1. Paediatric Rheumatology Phil Riley Consultant Paediatric Rheumatologist Teaching

2. 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

3. Differential Diagnosis n Inflammatory n Mechanical n Psychological

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

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

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

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

8. 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

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

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

11. 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)

12. 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

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

14. JIA n systemic rash

15. Systemic JIA n Quotidian fever

16. 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

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

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

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

20. 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

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

22. Treatment Concepts n Early n Monitoring n Multidisciplinary

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

24. Spotter n Butterfly Rash

25. Spotter n Buttterfly

26. Spotter n Raynaud’s phenomenon

27. 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

28. 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

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 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

32. Spotter n Neonatal lupus

33. Spotter n 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 n Heliotrope Rash

36. Spotter n Gottrons papules

37. Spotter n Calcinosis

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

39. 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

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/Cyclophos phamide/Anti TNF  Physio/Rehab

42. Spotter n en coup de sabre

43. Spotter n Sclerodactly

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

45. Questions

46. Q1

48. Q2

49. Q3

50. Q4

51. Q5

52. Q6

53. Q7

54. Q8

55. Q9

56. Q10

57. Q11

58. Q12

59. Q13

60. Q14

61. Q15

62. Q16

63. Q17