1. Kathy Bailey Consultant Paediatric Rheumatologist Coventry and Warwickshire

2.  Common  History and examination essential  Missed diagnosis permanent disability  Simple problems require confident diagnosis  Will become part of curriculum!

4.  Limp  stiffness  swelling  pain  restriction of movement  change in activities  not using limb  colour change in limb  fever  rash  unwell

5.  HISTORY!!! ◦ Inflammatory ◦ mechanical ◦ non-organic/psychosomatic

6.  HISTORY!!! ◦ Inflammatory ◦ mechanical ◦ non-organic/psychosomatic ◦ Acute or chronic

7.  HISTORY!!! ◦ Inflammatory ◦ mechanical ◦ non-organic/psychosomatic ◦ Acute or chronic  EXAMINATION ◦ objective signs

8.  HISTORY!!! ◦ Inflammatory ◦ mechanical ◦ non-organic/psychosomatic ◦ Acute or chronic  EXAMINATION ◦ objective signs  TESTS ◦ ???

9.  Age of child  Duration  Symptoms  Impact on activities  Joints affected  Family History  Antecedents ◦ infection/trauma/ ◦ illness

10.  Age of child  Duration  Symptoms  Impact on activities  Joints affected  Family History  Antecedents ◦ infection/trauma/ ◦ illness  Associated features: ◦ Constitutional ◦ Fever ◦ Rash ◦ Muscle weakness ◦ Eyes ◦ Weight loss ◦ GI ◦ bruising ◦ LN/mucusitis....etc

11.  Height and weight  Temp/pulse/BP  General observations  Rash  Systems examination  Urinalysis

12. Paediatric Gait Arms Legs Spine  www.arc.org.uk www.arc.org.uk/arthinfo/emedia.asp

13.  LOOK gait swelling deformity rash/colour changes

14.  FEEL heat swelling tenderness

15.  MOVE restriction +/-pain muscle strength

16. InflammatoryMechanicalPsychosomatic Pain+/-++++ Stiffness+++/-+ Swelling++++/- Sleep disturbance +/--++ Instability+/-+++/- Physical signs ++++/- (or ++++)

17. InflammatoryMechanicalIdiopathic InfectionReactive Post Strep JIA Connective tissue diseases - SLE - SLE - JDMS - JDMS - Scleroderma - Scleroderma - Vasculitis - VasculitisHypermobilityOsteochondroses - osgood-schlatter - osgood-schlatter - Scheuermann’s - Scheuermann’s - Perthes - Perthes Chondromalacia patella Osteochondritis dissecans Slipped upper femoral epiphysis Pain amplification syndromes - Localised - Localised - Generalised - Generalised Growing pains

18. Acute

19.  Fever  Localised tenderness  hot  Painful to move  Raised inflammatory markers

20.  Fever  Localised tenderness  hot  Painful to move  Raised inflammatory markers  JOINT ASPIRATION

21. Site %  Knee39  Hip 25  Ankle14  Elbow12 Organisms  Staph Aureus  Tuberculosis  Salmonella in sickle cell disease

24. May be history of recent infection Single or multiple joints No systemic features Resolves by 6 weeks Important to consider alternative diagnoses

25.  Reactive Vasculitis (small vessel)  Palpable Purpura  Arthralgia/Arthritis  Abdominal pain  Nephritis  Headaches

26.  1% of patients referred to paediatric rheumatology have underlying malignancy

27.  Acute Lymphoblastic Leukaemia ◦ Bone pain and arthralgia in 20-40% ◦ Suspect from history, exam, or blood count ◦ Bone Marrow aspirate

28.  Acute Lymphoblastic Leukaemia  Neuroblastoma ◦ Commonest solid tumour under infants ◦ Bone pain from secondary spread ◦ Urinary excretion of catecholamine metabolites (VMA)

29.  Acute Lymphoblastic Leukaemia  Neuroblastoma  Primary Bone tumour ◦ Osteoid osteoma – benign ◦ osteosarcoma

30.  Features to raise concern: ◦ Bone pain (night time) ◦ Weight loss ◦ Night sweats or fevers ◦ Abnormal bloods ◦ Xray changes

32. 5 of following 1. Fever>5 days; unresponsive to Abx 2. Non purulent conjunctivitis 3. lymphadenopathy>1.5cm 4. Rash - polymorphous 5. mucosal changes 6. extremities  early - swelling/palmar erythema  late – peeling OR 4 plus coronary artery aneurysms

36.  Prevent late sequel of coronary artery aneurysms ◦ Intravenous IVIG ◦ Aspirin –  initially high, anti inflammatory  then low dose, anti platelet

37. Chronic

38. JIA Juvenile Idiopathic Arthritis JRA Juvenile Rheumatoid Arthritis JCA Juvenile Chronic Arthritis

39. JIA Juvenile Idiopathic Arthritis JRA Juvenile Rheumatoid Arthritis JCA Juvenile Chronic Arthritis

40.  JIA commonest rheumatic condition in childhood ◦ 30 – 150 per 100,000  10 years follow up ◦ 1/3 achieve remission ◦ 30% have severe functional limitations Fantini et al, ACR 1996

41.  Disease of childhood onset ◦ under 16 years  Persistence of arthritis ◦ 1 or more joints ◦ 6 or more weeks ◦ Exclusion of other diagnoses

42. Defined by clinical features in first 6 months

43. ◦ Oligoarthritis1-4 joints  Persistent  Extended

44.  Girls >boys  Younger age  Best prognosis

45.  Girls >boys  Younger age  Best prognosis  Associated with uveitis

46. Defined by clinical features in first 6 months ◦ Oligoarthritis1-4 joints ◦ Polyarthritis 5 or more joints  RF positive  RF negative

47. Defined by clinical features in first 6 months ◦ Oligoarthritis1-4 joints ◦ Polyarthritis 5 or more joints ◦ Psoriatic Arthritis  Arthritis AND psoriasis OR  Arthritis plus 2 of:  Nail pitting  Dactylitis  First degree relative with confirmed psoriasis

48. Defined by clinical features in first 6 months ◦ Oligoarthritis1-4 joints ◦ Polyarthritis 5 or more joints ◦ Psoriatic Arthritis ◦ Enthesitis Related Arthritis  Arthritis AND enthesitis OR  Sacroiliac pain and HLA B27

49. Defined by clinical features in first 6 months ◦ Oligoarthritis1-4 joints  Persistent  Extended ◦ Polyarthritis 5 or more joints  RF positive  RF negative ◦ Psoriatic Arthritis ◦ Enthesitis Related Arthritis ◦ Systemic Arthritis

50.  Daily fever for at least 2 weeks duration (quotidian for 3 days)  Plus one or more of: ◦ Evanescent rash ◦ Generalized lymphadenopathy ◦ Hepatosplenomegaly ◦ Serositis  Arthritis  EXCLUSION OF OTHER DIAGNOSES

53.  Poor indicators  Polyarticular onset and course  Rheumatoid factor positive girls  Systemic disease with persistent features  Delay in starting effective treatment  Good indicators  Oligoarticular disease

54. Goals ◦ Disease remission ◦ Symptomatic improvement  Stiffness  Pain  Joint range of movement ◦ Prevent joint damage ◦ Normal growth and development ◦ Education and normal adolesence ◦ Prevent eye damage from Uveitis

55. Multidisciplinary team ◦ Paediatric rheumatologist ◦ Nurse specialist ◦ Occupational Therapist ◦ Physiotherapist ◦ Social worker ◦ Ophthalmologist ◦ Podiatrist

56.  Anti inflammatory drugs ◦ NSAIDs ◦ Glucocorticoids  “Disease modifying drugs” ◦ Methotrexate ◦ Etanercept ◦ New biologic agents for recalcitrant disease

58.  Avascular necrosis of the femoral head  usually 2-10 (peak 4-6) yrs.  3-5 boys:girls  Bilateral 30 %

59. Imaging:  Asymmetry in femoral heads  Consider MRI or Nuclear medicine if clinical suspicion is high

61.  10-13 years old  Overweight boys  25% bilateral within 18/12  Slip of femoral head through growth plate  (posteriorly and inferiorly)

62. Imaging:  AP and (frog) lateral films needed  CT/ MRI in cases of difficulty

63. Klein line should intersect femoral head

67.  Inappropriate history  Physical signs don’t match story  Other concerning features  Concerns raised by others

68. Chondromalacia patella  Adolescent girls  Painful knees- kneeling - going up stairs

69. Osgood-Schlatter disease  Adolescent boys  Pain and swelling at tibial tuberosity  Increased by exercise

70. Osgood-Schlatter disease  Adolescent boys  Pain and swelling at tibial tuberosity  Increased by exercise  Tenderness +/- swelling of tibial tuberosity  Pain on resisted extension of knee

71. Clinical diagnosis DO NOT XRAY

72.  Very common  May be generalised or localised  Frequently responsible for musculoskeletal pain

73.  Common cause of lower limb pain  If symptomatic – correct with good footware and insoles

75.  25-40% of children!  3-5 years and 8-12 years  Typical history

76.  Wake during night with pain  Eased with massage  May be worse after active day  No daytime symptoms  No abnormal physical signs

77.  No identifiable inflammatory or mechanical condition  Chronic pain  Impact on daily activities  Average age 9 – 12 years  Girls > boys  Disease of the developed world

78. Localised idiopathic pain eg RSD CFS/ME Fibromyalgia Diffuse idiopathic pain

80.  History  Examination  Investigations:targeted

81.  Blood Count ◦ ? Appropriate to clinical features  Inflammatory markers ◦ Usually mirror clinical features ◦ Not always raised in inflammatory conditions  Blood and synovial fluid cultures  ANA/Rh Factor ◦ Not helpful in making a diagnosis  Imaging ◦ Need to use best modality and ask the right question

82.  Musculoskeletal complaints are common in childhood  Serious pathology leads to long term disability if not appropriately managed  Diagnosis is dependant on good history and examination