Kathy Bailey Consultant Paediatric Rheumatologist Coventry and Warwickshire

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

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

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

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

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

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

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

 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

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

Paediatric Gait Arms Legs Spine 

 LOOK gait swelling deformity rash/colour changes

 FEEL heat swelling tenderness

 MOVE restriction +/-pain muscle strength

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

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

Acute

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

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

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

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

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

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

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

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

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

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

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

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

Chronic

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

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

 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

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

Defined by clinical features in first 6 months

◦ Oligoarthritis1-4 joints  Persistent  Extended

 Girls >boys  Younger age  Best prognosis

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

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

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

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

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

 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

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

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

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

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

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

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

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

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

Klein line should intersect femoral head

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

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

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

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

Clinical diagnosis DO NOT XRAY

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

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

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

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

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

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

 History  Examination  Investigations:targeted

 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

 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