Juvenile idiopathic arthritis Flashcards
Define JIA
Group of systemic inflammatory disorders affecting children below the age of 16
Why is JIA important to recognise?
most commonly diagnosed rheumatic disease In children
important cause of disability and blindness
History of JIA
Cornil 1864 - juvenile onset RA
12 years, polyarticular and dead by 29
peritonitis, pleurisy, renal disease –> amyloidosis?
Who helped to categorise JIA?
George Frederic still in 1896
aetiopathogenesis of JIA
autoimmune disease and multi-factorial
Age of onset
under 16
Duration of disease
more than 6 weeks
presence of arthritis in JIA
joint swelling or 2 of
1. painful or limited ROM 2. tenderness 3. warmth
What investigations are used to confirm arthritis?
USS or MRI
After how long from onset can 3 subtypes of JIA be identified?
6
3 subtypes of JIA and %
pauciarticular - 55%
polyarticular - 25%
systemic onset - 20%
3 types of pauciarticular JIA and %
type 1 - 25
type 2 - 15
type 3 - 15
Who does type 1 pauciarticular JIA affect?
pre-school aged girl
presentation of type 1 pauciarticular JIA
limp due to lower limb involved - knee >ankle>hand
chronic uveitis in 20% –> 95% if female under 2yrs
asymptomatic in 50%
posterior synechiae = irregular iris
positive ANA in 40-75%
Who does type 2 pauciarticular JIA affect?
after 8-9 years, BOYS
presentation of type 2 pauciarticular JIA
limp - lower limb (knee and ankle)
hip can be affected early with rapid damage
categorising type 2 pauciarticular JIA
Canadians - no back involvement = SEA
HLA-B27 and +ve back = juvenile AS
type 2 pauciarticular JIA iridocyclitis
acute in 10-20%
Who does type 3 pauciarticular JIA affect?
any age in childhood, girls
FH of psoriasis
presentation of 3 pauciarticular JIA
asymmetric UL and LL
dactylitis
chronic iridocyclitis in 10-20%
nail pitting
who does RF -ve polyarticular JIA affect?
any age - often early, girls
symptoms of RF -ve polyarticular JIA
constitutional = low grade fever, malaise, anaemia
hepato-splenomegaly, growth abnormalities
symmetric large and small joints
who does RF +ve polyarticular JIA affect?
12-16 years, girls
symptoms of RF +ve polyarticular JIA
constitutional symptoms, anaemia, nodules
What can RF +ve polyarticular JIA be complicated by?
sjogrens, vasculitis, pulmonary fibrosis etc
RF +ve polyarticular JIA x-ray
erosions appear early
systemic JIA - when do extra-articular features start?
early and disappear after 2-5yrs
systemic JIA - who does it affect?
4-6years, equal boys and girls
Systemic JIA features - summary
fever - 39.5 for 2 weeks high in afternoon
rash - 90% salmon red on trunk and thighs
arthritis - 12 months from fever onset
LN - general non tender lymphadenopathy
Abdo - pain, hepatosplenomegaly
serositis - polyserositis, 36% pericarditis
pulmonary - pleural effusion/fibrosis rare
1st line therapy
simple pain killers
NSAIDS
2nd line therapy
no response to NSAID/joint injection
methotrexate
Anti-TNF
IL-1/6 antagonist for systemic
When would systemic steroids be used? (4)
systemic JIA - pain and fever
serious complications
kids undergoing surgery
bridge for DMARDs
Risks of systemic steroids
osteoporosis, infections, growth abnormalities
Local steroids
intra-articular or eye disease oligo-articular JIA
Other treatment
rehab - physio and OT
surgery
synovectomy
reconstructive/joint replacement
