Paediatric Rheumatology Phil Riley Consultant Paediatric Rheumatologist Teaching

Introduction n Musculoskeletal pain common in childhood n Wide range of diagnoses -benign to malignant n JIA- early recognition and appropriate treatment prevents damage n JIA- Multidisciplinary team

Differential Diagnosis n Inflammatory n Mechanical n Psychological

Differential Diagnosis- Inflammatory n Reactive n Infection n Inflammatory disease n Systemic disease n Malignancy n Irritable hip

Differential Diagnosis- Mechanical n Trauma-accidental and NAI n Hypermobility n Osteochondritides n Degenerative disorders n Haematological n Metabolic n Tumours- benign and malignant

Differential- Psychogenic n Fibromyalgia n Functional illness n Reflex Sympathetic Dystrophy

Juvenile Idiopathic Arthritis n Unknown aetiology n Genetic predisposition n incidence 1 per 10,000 n prevalence 1 per 1,000

Disease Course n 50% still active after 25 years n X-ray Joint damage by 2.6 years n Increased mortality n Increased morbidity-Physical n -Growth n -Psychological n -Social

Classification of Juvenile Arthritis n <16 years n >6 weeks arthritis n Subtypes classified for first 6 months

Subtypes n Oligoarthritis1-4 -persistent-extended n Polyarthritis (RF negative)>=5 n Polyarthritis (RF positive)>=5 n systemic

Investigations-JIA n ANA- Antinuclear antibody n Inflammatory markers- CRP,ESR n FBC- Anaemia of chronic disease n x-ray -loss of joint space, erosions and carpal bone overcrowding n MRI- synovitis (gadolinium enhanced)

Uveitis n Chronic anterior uveitis n Often Asymptomatic n Young,female, oligoarthritis, positive ANA (30%) n Polyarthritis (5%) n systemic(rare) n Slit lamp 3-6 monthly for 7 years

Subtypes n Systemic Arthritis -Rash -temp over 2 weeks -with/without arthritis -with/without serositis -hepatosplenomegaly, lymphadenopathy

JIA n systemic rash

Systemic JIA n Quotidian fever

Systemic JIA n Macrophage Activation Syndrome(MAS) -Bleeding, purpura, bruising -  Nodes,liver,spleen -  FBC,ESR -  PT,APTT, FDP -  Fibrinogen,clotting factors n Bone Marrow n IV steroids,Cyclosporin

Subtypes n Enthesitis-related arthritis -HLA B27 n Psoriatic Arthritis n Other

Treatment Options n NSAIDs n Steroids n Joint injections n Methotrexate n Sulphasalazine n Ciclosporin n TNF drugs n Autologous stem cell transplantation

Questions n Oligoarthritis n ANA pos n normal slightly raised ESR,CRP n NSAIDS n Joint injection n Ophthalmology referral n Rehab/MDT

Questions n Polyarthritis n ANA positive or negative n Rh factor positive or negative n very raised ESR,CRP n Steroids n Methotrexate n Eyes n Rehab/MDT

Questions n Systemic n Rash n Quotidian fever n NSAIDS n Steroids n Methotrexate n Macrophage Activation Syndrome

Treatment Concepts n Early n Monitoring n Multidisciplinary

Treatment continued  Physiotherapy  restore function  improve muscle strength  splints/serial casts  Occupational therapy  Psychology

Spotter n Butterfly Rash

Spotter n Buttterfly

Spotter n Raynaud’s phenomenon

SLE- Diagnostic/ WHO classification criteria(4 of 11) n Malar rash(butterfly) n Discoid lupus rash n Photosensitivity n Oral/nasal mucosal ulceration n Non-erosive arthritis n nephritis -proteinuria/cellular casts

SLE-Diagnostic/WHO classification(4 of 11) n Encephalopathy-seizures/psychosis n Pleuritis/pericarditis n Haematological -lymphopaemia-thrombocytopaenia  positive immunoserology -anti ds-DNA n ANA positive

SLE- clinical features  Constitutional - fever/malaise/weight loss  Cutaneous  rash/photosensitivity/alopecia/mouth ulcers  Musculoskeletal  poly-arthritis/arthralgia  tenosynovitis  myopathy  avascular necrosis

SLE-Clinical features  Vascular  lupus crisis/Raynaud’s/livedo  Cardiac  pericarditis/myocarditis/endocarditis  Pulmonary  pleuritis/pneumonitis/haemorrhage

SLE-lab features n FBC- low platelets - low lymphocytes - low lymphocytes n Inflammatory markers- high ESR, normal CRP n ANA- very high ie >1:2560 n DsDNA- high n C3,C4- low

Spotter n Neonatal lupus

Spotter n Neonatal Lupus

Neonatal lupus  Maternal autoantibody transmission  Cong heart block (Ro/La) - 50%  Cutaneous neonatal lupus- 37%  Hepatic/GI tract - 8%  Haematologic-6%  Neurologic and pulmonary-1%

Spotter n Heliotrope Rash

Spotter n Gottrons papules

Spotter n Calcinosis

Spotter n Capillaroscopy n Abnormal “bushy” n Thickening and dropout

Definition of Juvenile Dermatomyositis(JDM) n limb-girdle and anterior neck flexor weakness n Muscle biopsy n Muscle enzyme increase n EMG n Dermatological features

JDM - clinical features  Proximal weakness  Vasculopathy  Heliotrope facial rash  Gottrons papules  Raised CK/LDH/AST/ALT  MR scan/muscle biopsy  Multi-organ occasionally

JDM - treatment  Steroids  pulse iv  oral tapering  Methotrexate  Cyclosporin/Immunoglobulin/Cyclophos phamide/Anti TNF  Physio/Rehab

Spotter n en coup de sabre

Spotter n Sclerodactly

Scleroderma in children  Systemic sclerosis  limited cutaneous/CREST  diffuse  Localised  linear scleroderma (en coup de sabre,morphoea)

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