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Paediatric Rheumatology

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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Paediatric Rheumatology

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  1. Paediatric Rheumatology Phil Riley Consultant Paediatric Rheumatologist Teaching

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

  3. Differential Diagnosis • Inflammatory • Mechanical • Psychological

  4. Differential Diagnosis-Inflammatory • Reactive • Infection • Inflammatory disease • Systemic disease • Malignancy • Irritable hip

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

  6. Differential-Psychogenic • Fibromyalgia • Functional illness • Reflex Sympathetic Dystrophy

  7. Juvenile Idiopathic Arthritis • Unknown aetiology • Genetic predisposition • incidence 1 per 10,000 • prevalence 1 per 1,000

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

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

  10. Subtypes • Oligoarthritis 1-4 -persistent -extended • Polyarthritis (RF negative) >=5 • Polyarthritis (RF positive) >=5 • systemic

  11. 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)

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

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

  14. JIA • systemic rash

  15. Systemic JIA • Quotidian fever

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

  17. Subtypes • Enthesitis-related arthritis -HLA B27 • Psoriatic Arthritis • Other

  18. Treatment Options • NSAIDs • Steroids • Joint injections • Methotrexate • Sulphasalazine • Ciclosporin • TNF drugs • Autologous stem cell transplantation

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

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

  21. Questions • Systemic • Rash • Quotidian fever • NSAIDS • Steroids • Methotrexate • Macrophage Activation Syndrome

  22. Treatment Concepts • Early • Monitoring • Multidisciplinary

  23. Treatment continued • Physiotherapy • restore function • improve muscle strength • splints/serial casts • Occupational therapy • Psychology

  24. Spotter • Butterfly Rash

  25. Spotter • Buttterfly

  26. Spotter • Raynaud’s phenomenon

  27. 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

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

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

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

  31. 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

  32. Spotter • Neonatal lupus

  33. Spotter • Neonatal Lupus

  34. 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%

  35. Spotter • Heliotrope Rash

  36. Spotter • Gottrons papules

  37. Spotter • Calcinosis

  38. Spotter • Capillaroscopy • Abnormal “bushy” • Thickening and dropout

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

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

  41. JDM - treatment • Steroids • pulse iv • oral tapering • Methotrexate • Cyclosporin/Immunoglobulin/Cyclophosphamide/Anti TNF • Physio/Rehab

  42. Spotter • en coup de sabre

  43. Spotter • Sclerodactly

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

  45. Questions

  46. Q1

  47. Q1

  48. Q2

  49. Q3

  50. Q4

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