Management

Nonoperative Management

 

Therapy

 

Physio

- prevent contractures

- prevent wasting

 

Splints

- dampen infection

- aid ADL's and schoolwork

 

Medications

 

NSAID

- mainstay of treatment

- paediatric suspensions available

- avoid Salicylates because of Reye's disease

 

IV Immunoglobin

- Still's

 

Methotrexate

- increased use

- much higher doses in kids

- attempt to prevent progression

- aggressive treatment in polyarticular disease with poor prognosis

 

Gold & Penicillamine 

- seropositive

 

Sulphasalazine

- spondyloarthropathy

 

Systemic Steroids

 

Issue

- doesn't affect progression of disease 

- se limited by SE: stunting / osteoporosis

 

Indications

- unresponsive Still's / Polyarthritis

 

Alternate day treatment

- minimal stunting

- avoid suppression hypothalamic - pituitary axis

 

HCLA 

 

Indications

- flares of pauciarticular disease 

- knee / flexor sheaths

 

Advantage

- non specific temporary palliative treatment

- allows good pain relief

- early mobilisation

- therefore preventing contractures / muscle atrophy

- preserving function

 

Infliximab

 

Monoclonal anti TNF antibody

- effective in 60%

- 5% develop allergic reactions

- 10% develop new autoantibodies

 

Operative Management

 

Joints

 

Shoulder

- may require soft tissue releases + synovectomy

 

Elbow

- may need synovectomy + radial head resection

 

Wrist

- very frequently involved

- short ulna

- ulnocarpal deviation (c.f. radial deviation in adult RA)

- require synovectomy / fusions

 

MCPJ

- radial deviation (c.f. ulna deviation in adult RA)

- synovectomy / realignments / replacement

 

Finger

- much less likely to be involved than adult form

- Boutonniere

- Swan neck quite rare

 

Thumb

- may develop flail thumb secondary to erosion

 

Hip

- Otto pelvis

- bilateral protrusio

- atlantoaxial subluxation

 

Synovectomy

 

Most common in wrist / hand / elbow

- limits pain and improves function in short term

- does not affect long term outcome or ROM (> 3 years)

 

Complications

 

Growth defects

- growth retardation with steroids

 

Epiphyseal disturbances 

- external tibial torsion

- dysplasia of distal ulna (commonly short)

- dens hypoplasia 

- scoliosis

 

Iridocyclitis

- pauciarticular disease

- can lead to blindness

 

Amyloidosis

- long standing Still's

- may be fatal

 

Prognosis

 

Polyarticular

- juvenile or systemic usually only last about 6/52

- 75% long remission with little residual disability

 

Poor prognosis

- systemic with multiple joint involvement

- RF +ve

- onset < 1 year old

- long active period disease > 5 years

 

Death uncommon

- infection early

- amyloidosis late