Professional Documents
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Documentm 9373
Documentm 9373
Oxford Handbook of
Rheumatology
Published and forthcoming Oxford Handbooks
Gavin Clunie
Consultant Rheumatologist and Metabolic Bone Physician,
Addenbrooke’s Hospital, Cambridge, UK
Nick Wilkinson
Consultant Paediatric and Adolescent Rheumatologist,
Evelina London Children’s Hospital, London, UK
Elena Nikiphorou
Consultant Rheumatologist, Whittington Hospital;
Clinical Researcher, Academic Rheumatology Department,
King’s College London, UK
Deepak Jadon
Consultant Rheumatologist and Director of the
Rheumatology Research Unit,
Addenbrooke’s Hospital, Cambridge, UK
1
1
Great Clarendon Street, Oxford, OX2 6DP,
United Kingdom
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First Edition published in 2002
Second Edition published in 2006
Third Edition published in 2011
Fourth Edition published in 2018
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v
Foreword
Preface
Acknowledgements
The authors would like to acknowledge the work of the co-founder author-
editor of the textbook Dr Alan Hakim for his contribution, whom together
with Dr Clunie, devised and wrote the book from the first edition and co-
author-editor Dr Inam Haq (who joined for the 2nd and 3rd editions), in
helping to establish the Oxford Handbook of Rheumatology as the market
leader small textbook for rheumatology.
ix
Contents
Contributors xi
Symbols and abbreviations xii
Part II T
he clinical features and management
of rheumatic diseases
5 Rheumatoid arthritis 243
6 Osteoarthritis 265
7 Crystal-induced musculoskeletal disease 277
8 The spondyloarthritides including
psoriatic arthritis 293
9 Juvenile idiopathic arthritis 321
10 Systemic lupus erythematosus 343
11 Antiphospholipid syndrome 375
12 Sjögren’s syndrome 389
13 Systemic sclerosis and related disorders 401
14 Idiopathic inflammatory myopathies including
polymyositis and dermatomyositis 425
x CONTENTS
Index 729
xi
Contributors
E cross-reference
α alpha
β beta
i increased
d decreased
n normal
> greater than
< less than
7 approximately
∴ therefore
AAV ANCA-associated vasculitis
ABA abatacept
AC adhesive capsulitis
ACA anticentromere antibody
ACE angiotensin-converting enzyme
AChA acrodermatitis chronicum atrophicans
AC( J) acromioclavicular (joint)
ACL anticardiolipin
ACPA anticitrullinated peptide antibody(-ies)
ACR American College of Rheumatology
AD autosomal dominant
ADM abductor digiti minimi
AFF atypical femoral fracture
AICTD autoimmune connective tissue disease
AIS autoinflammatory syndrome
AKI acute kidney injury
ALNT anterolateral neospinothalamic tract
ALP alkaline phosphatase
ALT alanine transaminase
AMA amyloid A
AML amyloid L
ANA antinuclear antibody
ANCA antineutrophil cytoplasmic antibody
Anti-β2GP1 β2 glycoprotein-1
anti-
AOSD adult-onset Still’s disease
AP anteroposterior
SYMBOLS AND ABBREVIATIONS xiii
HMG-COA 3-hydroxy-3-methyl-glutaryl-coenzyme A
HPOA hypertrophic pulmonary osteoarthropathy
HPT hyperparathyroidism
HRT hormone replacement therapy
HSCT haematopoietic stem cell transplantation
HSP Henoch–Schönlein purpura
HUS haemolytic uraemic syndrome
HTLV human T-cell leukaemia virus
IA intra-articular
IBD inflammatory bowel disease
IBM inclusion-body myositis
ICD implantable cardioverter defibrillator
IFN interferon
IgG4-RD immunoglobulin G4-related disease
IGRA interferon gamma release assay
IIM idiopathic inflammatory myopathy
IL interleukin
ILAR International League of Associations for Rheumatology
ILD interstitial lung disease
IM intramuscular(ly)
IMM idiopathic inflammatory myopathy
INR international normalized ratio
IP interphalangeal
ISCD International Society of Clinical Densitometry
ISG International Study Group
ISN International Society for Nephrology
ITB iliotibial band
IV intravenous(ly)
IVDU intravenous drug user
IVIg intravenous immunoglobulin
JAK Janus kinase
JAS juvenile ankylosing spondylitis
JCA juvenile chronic arthritis
JDM juvenile dermatomyositis
JIA juvenile idiopathic arthritis
JIIM juvenile idiopathic inflammatory arthritis
JIO juvenile idiopathic osteoporosis
JPM juvenile polymyositis
JPsA juvenile psoriatic arthritis
JRA juvenile rheumatoid arthritis
xviii SYMBOLS AND ABBREVIATIONS
MPO myeloperoxidase
MR magnetic resonance
MRA magnetic resonance angiography
MRI magnetic resonance imaging
MSA myositis-specific autoantibodies
MSK musculoskeletal
MSU monosodium urate
MTP(J) metatarsophalangeal (joint)
MTX methotrexate
MUA manipulation under anaesthesia
MVA mevalonic aciduria
MVK mevalonate kinase
MWS Muckle–Wells syndrome
MYOACT myositis disease activity index
MYODAM myositis damage index
NAI non-accidental injury
NCS nerve conduction study
NICE National Institute for Health and Care Excellence (UK)
NLE neonatal lupus erythematosus
NLRs NOD-like receptors
NMS neuromuscular scoliosis
NO nitrous oxide
NOAC novel oral anticoagulant
NOD nucleotide-binding oligomerization domain
NOMID neonatal-onset multisystem inflammatory disease
NSAID non-steroidal anti-inflammatory drug
NSF nephrogenic systemic fibrosis
OA osteoarthritis
OI osteogenesis imperfecta
OMIN Online Mendelian Inheritance in Man
ONFH osteonecrosis of the femoral head
OO osteoid osteoma
OT occupational therapist
PAH pulmonary artery hypertension
PAMPS pathogen-associated molecular patterns
PAN polyarteritis nodosum
PAPA pyogenic arthritis, pyoderma gangrenosum, and acne
PBC primary biliary cirrhosis
PCR polymerase chain reaction
PDB Paget’s disease of bone
xx SYMBOLS AND ABBREVIATIONS
RTX rituximab
sACE serum angiotensin converting enzyme
SAI subacromial impingement
SAA serum amyloid A
SADAI Simplified Disease Activity Index
SAPHO synovitis, acne, palmoplantar pustulosis, hyperostosis,
aseptic osteomyelitis (syndrome)
SARA sexually acquired reactive arthritis
SC subcutaneous
SC(J) sternoclavicular (joint)
Scl systemic scleroderma
SCS spinal cord stimulation
SD standard deviation
sDMARD synthetic disease-modifying antirheumatic drug
SERM selective oestrogen receptor modulator
SHPT secondary hyperparathyroidism
SI( J) sacroiliac (joint)
SIP Sickness Impact Profile
SLE systemic lupus erythematosus
SLEDAI Systemic Lupus Erythematosus Disease Activity Index
SLICC Systemic Lupus International Collaborating criteria
SNRI serotonin-norepinephrine re-uptake inhibitors
SoJIA systemic-onset juvenile idiopathic arthritis
SpA spondyloarthritis
SRC scleroderma renal crisis
SRP signal recognition peptide
SS Sjögren’s syndrome
SScl systemic sclerosis
SSRI selective serotonin reuptake inhibitor
SSZ sulfasalazine
STIR short tau inversion recovery
SUA serum uric acid
SUFE slipped upper femoral epiphysis
T thoracic (e.g. T5 is the fifth thoracic vertebra)
TA Takayasu arteritis
TB tuberculosis
TCZ tocilizumab
TENS transcutaneous electrical nerve stimulation
TFT thyroid function test
TGF transferring growth factor
xxii SYMBOLS AND ABBREVIATIONS
The presentation
of rheumatic
disease
Chapter 1 3
Evaluating rheumatological
and musculoskeletal
symptoms
Introduction 4
Musculoskeletal pain in adults 6
Elicited pain on examination in adults 10
Other presenting symptoms in adults 12
The adult gait, arms, legs, spine (GALS) screening
examination 14
Pain assessment in children and adolescents 20
Limp and gait concerns in children and adolescents 22
Pyrexia, fatigue and unexplained acute-phase response
in children and adolescents 23
The paediatric GALS screen 24
4 Chapter 1 Evaluating MSK symptoms
Introduction
Adults and children can present with musculoskeletal (MSK), inflamma-
tory, and autoimmune diseases in varied ways. Symptoms can be simple
and focal, such as regional pain, or general and non-specific, often in the
context of a generalized process such as fever or fatigue. The following are
important points in assessing the time, type, and nature of presentation:
• Why someone has presented at a particular time.
• What is the impact of symptoms, emotionally and functionally.
• The individual’s perceptions, fears, or cultural references that might
modify (amplify or suppress) expression of the symptoms.
• What fears, beliefs, and factors might present a barrier to effective
medical engagement.
• The same pathological processes might present variably at different
ages: broadly speaking, the young, adults, and the elderly.
In this chapter, the assessment of symptoms has been separated into two
parts. First, the assessment of symptoms in adults and second, the patterns
of disease presentation in children and adolescents.
Introduction 5
6 Chapter 1 Evaluating MSK symptoms
S IR JOHN had written to his steward to tell me that Mr. Barrie had
left the Established Church; the steward showed me the letter; it
was a compound of amazement, sympathy, and anger.
Scarcely had he finished, when Bell, evidently in a state of great
excitement, came in and said, “Eh, Mr. Martin, it’s ower true; we’ll
hae to flit! Mr. Barrie’s gi’en up the kirk, and he has nae richt noo to
the manse. I canna tell exactly when we’ll be putten out, but the
mistress says we’ll no’ be allowed to bide lang. I wadna like to be the
Government that put Mr. Barrie out o’ house and ha’. Some
Mordecai will rise ere lang to gi’e us relief, or some Nehemiah to
speak to the king and queen about the desolation and affliction and
reproach. Them that comes to pit us out will maybe find an enemy in
the garden, like Ahab in Naboth’s vineyard. What’s the world comin’
till?”
Sir John’s steward knew Bell well, and tried to soothe her by saying,
“Don’t bother yourself. If Mr. Barrie adhered to his resolution to leave
the Established Church, no one would presume to hurry him out of
the manse; at all events, he could have it until a successor was
appointed.”
But Bell was not in a sociable mood, and only said, “Sooner or later
we maun flit.” Then handing me a letter from Mr. Barrie, she said she
would step into the kitchen until I got it read, and wait to see if there
was any answer.
The letter confirmed the steward’s news, and stated it
would be a favour if I would reserve for him the first KNOWE
offer of Knowe Park, and that he would be home as PARK.
soon as possible. After the steward had left I went to
Bell, and told her that Mr. Barrie’s letter was about Knowe Park, and
asked if she would come with me and see if it would suit. This was
rather a sudden following up of the affair, as Bell had convinced
herself that Mr. Barrie would never leave the manse; and although
she had generally ended her remarks on the subject with “Wherever
could we gang?” she had not tried to answer the question. After a
little hesitation she said, “Knowe Park! that’s where Mr. Taylor lived,
—he had a grand apple tree—an ‘Oslin pippin.’ Is’t aye yonder? It’s a
nice garden—a nice place to stop. I’ve noticed them buildin’ a new
house there, but I haena paid muckle attention till’t. I wad like to
see’t. I’ll be very glad to gang.” And off we set.
It took a very few minutes to go. Bell was pleased with the outside of
the house. “It was a nice house—fine big windows—faced the south
—wad hae a grand view—the garden was bigger than she thocht,
and in gude order. Is thae my plants?—they were thrivin’.” With such
snatches did Bell accompany her survey of the place before she
went into the house. She criticized the inside more minutely, her
standard being the manse. As she went from room to room she
spoke of each, or rather thought aloud: “This is the kitchen—it’s very
nice—lichtsome—plenty o’ room—it looks a nice grate, or I fancy
ye’ll ca’ that a range. I never tried them, but they say they’re handy.
Presses—pantry. What’s ben here?—washing-house—coal-house—
awmrie[2]—bedroom. Very nice; this will do fine. Noo, what about a
study?” I suggested a bedroom, but she gave that only a partial
approval; the other rooms pleased her. When I showed the drawing-
room, “Drawing-room!” said she; “we hae nane at the manse, and
Mrs. Barrie thinks we get on fine without it. We’ll make this the study.
But what about the outhouses?”
[2] The old Scotch name for the store-room containing dry
food, such as flour, meal, etc.
This exercised Bell greatly. There was an old building in the north
corner of the place, behind the bleaching-green, which I had left as it
was; it was a compound of stable, hay-loft, cart-shed, fruit-room, and
potato-house, but required considerable repair. I was undecided
whether to repair it or replace it by a building more in keeping with
the house, but Bell protested against new outhouses. “Na, na; nae
mair expenses. Whitewash it, gi’e it a bit sort up, and it will fit us
exactly.” Then going to the hedge on the east, she said, “Wha’s
aught this park?”
“It goes with the house,” said I, “and is at Mr. Barrie’s service.”
Bell was delighted: she saw her way to bring “Daisy” and the hens.
She became quite cheerful, and left me, saying as she parted, “It’s
the very place for us. I wadna say it’s just up to the manse, but I’m
thankfu’ to ken of such a gude hame for them among kent folk
‘Better a wee bush than nae bield,’[3] as the saying is. But it’s no’ wee
either; it’s just real nice.”
[3] Shelter.