Transcript Phil Riley Presentation

Paediatric Rheumatology
Phil Riley
Consultant Paediatric Rheumatologist
Teaching
Introduction
Musculoskeletal pain common in
childhood
 Wide range of diagnoses -benign to
malignant
 JIA- early recognition and appropriate
treatment prevents damage
 JIA- Multidisciplinary team
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Differential Diagnosis
Inflammatory
 Mechanical
 Psychological
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Differential DiagnosisInflammatory
Reactive
 Infection
 Inflammatory disease
 Systemic disease
 Malignancy
 Irritable hip
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Differential DiagnosisMechanical
Trauma-accidental and NAI
 Hypermobility
 Osteochondritides
 Degenerative disorders
 Haematological
 Metabolic
 Tumours- benign and malignant
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DifferentialPsychogenic
Fibromyalgia
 Functional illness
 Reflex Sympathetic Dystrophy
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Juvenile Idiopathic Arthritis
Unknown aetiology
 Genetic predisposition
 incidence 1 per 10,000
 prevalence 1 per 1,000
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Disease Course
50% still active after 25 years
 X-ray Joint damage by 2.6 years
 Increased mortality
 Increased morbidity-Physical
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-Growth
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-Psychological
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-Social
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Classification of
Juvenile Arthritis
<16 years
 >6 weeks arthritis
 Subtypes classified for first 6 months
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Subtypes
Oligoarthritis
-persistent
-extended
 Polyarthritis (RF negative)
 Polyarthritis (RF positive)
 systemic
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1-4
>=5
>=5
Investigations-JIA
ANA- Antinuclear antibody
 Inflammatory markers- CRP,ESR
 FBC- Anaemia of chronic disease
 x-ray -loss of joint space, erosions and
carpal bone overcrowding
 MRI- synovitis (gadolinium enhanced)
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Uveitis
Chronic anterior uveitis
 Often Asymptomatic
 Young,female, oligoarthritis, positive
ANA (30%)
 Polyarthritis (5%)
 systemic(rare)
 Slit lamp 3-6 monthly for 7 years
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Subtypes
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Systemic Arthritis
-Rash
-temp over 2 weeks
-with/without arthritis
-with/without serositis
-hepatosplenomegaly, lymphadenopathy
JIA
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systemic rash
Systemic JIA
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Quotidian fever
Systemic JIA
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Macrophage Activation Syndrome(MAS)
-Bleeding, purpura, bruising
-Nodes,liver,spleen
-FBC,ESR
-PT,APTT, FDP
- Fibrinogen,clotting factors
Bone Marrow
IV steroids,Cyclosporin
Subtypes
Enthesitis-related arthritis
-HLA B27
 Psoriatic Arthritis
 Other
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Treatment Options
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NSAIDs
Steroids
Joint injections
Methotrexate
Sulphasalazine
Ciclosporin
TNF drugs
Autologous stem cell transplantation
Questions
Oligoarthritis
 ANA pos
 normal slightly raised ESR,CRP
 NSAIDS
 Joint injection
 Ophthalmology referral
 Rehab/MDT
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Questions
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Polyarthritis
ANA positive or negative
Rh factor positive or negative
very raised ESR,CRP
Steroids
Methotrexate
Eyes
Rehab/MDT
Questions
Systemic
 Rash
 Quotidian fever
 NSAIDS
 Steroids
 Methotrexate
 Macrophage Activation Syndrome
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Treatment Concepts
Early
 Monitoring
 Multidisciplinary
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Treatment continued
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Physiotherapy
 restore function
 improve muscle strength
 splints/serial casts
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Occupational therapy
Psychology
Spotter
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Butterfly Rash
Spotter
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Buttterfly
Spotter
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Raynaud’s
phenomenon
SLE- Diagnostic/ WHO
classification criteria(4 of 11)
Malar rash(butterfly)
 Discoid lupus rash
 Photosensitivity
 Oral/nasal mucosal ulceration
 Non-erosive arthritis
 nephritis
-proteinuria/cellular casts
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SLE-Diagnostic/WHO
classification(4 of 11)
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Encephalopathy-seizures/psychosis
Pleuritis/pericarditis
Haematological
-lymphopaemia
-thrombocytopaenia
positive immunoserology
-anti ds-DNA
ANA positive
SLE- clinical features
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Constitutional - fever/malaise/weight loss
Cutaneous
 rash/photosensitivity/alopecia/mouth ulcers
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Musculoskeletal
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poly-arthritis/arthralgia
tenosynovitis
myopathy
avascular necrosis
SLE-Clinical features
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Vascular
 lupus crisis/Raynaud’s/livedo
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Cardiac
 pericarditis/myocarditis/endocarditis
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Pulmonary
 pleuritis/pneumonitis/haemorrhage
SLE-lab features
FBC- low platelets
- low lymphocytes
 Inflammatory markers- high ESR,
normal CRP
 ANA- very high ie >1:2560
 DsDNA- high
 C3,C4- low
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Spotter
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Neonatal lupus
Spotter
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Neonatal Lupus
Neonatal lupus
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Maternal autoantibody transmission
Cong heart block (Ro/La)
- 50%
Cutaneous neonatal lupus
- 37%
Hepatic/GI tract
- 8%
Haematologic
-6%
Neurologic and pulmonary -1%
Spotter
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Heliotrope Rash
Spotter
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Gottrons papules
Spotter
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Calcinosis
Spotter
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Capillaroscopy
Abnormal “bushy”
Thickening and
dropout
Definition of Juvenile
Dermatomyositis(JDM)
limb-girdle and anterior neck flexor
weakness
 Muscle biopsy
 Muscle enzyme increase
 EMG
 Dermatological features
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JDM - clinical features
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Proximal weakness
Vasculopathy
 Heliotrope facial rash
 Gottrons papules
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Raised CK/LDH/AST/ALT
MR scan/muscle biopsy
Multi-organ occasionally
JDM - treatment
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Steroids
 pulse iv
 oral tapering
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Methotrexate
Cyclosporin/Immunoglobulin/Cyclophos
phamide/Anti TNF
Physio/Rehab
Spotter
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en coup de sabre
Spotter
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Sclerodactly
Scleroderma in children
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Systemic sclerosis
 limited cutaneous/CREST
 diffuse
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Localised
 linear scleroderma (en coup de
sabre,morphoea)
Questions
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Q11
Q12
Q13
Q14
Q15
Q16
Q17