoses, only to the most common. This section is divided in to discussions of the neck, upper limb (shoulder, elbow, wrist), hand, thoracolumbar spine, lower limb (pelvis, groin, thigh, knee), and foot. General considerations \200 Findings from conventional clinical examination and imaging o f the musculoskeletal system usually occur when the patient is at rest, and therefore only minima lly symptomatic. Examination in the context of function (i.e., carrying, lifting, walking, bending, etc.) is not easy, although it is arguably more appropriate. Therefore, a thorough history utilizing deep knowledge of functional anatomy is the best alternative and an inva luable way of obtaining good information about abnormal function and its causes. Time spent obtaining a detailed account of the onset of symptoms is often helpful, regardless of whether the symptoms are of recent onset, or chronic, or obvio usly associated with trauma. Patients usually have a clearer concept of injury-induced dise ase and may try to rationalize the appearance of nontrauma-related symptoms by association with an event or injury.