oxford desk reference - rheumatology

OXFORD MEDICAL PUBLICATIONS
Oxford Desk Reference
Rheumatology
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Readers must therefore always check the product informa-
tion and clinical procedures with the most up-to-date pub-
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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 Senior 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 Cambridge
Addenbrooke's Hospital
Cambridge
and
Tarnya Marshall
Consultant Rheumatologist
Norfolk and Norwich University Hospital
Norwich
1
1
Great Clarendon Street, Oxford OX2 6DP
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Typeset by Cepha Imaging Private Ltd., Bangalore, India
Printed in CPI Antony Rowe,
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ISBN 978–0–19–922999–4
10 9 8 7 6 5 4 3 2 1
v
Rheumatology covers a very broad range of conditions ranging from soft tissue rheumatology,
through the infl 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
conditions, approaches to investigation and diagnosis, and up to date management. Treat-
ment protocols, 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.

Preface
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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
viii
17 Chronic pain
479
18 Miscellaneous diseases
491
19 DMARDs and immunosuppressive drugs
531
Index 577
BRIEF CONTENTS
ix
1 Clinical assessment of
rheumatological disease
1
Rheumatology history taking 2
Rheumatological examination 8
Patterns of rheumatological disease:
oligoarticular pains in adults
14
Widespread pain (in adults) 18
2 Investigation of rheumatic
disease

23
Plain radiography 24
Ultrasound 26
Computed tomography 30
Magnetic resonance imaging 34
Radionuclide imaging 36
Autoimmune serology 38
Synovial fl uid analysis 42
Clinical neurophysiology 44
3 Organ involvement in
rheumatological disease
47
The eye 48
The chest 54
The heart 66
The kidney 74
The gut and hepatobiliary disease 82
The nervous system 90
The skin 98
4 Rheumatological procedures:
injection therapy
105
Aspiration of joints 106
Procedure 108
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: regional musculoskeletal
conditions
130
The shoulder 136
The shoulder girdle: regional musculoskeletal
conditions
140
The elbow 146
The elbow: regional musculoskeletal
conditions
148
The hand and wrist 152
Hand and wrist: regional musculoskeletal
conditions
158
Pelvis, hip, and groin 164
Pelvis, hip and groin: regional musculoskeletal
conditions
168
The knee 174
The knee: regional musculoskeletal
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
7 The spondylarthropathies 209
The spondylarthropathies: disease spectrum
(including undifferentiated
spondylarthropathy)
210
Ankylosing spondylitis 214
Psoriatic arthritis 220
Reactive arthritis 226
Enteropathic spondylarthropathy 230
Juvenile spondylarthropathy/enthesitis-related
arthritis
232
SAPHO (Le Syndrome Acné, Pustulose,
Hyperostose, Ostéite)
236
8 Autoimmune connective tissue
diseases
239
Systemic lupus erythematosus 240
Sjögren’s syndrome 254
Systemic sclerosis 264
Antiphospholipid syndrome 274
Polymyositis 282

Dermatomyositis 290
Juvenile dermatomyositis 298
Inclusion body myositis 302
Undifferentiated (autoimmune) connective tissue
disease
306
Mixed connective tissue disease 307
Overlap syndromes 308
Eosinophilic fasciitis 310
Detailed contents
x
9 Vasculitis 313
Introduction 314
Giant cell arteritis 318
Polymyalgia rheumatica 320
Takayasu arteritis 322
Wegener’s granulomatosis 324
Churg–Strauss Syndrome 326
Microscopic polyangiitis 328
Treatment of ANCA-associated vasculitis 330
Polyarteritis nodosa 332
Cryoglobulinaemia 334
Primary central nervous system vasculitis 336
Cogan’s syndrome 338
Behçet’s disease 340
Henoch–Schönlein purpura 342
Kawasaki disease 344
Relapsing polychondritis 346
Thromboangiitis obliterans 348
10 Juvenile idiopathic arthritis 351

Juvenile idiopathic arthritis: overview 352
Oligoarticular 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
diseases
365
Pregnancy and the rheumatic diseases 366
12 Osteoarthritis and related
disorders
371
Osteoarthritis 372
Osteoarthritis-related disorders 380
13 Crystal arthritis 383
Gout 384
Calcium pyrophosphate dihydrate disease 388
The basic calcium phosphate crystals 392
14 Bone diseases 395
Post-menopausal osteoporosis 396
Therapeutics of post-menopausal
osteoporosis
402
Osteoporosis in men 406
Glucocorticoid induced osteoporosis

(GIO)
408
Osteoporosis in children 412
Primary hyperparathyroidism 414
Paget’s disease of bone (osteodystrophia
deformans)
418
Osteomalacia and rickets 422
Renal bone disease 426
Osteogenesis imperfecta 428
Miscellaneous bone diseases 1 432
Miscellaneous bone diseases 2 434
15 Hereditary diseases of
connective tissue
437
Marfan syndrome 438
Ehlers–Danlos syndrome 440
Joint hypermobility syndrome 442
16 Musculoskeletal infection 445
Practical approach to a hot swollen joint 446
Septic arthritis 448
Gonococcal arthritis 452
Osteomyelitis 454
Soft tissue infection 458
Rheumatic fever 462
Brucellosis 464
Lyme disease 466
Viral arthritis 468
Mycobacterial infection 472
Fungal infection 476

17 Chronic pain 479
Chronic pain 480
Fibromyalgia syndrome 484
Complex regional pain syndrome type I 488
18 Miscellaneous diseases 491
Acromegaly 492
Diabetes mellitus 494
Adult onset Still’s disease 496
Amyloidosis 498
Autoinfl ammatory syndromes 500
Hyperimmunoglobulinaemia D with periodic
fever syndrome
502
TNF-receptor-associated periodic
syndrome
503
Cryopyrin-associated periodic syndrome 504
Haemochromatosis 506
Haemoglobinopathies 508
Haemophilia 510
Sarcoidosis 512
Panniculitis 514
Alkaptonuria 516
DETAILED CONTENTS
xi
Gaucher’s disease 518
Eosinophilia-myalgia syndrome and toxic oil
syndrome
520
Metabolic myopathies 522

Synovial osteochondromatosis 526
Pigmented villonodular synovitis 527
Bone tumours 528
19 DMARDs and
immunosuppressive drugs
531
Methotrexate 532
Sulfasalazine 538
Lefl unomide 540
Anti-malarials (hydroxychloroquine and
chloroquine)
544
Gold: intramuscular (sodium aurothimalate)
and oral (auranofi n)
546
Penicillamine 550
Azathioprine 552
Mycophenolate mofetil 556
Ciclosporin (previously cyclosporin A) 558
Cyclophosphamide 562
Biologics: anti-TNF (infl iximab, etanercept,
adalimumab), anti-CD20 (rituximab),
anti-IL-1 (anakinra), CTLA4–Ig (abatacept),
and anti-IL-6R (tocilizumab)
568
Index 577
DETAILED CONTENTS
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xiii
B2GPI B2 Glycoprotein I

25
OHD 25-hydroxyvitamin D
99m
Tc Technetium-99m
AA amyloid A
AAS ANCA-associated vasculitides
AAV ANCA-associated vasculitis
ACA anti-centromere antibodies
ACE angiotensin converting enzyme
ACJ acromioclavicular joint
ACL anterior cruciate ligament
aCL anticardiolipin
ACLE acute cutaneous lupus erythematosus
ACR American College of Rheumatology
AD autosomal dominant
ABD adynamic bone disease
ADHR autosomal dominant hypophosphataemic
rickets
ADM amyopathic dermatomyositis
ADO AD osteopetrosis
AECA anti-endothelial cell antibodies
AF atrial fi brillation
AICTD autoimmune connective tissue disease
AIDS Acquired immunodefi ciency syndrome
ALL acute lymphoblastic leukaemia
ALP Alkaline phosphatase
ALT alanine transaminase
AMA anti-mitochondrial antibodies
AMPD adenosine monophosphate deaminase
AN Anorexia nervosa

ANA Antinuclear antibody
ANCA Anti-neutrophil cytoplasmic antibody
AOSD Adult Onset Still’s disease
AoV Aortic valve
AP anteroposterior
APC Antigen-presenting cell
APL antiphospholipid
APLS Antiphospholipid Syndrome
APTT activated partial thromboplastin time
AR autosomal recessive
AS Ankylosing spondylitis
ASAS Assessment of Ankylosing spondylitis
(group)
ASIF Ankylosing Spondylitis International
Federation
ASIS Anterior superior iliac spine
ASOT anti-streptolysin O titre
AST aspartate transaminase
AVN Avascular necrosis
AZA azathioprine
BAFF B cell activating factor belonging to the
TNF family
BASDAI Bath ankylosing spondylitis disease activity
index
BASFI Bath ankylosing spondylitis functional
index
BCP basic calcium phosphate
bd twice daily
BD Behçet’s disease
BHPR British Health Professionals in

Rheumatology
BJH benign joint hypermobility
BILAG British Isles Lupus Assessment Group
BMC bone mineral content
BMD bone mineral density
BMI body mass index
BP blood pressure
BSPAR British Society for Paediatric and
Adolescent Rheumatology
BSR British Sociaty of Rheumatology
BUA broadband ultrasound attenuation
BVAS Birmingham Vasculitis Assessment Score
CAD coronary artery disease
CAPS cryopyrin associated periodic syndrome
CBC Complete blood count
CCF Congestive cardiac failure
CCP cyclic citrullinated peptide
CDC Centres for Disease Control and
Prevention
CF cystic fi brosis
CGRP calcitonin gene-related peptide
CHAQ Childhood Health Assessment
Questionnaire
CHB congenital heart block
CHCC Chapel Hill Consensus Criteria
CINCA chronic infantile neurological cutaneous
and articular syndrome
CK creatine kinase
CKD chronic kidney disease
CMAP compound muscle action potential

CMAS Childhood Myositis Assessment Scale
CMC carpometacarpal
CMCJ carpometacarpal’ joint
CMO cystoids macular oedema
CMV cytomegalovirus
CNS central nervous system
CPN common peroneal nerve
CPPDD calcium pyrophosphate dihydrate
deposition (disease/arthritis)
CRP C-reactive protein
CRPS Chronic Regional Pain Syndrome
CS Cogan’s syndrome
CSS Churg–Strauss Syndrome
CSVV cutaneous small vessel vasculitis
Abbreviations
xiv
CT computerized tomography
CTD connective tissue disease
CTPA CT pulmonary angiogram
CTS carpal tunnel syndrome
CTX C telopeptide [Collagen] crosslinked
CV conduction velocity
CVA Cerebrovascular accident
CVD cardiovascular disease
CXR chest X-Ray
CYC cyclophosphamide
DAD diffuse alveolar damage
DALY Disability Adjusted Life Years
DAS Disease Activity Score
DBPCS double-blind, placebo-controlled studies

DBPRCT double-blind, placebo-controlled
randomised trial
dcSSc diffuse cutaneous systemic sclerosis
DES diethylstilboestrol
DEXA dual energy X-Ray absorptiometry
DIC diffuse intravascular coagulation
DILS diffuse infi ltrative lymphocytosis syndrome
DIPJ distal interphalangeal joint
DISH diffuse idiopathic skeletal Hyperostosis
DLE discoid lupus erythematous
DM dermatomyositis
DMARD disease-modifying ant-rheumatic drug
DML distal motor latency
DMOADS disease modifying OA drugs
DMSA Dimercapto succinic acid
DNA deoxyribonucleic acid
DNIC diffuse Noxious Inhibitory Control
D-PCA D-penicillamine
DRL drug-related lupus
DRUJ distal radio-ulnar joint
DRVVT Dilute Russell Viper Venom Test
DTPA Diethylene triamine penta acetic acid
DVT deep vein thrombosis
DXA dual X-ray absorptiometry
DXR digital X-ray radiogrammetry
EBT ethylidene bis[tryptophan]
EBV Epstein–Barr virus
ECG electrocardiogram
ECHO echocardiogram
ECLAM European Consensus Lupus Activity

Measurement
ECRB extensor carpi radialis brevis
ECRL extensor carpi radialis longus
ECU extensor carpi ulnaris
EDC extensor digitorum communis
EDM extensor digiti minimi
EDS Ehlers–Danlos syndrome
EEG electroencephalography
EH endosteal hyperostosis
EIP extensor indicis proprius
EMG electromyography
EMS eosinophilia-myalgia syndrome
EN erythema nodosum
ENA extractable nuclear antigens
ENT ear nose throat
EPB extensor pollicis brevis
ER endoplasmic reticulum
ERA enthesitis related arthritis
eRA endothelin receptor antagonists
ERCP endoscopic retrograde
cholepancreatography
ESpA enteropathic spondylarthropathy
ESR erythrocyte sedimentation rate
ESRD end-stage renal disease
ESRF end-stage renal failure
ESSG European Spondylarthropathy Study
Group
EU endoscopic ultrasound
EULAR European League Against Rheumatism
EUVAS European vasculitis Study Group

FAI femoroacetabular impingement
FBC full blood count
FCAS familial cold infl ammatory condition
FCR fl exor carpi radialis
FCU fl exor carpi ulnaris
FD fi brous dysplasia
FDS fl exor digitorum superfi cialis
FGF fi broblast growth factor
FHL fl exor hallucis longus
FIO fi brogenesis imperfecta ossium
FM fi bromyalgia
FMF familial Mediterranean fever
FMS fi bromyalgia syndrome
FOP fi brodysplasia ossifi cans progressiva
FVC forced vital capacity
GAVE gastric antral vascular ectasia
GBM Glomerular Basement Membrane
GC glucocorticoid
GCA giant cell arteritis
GFR glomerular fi ltration rate
GH growth hormone
GHJ glenohumeral joint
GI gastrointestinal
GIO glucocorticoid-induced osteoporosis
GN glomerulonephritis
GORD gastro-oesophageal refl ux disease
GTN glyceryl trinitrate
HADS Health, Anxiety, Depression Score
HAQ Health Assessment Questionnaire (HAQ)
HBV hepatitis B virus

HCQ hydroxychloroquine
HCV hepatitis C virus
ABBREVIATIONS
xv
HDL high density lipoprotein
HELLP haemolysis, elevated

liver enzymes, low
platelets
Hgo homogenistic acid oxidase
HIDS hyperimmunoglobulin D with periodic
fever syndrome
HIV human immunodefi ciency virus
HLA human lymphocyte antigen
HME hereditary multiple hyperostosis
HMW high molecular weight
HOA hypertrophic osteoarthropathy
HPOA Hypertrophic pulmonary osteoarthropathy
HR high resolution
HRCT high resolution CT
HRQOL health-related quality of life
HRT hormone replacement therapy
HSP Henoch–Schönlein purpura
HSV Herpes Simplex Virus
HTLV human T cell lymphotropic virus
HUS haemolytic uraemic syndrome
IBD infl ammatory bowel disease
IBM inclusion body myositis
IBP infl ammatory back pain
ICAM intercellular adhesion molecule

ICD International Classifi cation of Disease
lcSSc localised cutaneous Systemic Sclerosis
ICU intensive care unit
IE infective endocarditis
IF immunofl uorescence
IFN interferon
IGF insulin related growth factor
IHD ischaemic heart disease
IIF immunofl uorescence
IIH idiopathic intracranial hypertension
IJO idiopathic juvenile osteoporosis
IL interleukin
ILAR International League of Associations for
Rheumatology
ILD interstitial lung disease
IMACS International Myositis Assessment and
Clinical Studies
INR International normalized ratio
IP interphalangeal
ITB iliotibial band
IUGR Intrauterine growth retardation
IV intravenous
IVIG intravenous Immunoglobulin G
JAS juvenile ankylosing spondylitis
JDM juvenile dermatomyositis
JHS joint hypermobility syndrome
JIA juvenile idiopathic arthritis
JIIM juvenile idiopathic infl ammatory
myopathies
JPsA juvenile psoriatic arthritis

JSpA juvenile spondylarthropathy
JVP jugular venous pressure
KCO Carbon monoxide transfer co-effi cient
KD Kawasaki disease
KIR killer cell immunoglobulin-like receptor
KP keratic precipitates
LA lupus anticoagulant
LAiP lupus activity index in pregnancy
LCL lateral collateral ligament
LCPD Legg–Calvé–Perthes Disease
lcSSc limited cutaneous systemic sclerosis
LDH lactate dehydrogenase
LDL low density lipoprotein
LE lupus erythematosus
LEF lefl unomide
LETM longitudinally extensive transverse myelitis
LFT liver function test
LIP lymphocytic interstitial pneumonia
LMWH low molecular weight heparin
LOS lower oesophageal
LP lumbar puncture
LRP lipoprotein receptor-related protein
LSp lumbar spine
LV left ventricle
LVEF left ventricular ejection fraction
LVH left ventricular hypertrophy
MAA myositis-associated autoantibodies
MAGIC mucosal and genital ulceration with
infl amed cartilage
MAHA microangiopathic haemolytic anaemia

MAS macrophage activation syndrome
MASES Maastricht Ankylosing Spondylitis
Enthesitis Score
MC&S microscopy, culture and (antibiotic)
sensitivities
MCL medial collateral ligament
MCP macrophage chemotactic protein
MCPJ metacarpophalangeal joint
MCV motor conduction velocity
MD multidetector
MDCT multidetector CT
MDAAT Myositis Disease Activity Assessment Tool
MDP methyl diphosphonate
MDRD modifi cation of diet in renal disease
MDT multi-disciplinary team
ME myalgic encephalopathy
MEPE matrix extracellular phosphoglycoprotein
MHC major histocompatibility complex
MI myocardial infarction
MIF macrophage inhibitory factor
MMF mycophenylate mofetil
MMP matrix metalloproteinase
ABBREVIATIONS
xvi
MMSE mini mental state examination
MMT manual muscle testing
MPA microscopic Polyangiitis
MPO myeloperoxidase
MR magnetic resonance
MRC Medical Research Council

MRA MR angiography
MRI magnetic resonance imaging
MRS magnetic resonance spectroscopy
MRSA methacillin-resistant Staphylococcus
aureus
MRV MR venography
MSA myositis specifi c autoantibodies
MSG minor salivary gland
MSU mid-stream urine
MTB Mycobacterium tuberculosis
MTPJ metatarsophalangeal joint
MTX methotrexate
MWS Muckle Well syndrome
NCS nerve conduction studies
NFκB nuclear factor κB
NHL non- Hodgkins lymphoma
NICE National Institute for Clinical Excellence
NK natural killer (cells)
NMDA N-methyl-
D-aspartate receptor
NNO number needed to offend
NNT number needed to treat
NOMID neonatal onset multi-system infl ammatory
disease
NSAID non-steroidal anti-infl ammatory drug
NSIP non-specifi c interstitial pneumonia
NTX N-teldopeptide [Collagen] Crosslinks
OA osteoarthritis
OCT ocular coherence tomography
od once daily

OF osteitis fi brosa
OI osteogenesis imperfecta
OLT osteochondral lesion of the talus
OMIM Online Mendelian Inheritance in Man
ONJ osteonecrosis of the jaw
OP organizing pneumonia
OPLL ossifi cation of the posterior longitudinal
ligament
OR Odds ratio
OSS Osgood–Schlatter’s syndrome
OT occupational therapy
PACNS primary angiitis of the CNS
PADI posterior atlantodental interval
PAH pulmonary arterial hypertension
PAN polyarteritis nodosa
PBC primary biliary cirrhosis
PCL posterior cruciate ligament
PCNSV Primary central nervous systemic vasculitis
PCP pneumocystis pneumonia
PCR polymerase chain reaction
PCS placebo-controlled studies
PD peritoneal dialysis
PDB Paget’s disease of bone
PDD progressive diaphyseal dysplasia
PDGF platelet-derived growth factor
PE Pulmonary embolism
PEFR peak expiratory fl ow rate
PET positron emission tomography
PF plantar fasciitis
PFK phosphofructokinase

PFTs pulmonary function tests
PHPT primary hyperparathyroidism
PIN posterior interosseus nerve
PIPJ proximal interphalangeal joint
PITF posteroinferior tibiofi bular
PLM polarized light microscopy
PM Polymyositis
PML progressive multifocal
leukoencephalopathy
PMN polymorphonuclear
PMO postmenopausal osteoporosis
PMR polymyalgia rheumatica
POPP psoriatic onycho pachydermo periostitis
PP pulsus paradoxus
PPI Proton pump inhibitor
PR3 proteinase 3
PCR placebo-controlled randomised trial
Ps psoriasis
PSA prostate-specifi c antigen
PsA psoriatic arthritis
PSIS posterior superior iliac spine
PSRA post-streptococcal reactive arthritis
pSS primary Sjögren’s syndrome
PT Prothrombin time
PTH parathyroid hormone
PVNS pigmented villonuclear synovitis
QCT quantitative computerised tomography
qds four times daily
QOF quality and outcome framework
QUS quantitative (Heel) ultrasound

RA rheumatoid arthritis
RANK receptor activator of nuclear factor kB
RCA regulators of complement activation
RCP Royal College of Physicians
RCT randomized controlled trial
RDBPCT Randomized double-blind,
placebo-controlled trial
ReA reactive arthritis
REMS
RF rheumatoid factor
RNP ribonucleoprotein
ABBREVIATIONS
xvii
ROD renal osteodystrophy
RP Raynaud’s phenomenon/relapsing
polychondritis
RSD refl ex sympathetic dystrophy
RTA renal tubular acidosis
RVSP Right ventricular systolic pressure
SAA serum amyloid A
SACLE subacute cutaneous lupus
SaP serum amyloid P
SAP sensory action potentials
SAPHO (LC) Syndrome Acné, Pustulose,
Hyperostose, Ostéite
SBE Subacute bacterial endocarditis
SC subcutaneous
SCFE slipped capital femoral epiphysis
SCJ sternoclavicular joint
SCLE sub-acute cutaneous lupus erythematosus

SD standard deviation
SEA seronegative enthesopathy arthritis
SERM selective estrogen receptor modulators
SHPT secondary hyperparathyroidism
SIJ sacroiliac joint
SLAC scapho-lunate advanced collapse
SLAM SLE Lupus Activity Measure
SLE systemic lupus erythematosus
SLEDAI SLE Disease Activity Index
SLICC Systemic Lupus International
Collaborating Clinics
SNAC scaphoid non-union and advanced collapse
SOS speed of sound
SpA spondylarthropathy
SPARCC Spondyloarthritis Research Consortium
(Canada)
SPECT single photon emission computerised
tomography
SR strontium ranelate
SRP signal recognition peptide
SS Sjögren’s syndrome
SSA Sjögren’s syndrome A
SSc systemic sclerosis
SSRI selective serotonin receptor antagonists
SST supraspinatus tendon
sSS secondary Sjögren’s syndrome
SVC Superior vena cava
SZP sulphasalazine
TA Temporal Artery
TAO thromboangiitis obliterans

TCE trichloroethylene
tds three times daily
TENS transcutaneous electrical nerve
stimulation
TFCC triangular fi brocartilage
TFL tensor of fascia lata
TGF-B transforming growth factor beta
TIMP tissue inhibitors of MMPs
TIN tubulointerstitial nephritis
TLC total lung capacity
TLCO transfer capacity
TMJ temporomandibular joint
TNF tumour necrosis factor
TOE transoesophagal echocardiogram
TPHA T. palladium haemagglutination assay
TPMT thiopurine methyl transferase
TR tricuspid regurgitation
TRAPS tumour necrosis factor receptor-
associated periodic syndrome
TSH thyroid stimulating hormone
TSp thoracic spine
TTE transthoracic echocardiogram
TTP thrombotic thrombocytopenic purpura
TTR transthyretin
U&Es urea and electrolytes
UAICTD undifferentiated autoimmune connective
tissue disease
UCTD undifferentiated connective tissue disease
UIP usual interstitial pneumonia
US ultrasound

USpA undifferentiated Spondylarthropathy
USS Ultra sound scan
UTI urinary tract infection
VAS visual analogue scale
VATS video-assisted thoracoscopic surgical
VDI Vasculitis Damage Index
VDR vitamin D Receptor
VEGF vascular endothelial growth factor
VF vertical fracture
VFA vertebral fracture analysis
VGKC voltage-gated potassium channel
VLCAD very long-chain acyl-CoA dehydrogenases
VT ventricular tachycardia
vZV Varicella zoster virus
vWF von Willebrand factor
WBC white blood cell
WG Wegener’s Granulomatosis
WHI Womens’ Health Initiative
WHO World Health Organisation
XLH X-linked hypophosphataemia
YP yak pox
ZCD Zebra Fish Disease
ABBREVIATIONS
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xix
Dr Ademola Adejuwon
Registrar in Trauma and Orthopaedic
Surgery
University College Hospital
London

Dr Kate Armon
Consultant Paediatrician
Norfolk and Norwich University Hospital
NHS Foundation Trust
Norwich
Dr Fraser Birrell
Senior Lecturer in Rheumatology
University of Newcastle upon Tyne
Newcastle
Dr Julian Blake
Consultant Neurophysiologist
Norfolk and Norwich University
Foundation NHS Trust
Dr Marian L Burr
Academic Clinical Fellow in Rheumatology
ARC Epidemiology Unit
University of Manchester
Manchester
Mr Ben Burton
Consultant Opthalmologist
James Paget University Hospital NHS
Foundation Trust
Norfolk
Dr Gavin Clunie
Consultant Rheumatologist
Ipswich Hospital NHS Trust
Ipswich
Dr Carlos Cobiella
Consultant Orthopaedic Surgeon
The Shoulder Unit

Hospital of St John & St Elizabeth
London
Dr Dave Dutka
University Lecturer and Honorary
Consultant Cardiologist
Addenbrooke’s Hospital
Cambridge
Dr Amel Ginawi
Specialist Registrar in Rheumatology
Addenbrooke’s Hospital
Cambridge
Dr Mark Goodfi eld
Consultant Dermatologist
Department of Dermatology
Leeds General Infi rmary
Leeds
Dr Richard Goodwin
Consultant Radiologist
Norfolk and Norwich University NHS
Foundation Trust
Norwich
Mr Fares S Haddad
Consultant Orthopaedic Surgeon
University College Hospital
London
Dr Frances Hall
University Lecturer and Honorary
Consultant in Rheumatology
University of Cambridge School of
Clinical Medicine

Cambridge
Mr James Hopkinson-Woolley
Consultant Orthopaedic Surgeon
University of Cambridge
Addenbrooke’s Hospital
Cambridge
Mr Maxim Horwitz
Specialist Registrar in
Trauma and Orthopaedics
Royal National Orthopaedic Hospital
Stanmore
Dr Sujith Konan
Clinical and Research Fellow
University College Hospital
London
Dr Mark Lillicrap
Consultant Rheumatologist
Addenbrooke’s Hospital
Cambridge
Mr Lennel Lutchman
Consultant Orthopaedic Surgeon
Norfolk and Norwich University Hospital
Norwich
Dr Tarnya Marshall
Consultant Rheumatologist
Norfolk and Norwich University Hospital
Norwich
Dr Maninder Mundae
Research Fellow in Rheumatology
Addenbrooke’s Hospital

Cambridge
Dr Andrew J K Östör
Consultant Rheumatologist
School of Clinical Medicine
University of Cambridge
Cambridge
Contributors
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Dr Helen Parfrey
University Lecturer and Honorary
Consultant in Medicine
University of Cambridge School of
Clinical Medicine
Cambridge
Dr Shin-Jae Rhee
Orthopaedic Registrar
University College Hospital
London
Dr Nick Shenker
Consultant Rheumatologist
Addenbrooke’s Hospital
Cambridge
Mr Elliot Sorene
Consultant Orthopaedic Surgeon
University College London Hospitals
London
Dr Michael Walsh
Clinical Fellow in Lupus and Vasculitis
Addenbrooke’s Hospital
Cambridge

Dr Richard Watts
Consultant Rheumatologist
Ipswich Hospital NHS Trust
Ipswich
and Clinical Senior Lecturer
University of East Anglia
Dr Jeremy Woodward
Consultant Gastroenterologist
Addenbrooke’s Hospital
Cambridge
Dr Michael Zandi
Neurology Research Fellow
Addenbrooke’s Hospital
Cambridge
CONTRIBUTORS
Rheumatology history taking 2
Rheumatological examination 8
Patterns of rheumatological disease: oligoarticular pains in adults 14
Widespread pain (in adults) 18
Clinical assessment of
rheumatological disease
1
Chapter 1
2
Rheumatology history taking
There is a wide spectrum of musculoskeletal and other
disease that can present with musculoskeletal symptoms.
Given the nature of those symptoms and the context
in which they are presented, however, there are some
principles of history taking worth highlighting here. The

following issues are discussed.
The complaint of pain.
The complaint of stiffness.
Multiple musculoskeletal symptoms.
Rheumatology Questionnaire tools.
Additional (non-musculoskeletal) symptoms.
Reporting styles.
History from others: assent and necessity.
Pain
Pain is the most common musculoskeletal symptom. It is
defi ned by its subjective description, which may vary
depending on its physical or biological cause, the patient’s
understanding of it, its impact on function, and the
emotional and behavioural response it invokes. Pain is
also often ‘coloured’ by cultural, linguistic, and religious
differences and beliefs. Therefore, pain is not merely an
unpleasant sensation; it is also an ‘emotional change’. The
experience is different for every individual. In children and
adolescents the evaluation of pain is sometimes compli-
cated further by the interacting infl uences of the experi-
ence of pain within the family, school, and peer group.
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 directly or indirectly
(referred) by a distant lesion, e.g.:
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 without pain in the
groin) down the thigh, even to the knee.
Pain caused by neurological abnormalities, ischaemic
pain, and pain referred from viscera are less easy for the
patient to visualize or express, and the history may be
given with varied 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, tumour, and metabolic bone disease are all
possible causes. Such constant, local, sleep-disturbing
pain should always be considered potentially sinister and
investigated.
It is worth noting that certain descriptors of pain (at
least in English-speaking patients) have consistently been
associated with the infl uence of non-organic modifi ers
of pain, the pain experience, and it’s reporting. These
descriptors can be found in a number of pain evaluation
questionnaire tools (Table 1.1.)
Pain in children
The assessment of pain in young children is often diffi cult.
The presence of pain may have to be surmised from
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behaviour, since young children may not be able to verbalize
their pain.
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 examination, a child may not admit to pain, but will
withdraw the limb or appear anxious when the painful
area is examined. Observing both the child’s and
parent’s facial expression during an examination is very
important.
Although a description of the quality of pain is beyond
young children, often an indication of its severity can be
obtained through 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.
Stiffness
Stiffness can be an indication of oedema and/or infl ammation.
Stiffness, however, is not specifi c for infl ammatory mus-
culoskeletal lesions. Musculoskeletal stiffness is assumed

to be due to accumulation of fl uid in structures thus
oedema resulting from traumatic or degenerative lesions
may produce stiffness in theory.
Infl ammatory-induced stiffness in any musculoskeletal
structure often improves with movement.
Neurological stiffness (increased tone) can mimic stiff-
ness from musculoskeletal lesions. It typically occurs in
insidiously developing myelopathy, early Parkinson’s
Disease and some other extra-pyramidal disorders, for
example.
Some patients with infl ammatory diseases complain
about stiffness without pain [e.g. in some patients with
ankylosing spondylitis, in early or mild rheumatoid
arthritis (RA)].
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Table 1.1 Descriptors of pain that may be relevant in
revealing the infl uence of non-organic/amplifying
‘interpretative’ factors on the reporting of pain
Organic Non-organic or pain amplifi cation
Pounding Flickering
Jumping Shooting
Pricking Lancinating
Sharp Lacerating
Pinching Crushing

Hot Searing
Tender Splitting
Nagging Torturing
Spreading Piercing
Annoying Unbearable
Tiring Exhausting
Fearful Terrifying
Tight Tearing
RHEUMATOLOGY HISTORY TAKING
3CHAPTER 1 Clinical assessment of rheumatological disease
Multiple musculoskeletal symptoms
Multiple symptoms can occur simultaneously or seemingly
linked over time (patterns). Either may be due to a single
systemic condition or multiple separate musculoskeletal
lesions. The assessment of symptoms can be complicated
by how long patients have had symptoms (recall bias). The
likelihood that each scenario exists depends on a number
of variables including:
The background prevalence of any condition in the
reference population.
The confi guration of the healthcare system particularly
the ease or diffi culty with which patients can access
specialist care.
Individual factors, which may infl uence presentation to
doctors (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 specifi c, but help in forming a working
diagnosis. Patterns can be interpreted from either the

simultaneous presentation of symptoms given their distri-
bution or from a presumed link between symptoms over
time (common originsee Case 1.1). The latter, of course, is
the most diffi cult situation to unravel. Some examples of
patterns include:
Simultaneous symmetrical small joint infl ammation in
the hands and wrists [e.g. RA or calcium pyrophosphate
dihydrate disease (CPPDD) polyarthritis or pseudo-RA
psoriatic arthritis].
Simultaneous asymmetric, lower limb pains associated
with infl ammatory low back pain 4 weeks after Salmonella
infection (e.g. reactive arthritis).
A history of acute, but also previously recurrent monoar-
ticular, peripheral joint, symptoms over many years
(e.g. gout or the pseudogout form of CPPDD arthritis/
disease).
Recurrent ‘tennis elbow’ and episodic inflammatory
back pain symptoms – both for many years, recalcitrant
bilateral plantar fasciitis 5 years previously in a patient
known to get recurrent bouts of psoriasis presenting
now with a single swollen knee (psoriatic arthritis).
Separate co-prevalent conditions
Musculoskeletal conditions are common, particularly in
the elderly. It is common for the elderly to present to
specialists infrequently and/or late, and have a consider-
able amount of symptoms. In the elderly, both over- and
under-diagnosis of unifying conditions are quite easy
mistakes to make given the problems in assessment
(see Case 1.2). Common ‘degenerative’ and other lesions
giving (either chronic variable or persistent) symptoms

are:
Osteoarthritis.
Crystal-induced infl ammation or accelerated degeneration
of joints and other structures.
Rotator cuff degeneration/arthropathy.
Radicular symptoms (usually nerve root exit foramen
stenosis in spinal canal lateral recess).
Frank lumbar spinal stenosis.
Mild myopathy (e.g. chronic hypovitaminosis-D).
Various contributors to back pain [osteoarthritis
(OA) facet joints, degenerative disc disease, osteoporotic
fracture].
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Evaluating a history of illness in children and
young people
A musculoskeletal disorder will affect a growing, developing
child differently to an adult. Children with infl ammatory

disorders, as in adults, will be affected systemically by their
illness. Non-musculoskeletal symptoms associated with
musculoskeletal disease can occur in children. In addition,
specifi c aspects of a child’s life should be addressed.
Development. Is the young child meeting normal devel-
opmental milestones? Is walking delayed or has it
regressed in any way? Are they keeping up with their
peers in toddler groups or nursery?
Appetite and growth. Is the child eating normally and
gaining weight? Do they have a record of weight and
height with them (red book in the UK)? Does it show
good growth or a falling through the centiles? Older children
are not often weighed, but you can ask if the clothes
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Case 1.1. A 40-year-old woman presents with 6 months
achy pain and stiffness of fi ngers, 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 symptoms from past history linked or separate?
The differential diagnosis needs to be wide and history
taking extensive. Primary care physicians may not have
referred her to specialists for some of her previous
problems. Previous clinical diagnoses may not have
been made cognisant of the possibility that autoim-
mune disease can cause periodic symptoms in different
body systems. Consideration of her having either an
autoimmune connective tissue disease (e.g. lupus or

primary Sjögren’s disease), Lyme disease, or chronic
sarcoid must be given. Distinction of current symptoms
from chronic pain, evidence for pathology at sites of
symptoms, and blood tests will be important in deter-
mining diagnosis [e.g. compared with fi bromyalgia (FM)].
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 fi ngers,
swollen knees, and collapsed (valgus) painful swollen hind-
feet. She is used to being ill, does not present to doctors
early, and many symptoms are long-standing. The time of
onset of each symptom cannot be determined accurately.
Late-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-
thritis (e.g. knee), carpal tunnel syndrome, Charcot ar-
thritis or diabetic osteolysis (e.g. subtalar 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 (infl uencing the reporting of symptoms)
perhaps influenced by fatigue from diabetes and
(undertreated) hypothyroidism!
4
have become loose. Also, is there any change in bowel or

bladder function?
Energy levels. Young children who are normally
described as ‘… into everything …’, or ‘… you can’t take
your eyes off her for a minute …’ may become ‘… well
behaved …’, and ‘… now plays quietly in one place…’
Establish, therefore, if the child is abnormally fatigued?
Is there weakness? The things the child used to do
independently like brushing hair, cutting up their own
food, may be lost.
Hobbies. What do they enjoy doing? Have they had to
stop doing any activities? Is there anything they want to
do, but can’t?
School. How much school has been missed? Are they
keeping up with their peers at school, both in terms of
physical activity and academically? Is there any deteriora-
tion in handwriting?
Vision. Has there been any deterioration in vision?
Those <10–11 years old often do not notice if the sight
in one eye has deteriorated.
Rheumatology Questionnaire tools
Most rheumatologists use some questionnaire tools to
assess different aspects of disease in certain (validated)
situations. Tools can be used in clinical practice or, in
certain examples, to grade outcome in research.
Measuring disability: questionnaire tools
Many questionnaire tools exist that are used to score
disability in specifi c diseases. Usually, a tool has been devel-
oped to measure disability in a specifi c population for a
specifi c reason. Some tools have been well validated for
that purpose. It is also true that disability tools get adopted

to use in other scenarios in the same disease (e.g. early as
opposed to late arthritis or in different countries or
cultures), but also adopted/applied in other diseases. The
degree to which a tool is validated in situations other than
which it was devised for (and by implication applicable to),
is variable (examples listed below).
Health Assessment Questionnaire (HAQ)
Self-assessment questionnaire originally developed and
validated in hospital populations of RA patients to measure
functional disability.
Easy and quick to complete for patients, the scoring is
weighted and a little complicated to summarize for
assessors.
Has been extensively validated in different scenarios in
RA populations over the last 25 years.
Although responsive to change (usually deterioration) in
RA when used extensively some years ago, the tool’s
ability to respond to less overt changes in functional
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ability, either in disease deterioration or improvement
with treatment has been questioned.
Other tools for RA. Some generic measures do include
assessment of features of disability in RA and have been

applied as such (Stanford Health Assessment
Questionnaire, Nottingham Health Profile, Short
Form-36).
WOMAC
The Western Ontario and McMaster Universities
Osteoarthritis Index (WOMAC) has been used to assess
disability in OA since 1982.
WOMAC utilizes a 24-question proforma including
domains on pain, disability, and stiffness in regard of knee
and hip OA.
For its purpose WOMAC has been extensively validated
in clinical practice and research settings, is quickly com-
pleted, is reliable and responsive to change. It has been
translated into >60 language forms.
The most recent version of WOMAC (WOMAC™
3.1 Index) is a joint-targeted version of the index.
Bath Ankylosing Spondylitis Functional Index (BASFI)
Devised over 15 years ago, this self-assessed questionnaire
has been validated in measuring Ankylosing spondylitis
(AS) disability in different populations and in early and late
disease. Its strength is its simplicity.
BASFI is done by the patient. There are 10 questions and
responses are on a visual analogue scale.
Eight questions cover AS-relevant functional issues and
2 questions refer to overall effects of disability.
BASFI has not been extensively validated over the long-
term in the context of immunotherapy for AS. There is
some concern that, with the short-term variation of
effects from anti-TNFA therapies, BASFI may be over-
responsive on a single assessment.

Alternative functional indices for AS include The
Dougados Functional Index; HAQ-S (HAQ adapted for
spondylarthropathies).
The Child Health Assessment Questionnaire (CHAQ)
The CHAQ is a disease-specifi c measure of functional
status that comprises two indices, disability, and discom-
fort. Both indices focus on physical function. Disability is
assessed in eight areas with a total of 30 items with diffi culty
rated 0–3. Pain is measured on a 100 mm visual analogue
scale.
The reliability and validity of the tool are excellent.
It takes an average 10 min to complete, and can be
completed either by a parent or the older child, since
the two raters correlate well.
The CHAQ is now commonly used in juvenile idiopathic
arthritis (JIA) randomized control trials (RCTs) and is
helpful in clinic to monitor response to treatments.
Additional (non-musculoskeletal) symptoms
When are non-musculoskeletal symptoms relevant
to making (or not making) a diagnosis of a musculos-
keletal condition (see Table 1.2)? The diagnostician will
need to:
Have a broad knowledge base of general medical and
musculoskeletal conditions.
Have an in-depth understanding of systemic conditions
with musculoskeletal features (see p 47–104).
Be aware of the potential of some chronic conditions to
have relapsing features.
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Some questionnaire tools used in assessing
rheumatological disease
Health Activity Questionnaire.
Short form 36 (SF36).
Bath Index Questionnaires (AS).
Quality of Life Questionnaires (RAQoL, ASQoL
etc.).
Hospital Anxiety and Depression Scale (HADS).
Child Health Assessment Questionnaire (CHAQ).
For more detail on disease-specifi c assessment tools, see relevant
chapter on that disease.
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RHEUMATOLOGY HISTORY TAKING