kathy bailey consultant paediatric rheumatologist coventry and warwickshire

Kathy BaileyConsultant 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

www.arc.org.uk/arthinfo/emedia.asp

LOOKgait

swelling

deformity

rash/colour changes

FEELheat

swelling

tenderness

MOVErestriction

+/- pain

muscle strength

Inflammatory Mechanical Psychosomatic

Pain +/- + +++

Stiffness ++ +/- +

Swelling +++ +/- +/-

Sleep disturbance

+/- - ++

Instability +/- ++ +/-

Physical signs

++ + +/-

(or ++++)

InflammatoryInflammatory MechanicalMechanical IdiopathicIdiopathic

InfectionInfection

ReactiveReactive

Post StrepPost Strep

JIAJIA

Connective Connective tissue diseasestissue diseases

- SLE- SLE

- JDMS- JDMS

- Scleroderma- Scleroderma

- Vasculitis- Vasculitis

HypermobilityHypermobility

OsteochondrosesOsteochondroses

- osgood-schlatter- osgood-schlatter

- Scheuermann’s- Scheuermann’s

- Perthes- Perthes

Chondromalacia Chondromalacia patellapatella

Osteochondritis Osteochondritis dissecansdissecans

Slipped upper Slipped upper femoral epiphysisfemoral epiphysis

Pain Pain amplification amplification syndromessyndromes

- Localised- Localised

- Generalised- Generalised

Growing painsGrowing 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 %

Knee 39 Hip 25 Ankle 14 Elbow 12

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 following1. Fever >5 days; unresponsive to Abx2. Non purulent conjunctivitis3. lymphadenopathy >1.5cm4. Rash - polymorphous5. mucosal changes6. 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

Defined by clinical features in first 6 months◦ Oligoarthritis 1-4 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◦ Oligoarthritis 1-4 joints◦ Polyarthritis 5 or more joints

RF positive RF negative

Defined by clinical features in first 6 months◦ Oligoarthritis 1-4 joints◦ Polyarthritis 5 or more joints◦ Psoriatic Arthritis

Arthritis AND psoriasisOR Arthritis plus 2 of:

Nail pitting Dactylitis First degree relative with confirmed psoriasis

Defined by clinical features in first 6 months◦ Oligoarthritis 1-4 joints◦ Polyarthritis 5 or more joints◦ Psoriatic Arthritis◦ Enthesitis Related Arthritis

Arthritis AND enthesitisOR Sacroiliac pain and HLA B27

Defined by clinical features in first 6 months◦ Oligoarthritis 1-4 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

10-13 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 History History Examination Examination 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