OXFORD MEDICAL PUBLICATIONS Oxford Desk Reference Rheumatology Oxford University Press makes no representation, express or implied, that the drug dosages in this book are correct. Readers must therefore always check the product informa- tion and clinical procedures with the most up-to-date pub- lished product information and data shee ts provided by the manufacturers and the most recent codes of conduct and safety regulation s. The authors and the publishers do not accept responsibility or legal liability for any e rrors in the text or for the misuse or misapplication of material in this work. 2 Except where otherwise stated, drug doses and recom- mendations are for the non-pregnant adult who is not breast-feeding. Oxford Desk Reference Rheumatology Richard Watts Consultant Rheumatologist Ipswich Hospital NHS Trust Ipswich and Clinical Sen ior Lecturer University of East Anglia Norwich Gavin Clunie Consultant Rheumatologist Ipswich Hospital NHS Trust Ipswich Frances Hall University Lecturer and Honorary Consultant in Rheumatology University of Camb ridge Addenbrooke s Hospital Cambridge and Tarnya Marshall Consultant Rheumatologist Norfolk and Norwich University Hospital Norwich 1 1 Great Clarendon Street, Oxford OX2 6DP 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 in Oxford New York Auckland Cape Town Dar es Salaam Hong Kong Karachi Kuala Lumpur Madrid Melbourne Mexico Cit y Nairobi New Delhi Shanghai Taipei Toronto With of \201 ces in Argentina Austria Brazil Chile Czech Republic France Greece Guatemala Hungary Italy Japan P oland Portugal Singapore South Korea Switzerland Thailand Turkey Ukraine Vietnam Oxford is a registered trade mark of Oxford University Press in the UK and in certain other countries Published in the United States by Oxford University Press Inc., New York Oxford University Press 2009 The moral rights of the author have been asserted Database right Oxford University Press (m aker) First published 2009 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, or under terms agreed with t he appropriate reprographics rights organization. Enquiries concerning reproduction outside the scope of the above should be sent to the Rights Department, Oxford University Press, a t the address above You must not circulate this book in any other binding or cover and you must impose this sam e condition on any acquirer British Library Cataloging in Publication Data Data available Library of Congress Cataloging in Publication Data Data available Typeset by Cepha Imaging Private Ltd., Bangalore, India Printed in CPI Antony Rowe, Chippen ham, Wiltshire ISBN 978 \200\2230 \200\22319 \200\223922999 \200\2234 10 9 8 7 6 5 4 3 2 1 v Preface Rheumatology covers a very broad range of conditions ranging from soft tissue rheumatology, through the in \202 ammatory arthritides to complex multi system disease. Our aim in writing this volume is to provide easily accessible and practical information in a single desk-sized book. We aim to help the specialist rheumatologist with the diagnosis and management of these problems. We emphasize the clinical presentations of these conditio ns, approaches to investigation and diagnosis, and up to date management. Treat- ment proto cols, where ever possible, are evidence-based and we provide the evidence base for the use of drug therapy. We hope that the information provided, will help the clinician in managing patients and thus reduce the impact of these diseases. We are grateful for the long suffering support of our families during the writing of this book. This page intentionally left blank vii Brief contents Detailed contents ix Abbreviations xiii Contributors xix 1 Clinical assessment of rheumatological disease 1 2 Investigation of rheumatic disease 23 3 Organ involvement in rheumatological disease 47 4 Rheumatological procedures: injection therapy 105 5 Regional musculoskeletal anatomy and conditions 117 6 Rheumatoid arthritis 197 7 The spondylarthropathies 209 8 Autoimmune connective tissue diseases 239 9 Vasculitis 313 10 Juvenile idiopathic arthritis 351 11 Pregnancy and the rheumatic diseases 365 12 Osteoarthritis and related disorders 371 13 Crystal arthritis 383 14 Bone diseases 395 15 Hereditary diseases of connective tissue 437 16 Musculoskeletal infection 445 BRIEF CONTENTS viii 17 Chronic pain 479 18 Miscellaneous diseases 491 19 DMARDs and immunosuppressive drugs 531 Index 577 ix Detailed contents 1 Clinical assessment of The elbow: regional musculoskeletal conditions 148 rheumatological disease 1 Rheumatology history taking 2 Rheumatological examination 8 Patte rns of rheumatological disease: oligoarticular pains in adults 14 The hand and wrist 152 Hand and wrist: regional musculoskeletal conditions 158 Pelvis, hip, and groin 164 Pelvis, hip and groin: regional musculoskeletal Widespread pain (in adults) 18 conditions 168 The knee 174 The knee: regional musculoskeletal 2 Investigation of rheumatic disease 23 Plain radiography 24 Ultrasound 26 Computed tomography 30 Magnetic resonance ima ging 34 Radionuclide imaging 36 Autoimmune serology 38 Synovial \202 uid analysis 42 Clin ical neurophysiology 44 conditions 178 Lower leg and foot 184 Lower leg and foot: regional musculoskeletal conditions 188 Conditions of the ankle and hindfoot 190 Conditions of the mid and forefoot 194 6 Rheumatoid arthritis 197 Guidelines for the management of rheumatoid arthritis 208 3 Organ involvement in 7 The spondylarthropathies 209 The spondylarthropathies: disease spectrum rheumatological disease 47 The eye 48 The chest 54 The heart 66 The kidney 74 The gut and h epatobiliary disease 82 The nervous system 90 The skin 98 (including undifferentiated spondylarthropathy) 210 Ankylosing spondylitis 214 Psoriatic ar thritis 220 Reactive arthritis 226 Enteropathic spondylarthropathy 230 Juvenile spondylarth ropathy/enthesitis-related arthritis 232 4 Rheumatological procedures: SAPHO (Le Syndrome Acn , Pustulose, Hyperostose, Ost ite) 236 injection therapy 105 Aspiration of joints 106 Procedure 108 8 Autoimmune connective tissue diseases 239 Systemic lupus erythematosus 240 Sj gren \200\231s syndrome 254 Systemic scle rosis 264 Antiphospholipid syndrome 274 Polymyositis 282 Dermatomyositis 290 Juvenile derma tomyositis 298 Inclusion body myositis 302 Undifferentiated (autoimmune) connective tissue 5 Regional musculoskeletal anatomy and conditions 117 The spine 118 Cervical spine: regional musculoskeletal conditions 124 Thoracic spine and chest wall: regional musculoskeletal conditions 128 Lumbar spine: region al musculoskeletal conditions 130 The shoulder 136 The shoulder girdle: regional musculoskeletal disease 306 Mixed connective tissue disease 307 Overlap syndromes 308 Eosinophilic fasciitis 310 conditions 140 The elbow 146 DETAILED CONTENTS x 9 Vasculitis 313 Introduction 314 Giant cell arteritis 318 Polymyalgia rheumatica 320 Takay asu arteritis 322 Wegener \200\231s granulomatosis 324 Churg \200\223Strauss Syndrome 326 M icroscopic polyangiitis 328 Treatment of ANCA-associated vasculitis 330 Polyarteritis nodos a 332 Cryoglobulinaemia 334 Primary central nervous system vasculitis 336 Cogan \200\231s s yndrome 338 Beh et \200\231s disease 340 Henoch \200\223Sch nlein purpura 342 Kawasaki di sease 344 Relapsing polychondritis 346 Thromboangiitis obliterans 348 Osteoporosis in children 412 Primary hyperparathyroidism 414 Paget \200\231s disease of bon e (osteodystrophia deformans) 418 Osteomalacia and rickets 422 Renal bone disease 426 Osteogenesis imperfecta 428 Miscellaneo us bone diseases 1 432 Miscellaneous bone diseases 2 434 15 Hereditary diseases of connective tissue 437 Marfan syndrome 438 Ehlers \200\223Danlos s yndrome 440 Joint hypermobility syndrome 442 16 Musculoskeletal infection 445 Practical approach to a hot swollen joint 446 Septic arthr itis 448 Gonococcal arthritis 452 Osteomyelitis 454 Soft tissue infection 458 Rheumatic fev er 462 Brucellosis 464 Lyme disease 466 Viral arthritis 468 Mycobacterial infection 472 Fun gal infection 476 10 Juvenile idiopathic arthritis 351 Juvenile idiopathic arthritis: overview 352 Oligoartic ular juvenile idiopathic arthritis 358 Rheumatoid factor (RF) negative polyarticular JIA 359 Rheumatoid factor (RF) positive polyarticular JIA 360 Systemic onset JIA 362 Juvenile PsA 364 11 Pregnancy and the rheumatic 17 Chronic pain 479 Chronic pain 480 Fibromyalgia syndrome 484 Complex regional pain syndro me type I 488 diseases 365 Pregnancy and the rheumatic diseases 366 12 Osteoarthritis and related 18 Miscellaneous diseases 491 disorders 371 Osteoarthritis 372 Osteoarthritis-related disorders 380 Acromegaly 492 Diabetes mellitus 494 Adult onset Still \200\231s disease 496 Amyloidosis 49 8 Autoin \202 ammatory syndromes 500 Hyperimmunoglobulinaemia D with periodic 13 Crystal arthritis 383 Gout 384 Calcium pyrophosphate dihydrate disease 388 The basic calcium phosphate crystals 3 92 fever syndrome 502 TNF-receptor-associated periodic syndrome 503 14 Bone diseases 395 Cryopyrin-associated periodic syndrome 504 Haemochromatosis 506 Haemoglobinopathies 508 Hem ophilia 510 Sarcoidosis 512 Panniculitis 514 Alkaptonuria 516 Post-menopausal osteoporosis 396 Therapeutics of post-menopausal osteoporosis 402 Osteoporosis in men 406 Glucocorticoid induced osteoporosis (GIO) 408 DETAILED CONTENTS xi Gaucher \200\231s disease 518 Eosinophilia-myalgia syndrome and toxic oil Gold: intramuscular (sodium aurothimalate) and oral (aurano \201 n) 546 syndrome 520 Penicillamine 550 Azathioprine 552 Mycophenolate mofetil 556 Ciclosporin (previously cyclos porin A) 558 Cyclophosphamide 562 Biologics: anti-TNF (in \202 iximab, etanercept, adalimu mab), anti-CD20 (rituximab), anti-IL-1 (anakinra), CTLA4 \200\223Ig (abatacept), and anti-I L-6R (tocilizumab) 568 Metabolic myopathies 522 Synovial osteochondromatosis 526 Pigmented villonodular synovitis 527 Bone tumors 528 19 DMARDs and immunosuppressive drugs 531 Methotrexate 532 Sulfasalazine 538 Le \202 unomide 540 Anti-ma larials (hydroxychloroquine and Index 577 chloroquine) 544 This page intentionally left blank xiii Abbreviations B2GPI 25OHD 99mTc AA AAS AAV ACA ACE ACJ ACL aCL ACLE ACR AD ABD ADHR B2 Glycoprotein I 25-hydroxyvitamin D Technetium-99m amyloid A ANCA-associated vasculitides ANCA-associated vasculitis anti-centromere antibodies angiotensin converting enzyme acromi oclavicular joint anterior cruciate ligament anticardiolipin acute cutaneous lupus erythema tosus American College of Rheumatology autosomal dominant adynamic bone disease autosomal d ominant hypophosphataemic rickets amyopathic dermatomyositis AD osteopetrosis anti-endothel ial cell antibodies atrial \201 brillation autoimmune connective tissue disease Acquired immunode \201 ciency syndrome acute lymphoblastic leukemia Alkaline phosphatase alanine tr ansaminase anti-mitochondrial antibodies adenosine monophosphate deaminase Anorexia nervosa Antinuclear antibody Anti-neutrophil cytoplasmic antibody Adult Onset Still \200\231s dise ase Aortic valve anteroposterior Antigen-presenting cell antiphospholipid Antiphospholipid Syndrome activated partial thromboplastin time autosomal recessive Ankylosing spondylitis A ssessment of Ankylosing spondylitis (group) Ankylosing Spondylitis International Federation Anterior superior iliac spine anti-streptolysin O titre aspartate transaminase Avascular n ecrosis azathioprine B cell activating factor belonging to the TNF family BASDAI Bath ankylosing spondylitis disease activity index Bath ankylosing spondylitis functional i ndex basic calcium phosphate twice daily Beh et \200\231s disease British Health Professio nals in Rheumatology benign joint hypermobility British Isles Lupus Assessment Group bone m ineral content bone mineral density body mass index blood pressure British Society for Pedi atric and Adolescent Rheumatology British Sociaty of Rheumatology broadband ultrasound atte nuation Birmingham Vasculitis Assessment Score coronary artery disease cryopyrin associated periodic syndrome Complete blood count Congestive cardiac failure cyclic citrullinated pep tide Centers for Disease Control and Prevention cystic \201 brosis calcitonin gene-relate d peptide Childhood Health Assessment Questionnaire congenital heart block Chapel Hill Cons ensus Criteria chronic infantile neurological cutaneous and articular syndrome creatine kin ase chronic kidney disease compound muscle action potential Childhood Myositis Assessment S cale carpometacarpal carpometacarpal \200\231 joint cystoids macular oedema cytomegalovirus central nervous system common peroneal nerve calcium pyrophosphate dihydrate deposition (d isease/arthritis) C-reactive protein Chronic Regional Pain Syndrome Cogan \200\231s syndrom e Churg \200\223Strauss Syndrome cutaneous small vessel vasculitis BASFI BCP bd BD BHPR BJH BILAG BMC BMD BMI BP BSPAR BSR BUA BVAS CAD CAPS CBC CCF CCP CDC ADM ADO AECA AF AICTD AIDS ALL ALP ALT AMA AMPD AN ANA ANCA AOSD AoV AP APC APL APLS APTT A R AS ASAS CF CGRP CHAQ CHB CHCC CINCA CK CKD CMAP CMAS CMC CMCJ CMO CMV CNS CPN CPPDD ASIF ASIS ASOT AST AVN AZA BAFF CRP CRPS CS CSS CSVV ABBREVIATIONS xiv EIP EMG EMS EN ENA ENT EPB ER ERA eRA ERCP extensor indicis proprius electromyography eosinophilia-myalgia syndrome erythema nodosum e xtractable nuclear antigens ear nose throat extensor pollicis brevis endoplasmic reticulum enthesitis related arthritis endothelin receptor antagonists endoscopic retrograde cholepan creatography enteropathic spondylarthropathy erythrocyte sedimentation rate end-stage renal disease end-stage renal failure European Spondylarthropathy Study Group endoscopic ultraso und European League Against Rheumatism European vasculitis Study Group femoroacetabular imp ingement full blood count familial cold in \202 ammatory condition \202 exor carpi radia lis \202 exor carpi ulnaris \201 brous dysplasia \202 exor digitorum super \201 cial is \201 broblast growth factor \202 exor hallucis longus \201 brogenesis imperfecta o ssium \201 bromyalgia familial Mediterranean fever \201 bromyalgia syndrome \201 brod ysplasia ossi \201 cans progressiva forced vital capacity gastric antral vascular ectasia Glomerular Basement Membrane glucocorticoid giant cell arteritis glomerular \201 ltration rate growth hormone glenohumeral joint gastrointestinal glucocorticoid-induced osteoporosi s glomerulonephritis gastro-oesophageal re \202 ux disease glyceryl trinitrate Health, Anx iety, Depression Score Health Assessment Questionnaire (HAQ) hepatitis B virus hydroxychlor oquine hepatitis C virus CT CTD CTPA CTS CTX CV CVA CVD CXR CYC DAD DALY DAS DBPCS DBPRCT computerized tomography connective tissue disease CT pulmonary angiogram carpal tunnel synd rome C telopeptide [Collagen] crosslinked conduction velocity Cerebrovascular accident card iovascular disease chest X-Ray cyclophosphamide diffuse alveolar damage Disability Adjusted Life Years Disease Activity Score double-blind, placebo-controlled studies double-blind, p lacebo-controlled randomized trial diffuse cutaneous systemic sclerosis diethylstilboestrol dual energy X-Ray absorptiometry diffuse intravascular coagulation diffuse in \201 ltrati ve lymphocytosis syndrome distal interphalangeal joint diffuse idiopathic skeletal Hyperost osis discoid lupus erythematous dermatomyositis disease-modifying ant-rheumatic drug distal motor latency disease modifying OA drugs Dimercapto succinic acid deoxyribonucleic acid di ffuse Noxious Inhibitory Control D-penicillamine drug-related lupus distal radio-ulnar join t Dilute Russell Viper Venom Test Diethylene triamine penta acetic acid deep vein thrombosi s dual X-ray absorptiometry digital X-ray radiogrammetry ethylidene bis[tryptophan] Epstein \200\223Barr virus electrocardiogram echocardiogram European Consensus Lupus Activity Meas urement extensor carpi radialis brevis extensor carpi radialis longus extensor carpi ulnari s extensor digitorum communis extensor digiti minimi Ehlers \200\223Danlos syndrome electro encephalography endosteal hyperostosis ESpA ESR ESRD ESRF ESSG dcSSc DES DEXA DIC DILS DIPJ DISH DLE DM DMARD DML DMOADS DMSA DNA DNIC D-PCA DRL DRUJ DRVV T DTPA DVT DXA DXR EBT EBV ECG ECHO ECLAM EU EULAR EUVAS FAI FBC FCAS FCR FCU FD FDS FGF FHL FIO FM FMF FMS FOP FVC GAVE GBM GC GCA G FR GH GHJ GI GIO GN GORD GTN HADS HAQ HBV HCQ HCV ECRB ECRL ECU EDC EDM EDS EEG EH ABBREVIATIONS xv HDL HELLP high density lipoprotein haemolysis, elevated liver enzymes, low platelets homogenistic aci d oxidase hyperimmunoglobulin D with periodic fever syndrome human immunode \201 ciency vi rus human lymphocyte antigen hereditary multiple hyperostosis high molecular weight hypertr ophic osteoarthropathy Hypertrophic pulmonary osteoarthropathy high resolution high resolut ion CT health-related quality of life hormone replacement therapy Henoch \200\223Sch nlein purpura Herpes Simplex Virus human T cell lymphotropic virus haemolytic uraemic syndrome i n \202 ammatory bowel disease inclusion body myositis in \202 ammatory back pain intercel lular adhesion molecule International Classi \201 cation of Disease localized cutaneous Sy stemic Sclerosis intensive care unit infective endocarditis immuno \202 uorescence interfe ron insulin related growth factor ischaemic heart disease immuno \202 uorescence idiopathi c intracranial hypertension idiopathic juvenile osteoporosis interleukin International Leag ue of Associations for Rheumatology interstitial lung disease International Myositis Assess ment and Clinical Studies International normalized ratio interphalangeal iliotibial band In trauterine growth retardation intravenous intravenous Immunoglobulin G juvenile ankylosing spondylitis juvenile dermatomyositis joint hypermobility syndrome juvenile idiopathic arthr itis juvenile idiopathic in \202 ammatory myopathies JPsA JSpA JVP KCO KD KIR KP LA LAiP LCL LCPD lcSSc LDH LDL LE LEF LETM LFT LIP LMWH LOS LP LRP LSp LV LVEF LVH MAA MAGIC juvenile psoriatic arthritis juvenile spondylarthropathy jugular venous pressure Carbon mon oxide transfer co-ef \201 cient Kawasaki disease killer cell immunoglobulin-like receptor keratic precipitates lupus anticoagulant lupus activity index in pregnancy lateral collater al ligament Legg \200\223Calv \200\223Perthes Disease limited cutaneous systemic sclerosi s lactate dehydrogenase low density lipoprotein lupus erythematosus le \202 unomide longit udinally extensive transverse myelitis liver function test lymphocytic interstitial pneumon ia low molecular weight heparin lower oesophageal lumbar puncture lipoprotein receptor-rela ted protein lumbar spine left ventricle left ventricular ejection fraction left ventricular hypertrophy myositis-associated autoantibodies mucosal and genital ulceration with in \202 amed cartilage microangiopathic haemolytic anemia macrophage activation syndrome Maast richt Ankylosing Spondylitis Enthesitis Score microscopy, culture and (antibiotic) sensitiv ities medial collateral ligament macrophage chemotactic protein metacarpophalangeal joint m otor conduction velocity multidetector multidetector CT Myositis Disease Activity Assessmen t Tool methyl diphosphonate modi \201 cation of diet in renal disease multi-disciplinary t eam myalgic encephalopathy matrix extracellular phosphoglycoprotein major histocompatibilit y complex myocardial infarction macrophage inhibitory factor mycophenylate mofetil matrix m etalloproteinase Hgo HIDS HIV HLA HME HMW HOA HPOA HR HRCT HRQOL HRT HSP HSV HTLV HUS IBD IBM IBP ICAM ICD lcSSc ICU IE IF IFN IGF IHD IIF IIH IJO IL ILAR MAHA MAS MASES MC&S MCL MCP MCPJ MCV MD MDCT MDAAT MDP MDRD MDT ME MEPE MHC MI MIF MMF MMP ILD IMACS INR IP ITB IUGR IV IVIG JAS JDM JHS JIA JIIM ABBREVIATIONS xvi MMSE MMT MPA MPO MR MRC MRA MRI MRS MRSA mini mental state examination manual muscle testing microscopic Polyangiitis myeloperoxidas e magnetic resonance Medical Research Council MR angiography magnetic resonance imaging mag netic resonance spectroscopy methacillin-resistant Staphylococcus aureus MR venography myos itis speci \201 c autoantibodies minor salivary gland mid-stream urine Mycobacterium tuber culosis metatarsophalangeal joint methotrexate Muckle Well syndrome nerve conduction studie s nuclear factor B non- Hodgkins lymphoma National Institute for Clinical Excellence natu ral killer (cells) N-methyl-D-aspartate receptor number needed to offend number needed to t reat neonatal onset multi-system in \202 ammatory disease non-steroidal anti-in \202 amma tory drug non-speci \201 c interstitial pneumonia N-teldopeptide [Collagen] Crosslinks ost eoarthritis ocular coherence tomography once daily osteitis \201 brosa osteogenesis imper fecta osteochondral lesion of the talus Online Mendelian Inheritance in Man osteonecrosis o f the jaw organizing pneumonia ossi \201 cation of the posterior longitudinal ligament Odd s ratio Osgood \200\223Schlatter \200\231s syndrome occupational therapy primary angiitis o f the CNS posterior atlantodental interval pulmonary arterial hypertension polyarteritis no dosa primary biliary cirrhosis posterior cruciate ligament Primary central nervous systemic vasculitis PCP PCR PCS PD PDB PDD PDGF PE PEFR PET PF PFK PFTs PHPT PIN PIPJ PITF PLM PM PML pneumocystis pneumonia polymerase chain reaction placebo-controlled studies peritoneal dial ysis Paget \200\231s disease of bone progressive diaphyseal dysplasia platelet-derived grow th factor Pulmonary embolism peak expiratory \202 ow rate positron emission tomography pl antar fasciitis phosphofructokinase pulmonary function tests primary hyperparathyroidism po sterior interosseus nerve proximal interphalangeal joint posteroinferior tibio \201 bular polarized light microscopy Polymyositis progressive multifocal leukoencephalopathy polymorp honuclear postmenopausal osteoporosis polymyalgia rheumatica psoriatic onycho pachydermo pe riostitis pulsus paradoxus Proton pump inhibitor proteinase 3 placebo-controlled randomized trial psoriasis prostate-speci \201 c antigen psoriatic arthritis posterior superior ilia c spine post-streptococcal reactive arthritis primary Sj gren \200\231s syndrome Prothromb in time parathyroid hormone pigmented villonuclear synovitis quantitative computerized tomo graphy four times daily quality and outcome framework quantitative (Heel) ultrasound rheuma toid arthritis receptor activator of nuclear factor kB regulators of complement activation Royal College of Physicians randomized controlled trial Randomized double-blind, placebo-co ntrolled trial reactive arthritis MRV MSA MSG MSU MTB MTPJ MTX MWS NCS NF B NHL NICE NK NMDA NNO NNT NOMID PMN PMO PMR POPP PP PPI PR3 PCR Ps PSA PsA PSIS PSRA pSS PT PTH PVNS QCT qds QOF QUS RA RAN K RCA RCP RCT RDBPCT NSAID NSIP NTX OA OCT od OF OI OLT OMIM ONJ OP OPLL OR OSS OT PACNS PADI PAH PAN PBC PCL PCNSV ReA REMS RF RNP rheumatoid factor ribonucleoprotein ABBREVIATIONS xvii TFCC TFL TGF-B TIMP TIN TLC TLCO TMJ TNF TOE TPHA TPMT TR TRAPS triangular \201 brocartilage tensor of fascia lata transforming growth factor beta tissue inhibitors of MMPs tubulointerstitial nephritis total lung capacity transfer capacity temp oromandibular joint tumor necrosis factor transoesophagal echocardiogram T. palladium haema gglutination assay thiopurine methyl transferase tricuspid regurgitation tumor necrosis fac tor receptor- associated periodic syndrome thyroid stimulating hormone thoracic spine trans thoracic echocardiogram thrombotic thrombocytopenic purpura transthyretin urea and electrol ytes undifferentiated autoimmune connective tissue disease undifferentiated connective tiss ue disease usual interstitial pneumonia ultrasound undifferentiated Spondylarthropathy Ultr a sound scan urinary tract infection visual analog scale video-assisted thoracoscopic surgi cal Vasculitis Damage Index vitamin D Receptor vascular endothelial growth factor vertical fracture vertebral fracture analysis voltage-gated potassium channel very long-chain acyl-C oA dehydrogenases ventricular tachycardia Varicella zoster virus von Willebrand factor whit e blood cell Wegener \200\231s Granulomatosis Womens \200\231 Health Initiative World Healt h Organization X-linked hypophosphataemia yak pox Zebra Fish Disease ROD RP renal osteodystrophy Raynaud \200\231s phenomenon/relapsing polychondritis re \202 ex symp athetic dystrophy renal tubular acidosis Right ventricular systolic pressure serum amyloid A subacute cutaneous lupus serum amyloid P sensory action potentials (LC) Syndrome Acn , P ustulose, Hyperostose, Ost ite Subacute bacterial endocarditis subcutaneous slipped capita l femoral epiphysis sternoclavicular joint sub-acute cutaneous lupus erythematosus standard deviation seronegative enthesopathy arthritis selective estrogen receptor modulators secon dary hyperparathyroidism sacroiliac joint scapho-lunate advanced collapse SLE Lupus Activit y Measure systemic lupus erythematosus SLE Disease Activity Index Systemic Lupus Internatio nal Collaborating Clinics scaphoid non-union and advanced collapse speed of sound spondylar thropathy Spondyloarthritis Research Consortium (Canada) single photon emission computerize d tomography strontium ranelate signal recognition peptide Sj gren \200\231s syndrome Sj gren \200\231s syndrome A systemic sclerosis selective serotonin receptor antagonists supra spinatus tendon secondary Sj gren \200\231s syndrome Superior vena cava sulphasalazine Tem poral Artery thromboangiitis obliterans trichloroethylene three times daily transcutaneous electrical nerve stimulation RSD RTA RVSP SAA SACLE SaP SAP SAPHO SBE SC SCFE SCJ SCLE SD SEA SERM SHPT SIJ SLAC SLAM SLE SLEDAI SLICC TSH TSp TTE TTP TTR U&Es UAICTD UCTD UIP US USpA USS UTI VAS VATS VDI VDR VEGF VF VFA VGKC VLCAD VT vZV vWF WBC WG WHI WHO XLH YP ZCD SNAC SOS SpA SPARCC SPECT SR SRP SS SSA SSc SSRI SST sSS SVC SZP TA TAO TCE tds TENS This page intentionally left blank xix Contributors Dr Ademola Adejuwon Registrar in Trauma and Orthopedic Surgery University College Hospital London Dr Richard Goodwin Consultant Radiologist Norfolk and Norwich University NHS Foundation Tru st Norwich Dr Kate Armon Consultant Pediatrician Norfolk and Norwich University Hospital NHS Foundatio n Trust Norwich Mr Fares S Haddad Consultant Orthopedic Surgeon University College Hospital London Dr Frances Hall University Lecturer and Honorary Consultant in Rheumatology University of C ambridge School of Clinical Medicine Cambridge Dr Fraser Birrell Senior Lecturer in Rheumatology University of Newcastle upon Tyne Newcast le Dr Julian Blake Consultant Neurophysiologist Norfolk and Norwich University Foundation NHS Trust Mr James Hopkinson-Woolley Consultant Orthopedic Surgeon University of Cambridge Addenbrook e \200\231s Hospital Cambridge Dr Marian L Burr Academic Clinical Fellow in Rheumatology ARC Epidemiology Unit University of Manchester Manchester Mr Maxim Horwitz Specialist Registrar in Trauma and Orthopedics Royal National Orthopedic H ospital Stanmore Mr Ben Burton Consultant Opthalmologist James Paget University Hospital NHS Foundation Trus t Norfolk Dr Sujith Konan Clinical and Research Fellow University College Hospital London Dr Gavin Clunie Consultant Rheumatologist Ipswich Hospital NHS Trust Ipswich Dr Mark Lillicrap Consultant Rheumatologist Addenbrooke \200\231s Hospital Cambridge Dr Carlos Cobiella Consultant Orthopedic Surgeon The Shoulder Unit Hospital of St John & St Elizabeth London Mr Lennel Lutchman Consultant Orthopedic Surgeon Norfolk and Norwich University Hospital No rwich Dr Dave Dutka University Lecturer and Honorary Consultant Cardiologist Addenbrooke \200\231 s Hospital Cambridge Dr Tarnya Marshall Consultant Rheumatologist Norfolk and Norwich University Hospital Norwic h Dr Maninder Mundae Research Fellow in Rheumatology Addenbrooke \200\231s Hospital Cambridge Dr Amel Ginawi Specialist Registrar in Rheumatology Addenbrooke \200\231s Hospital Cambridg e Dr Andrew J K \226st r Consultant Rheumatologist School of Clinical Medicine University o f Cambridge Cambridge Dr Mark Good \201 eld Consultant Dermatologist Department of Dermatology Leeds General In \201 rmary Leeds CONTRIBUTORS xx Dr Helen Parfrey University Lecturer and Honorary Consultant in Medicine University of Camb ridge School of Clinical Medicine Cambridge Dr Michael Walsh Clinical Fellow in Lupus and Vasculitis Addenbrooke \200\231s Hospital Cam bridge Dr Richard Watts Consultant Rheumatologist Ipswich Hospital NHS Trust Ipswich and Clinical Senior Lecturer University of East Anglia Dr Shin-Jae Rhee Orthopedic Registrar University College Hospital London Dr Nick Shenker Consultant Rheumatologist Addenbrooke \200\231s Hospital Cambridge Dr Jeremy Woodward Consultant Gastroenterologist Addenbrooke \200\231s Hospital Cambridge Mr Elliot Sorene Consultant Orthopedic Surgeon University College London Hospitals London Dr Michael Zandi Neurology Research Fellow Addenbrooke \200\231s Hospital Cambridge 1 Chapter 1 Clinical assessment of rheumatological disease Rheumatology history taking 2 Rheumatological examination 8 Patterns of rheumatological dis ease: oligoarticular pains in adults 14 Widespread pain (in adults) 18 RHEUMATOLOGY HISTORY TAKING 2 Rheumatology history taking There is a wide spectrum of musculoskeletal and other disease that can present with musculo skeletal symptoms. Given the nature of those symptoms and the context in which they are pre sented, however, there are some principles of history taking worth highlighting here. The f ollowing issues are discussed. \200 \200 \200 \200 \200 \200 \200 behavior, since young children may not be able to verbalize their pain. \200 Children may be fractious and irritable, or quiet and withdrawn; they may be off their food and have disturbed nights. If they can verbalize they often localize pain poorly. On exami nation, a child may not admit to pain, but will withdraw the limb or appear anxious when th e painful area is examined. Observing both the child \200\231s and parent \200\231s facial expression during an examination is very important. Although a description of the quality o f pain is beyond young children, often an indication of its severity can be obtained throug h asking them to indicate on a diagram how they feel about it (e.g. the Faces Rating Scale) . Older children are often able to score pain from 0 to 10. The complaint of pain. The complaint of stiffness. Multiple musculoskeletal symptoms. Rheum atology Questionnaire tools. Additional (non-musculoskeletal) symptoms. Reporting styles. H istory from others: assent and necessity. Pain Pain is the most common musculoskeletal symptom. It is de \201 ned by its subjective description, which may vary depending on its physical or biological cause, the patient \200 \231s understanding of it, its impact on function, and the emotional and behavioral respons e it invokes. Pain is also often \200\230colored \200\231 by cultural, linguistic, and rel igious differences and beliefs. Therefore, pain is not merely an unpleasant sensation; it i s also an \200\230emotional change \200\231. The experience is different for every individ ual. In children and adolescents the evaluation of pain is sometimes compli- cated further by the interacting in \202 uences of the experi- ence of pain within the family, school, a nd peer group. \200 Table 1.1 Descriptors of pain that may be relevant in revealing the in \202 uence of non-o rganic/amplifying \200\230interpretative \200\231 factors on the reporting of pain Organic Non-organic or pain ampli \201 cation Pounding Flickering Jumping Shooting Pricking Lancinating Sharp Lacerating Adults usually accurately localize pain, although there are some situations worth noting in rheumatic disease, where pain can be poorly localized. Pain may be localized, but caused d irectly or indirectly (referred) by a distant lesion, e.g.: \200 Pinching Crushing Hot Searing Tender Splitting Nagging Torturing interscapular pain caused by postural/mechanical problems in the cervical spine; pain from shoulder lesions referred to the area around deltoid insertion in the humerus; lumbosacral pain referred to the area around the greater trochanters; hip joint pain referred (often wi thout pain in the groin) down the thigh, even to the knee. Spreading Piercing Annoying Unbearable Tiring Exhausting Fearful Terrifying Tight Tearing Pain caused by neurological abnormalities, ischaemic pain, and pain referred from viscera a re less easy for the patient to visualize or express, and the history may be given with var ied interpretations. Bone pain is generally constant, despite movement or change in posture . In comparison, muscular, synovial, ligament, or tendon pain tends to alter with movement. Fracture, tumor, and metabolic bone disease are all possible causes. Such constant, local, sleep-disturbing pain should always be considered potentially sinister and investigated. I t is worth noting that certain descriptors of pain (at least in English-speaking patients) have consistently been associated with the in \202 uence of non-organic modi \201 ers of pain, the pain experience, and it \200\231s reporting. These descriptors can be found in a number of pain evaluation questionnaire tools (Table 1.1.) Stiffness Stiffness can be an indication of oedema and/or in \202 ammation. \200 Stiffne ss, however, is not speci \201 c for in \202 ammatory mus- culoskeletal lesions. Musculos keletal stiffness is assumed to be due to accumulation of \202 uid in structures thus oed ema resulting from traumatic or degenerative lesions may produce stiffness in theory. In \202 ammatory-induced stiffness in any musculoskeletal structure often improves with moveme nt. Neurological stiffness (increased tone) can mimic stiff- ness from musculoskeletal lesi ons. It typically occurs in insidiously developing myelopathy, early Parkinson \200\231s Di sease and some other extra-pyramidal disorders, for example. Some patients with in \202 am matory diseases complain about stiffness without pain [e.g. in some patients with ankylosin g spondylitis, in early or mild rheumatoid arthritis (RA)]. Pain in children The assessment of pain in young children is often dif \201 cult. The pres ence of pain may have to be surmised from CHAPTER 1 Clinical assessment of rheumatological disease 3 Multiple musculoskeletal symptoms Multiple symptoms can occur simultaneously or seemingly l inked over time (patterns). Either may be due to a single systemic condition or multiple se parate musculoskeletal lesions. The assessment of symptoms can be complicated by how long p atients have had symptoms (recall bias). The likelihood that each scenario exists depends o n a number of variables including: \200 Case 1.1. A 40-year-old woman presents with 6 months achy pain and stiffness of \201 nger s, both hindfeet and lower legs. No back pain is present. She has had fatigue for 5 years ( diagnosed with ME), mild myositis was diagnosed 2 years previously on clinical grounds and she has had some rashes over the past 10 years, dry, gritty eyes, and xerostomia. Are the s ymptoms from past history linked or separate? The differential diagnosis needs to be wide a nd history taking extensive. Primary care physicians may not have referred her to specialis ts for some of her previous problems. Previous clinical diagnoses may not have been made co gnisant of the possibility that autoim- mune disease can cause periodic symptoms in differe nt body systems. Consideration of her having either an autoimmune connective tissue disease (e.g. lupus or primary Sj gren \200\231s disease), Lyme disease, or chronic sarcoid must be given. Distinction of current symptoms from chronic pain, evidence for pathology at site s of symptoms, and blood tests will be important in deter- mining diagnosis [e.g. compared with \201 bromyalgia (FM)]. The background prevalence of any condition in the reference population. The con \201 gurat ion of the healthcare system particularly the ease or dif \201 culty with which patients c an access specialist care. Individual factors, which may in \202 uence presentation to doc tors (e.g. ethnic or socio-economic factors). Patterns of musculoskeletal symptoms Patterns of musculoskeletal symptoms are recognized in association with certain rheumatic conditions. Patterns are not usually speci \201 c, but help in forming a working diagnosis. Patterns can be interpreted from either the simultano us presentation of symptoms given their distri- bution or from a presumed link between symp toms over time (common originsee Case 1.1). The latter, of course, is the most dif \201 cu lt situation to unravel. Some examples of patterns include: \200 Case 1.2. An 80-year-old lady known to have type 2 dia- betes (20-year history) complicated by CKD3 (renal im- pairment) and hypothyroidism presents with bilateral shoulder and wrist pain, pain in 2nd and 3rd \201 ngers, swollen knees, and collapsed (valgus) painful swol len hind- feet. She is used to being ill, does not present to doctors early, and many sympt oms are long-standing. The time of onset of each symptom cannot be determined accurately. L ate-onset RA, gout, or CPPDD arthritis might be single causes of all her symptoms; however, it would be wise to initially consider some combination of separate, but common conditions , which could be contributing to her predicament. Conditions might include: osteoar- thriti s (e.g. knee), carpal tunnel syndrome, Charcot ar- thritis or diabetic osteolysis (e.g. sub talar joints), adhe- sive capsulitis (diabetes-associated), and subacromial impingement (e. g. 2nd to a rotator cuff lesion). The situation may be complicated by any diabetic cheirar- thropathy in her hands, peripheral neuropathy or cardiac failure (swollen ankles), and her ability to tolerate symptoms (in \202 uencing the reporting of symptoms) perhaps influenc ed by fatigue from diabetes and (undertreated) hypothyroidism! Simultanous symmetrical small joint in \202 ammation in the hands and wrists [e.g. RA or c alcium pyrophosphate dihydrate disease (CPPDD) polyarthritis or pseudo-RA psoriatic arthrit is]. Simultanous asymmetric, lower limb pains associated with in \202 ammatory low back pa in 4 weeks after Salmonella infection (e.g. reactive arthritis). A history of acute, but al so previously recurrent monoar- ticular, peripheral joint, symptoms over many years (e.g. g out or the pseudogout form of CPPDD arthritis/ disease). Recurrent \200\230tennis elbow \200\231 and episodic inflammatory back pain symptoms \200\223 both for many years, recalc itrant bilateral plantar fasciitis 5 years previously in a patient known to get recurrent b outs of psoriasis presenting now with a single swollen knee (psoriatic arthritis).