Juvenile Idiopathic Arthritis Flashcards
what is JIA?
JIA is a group of systemic inflammatory disorders affecting children below the age of 16.
diagnosis of JIA
<16, smyptoms > 6weeks presence of arthritis = joint swelling or 2 of: painful or limited joint motion tenderness warmth
after 6 months, 3 major subtypes of JIA can be identified. Name them and their relative %
pauciarticular 55%
polyarticular 25%
systemic onsent 20%
what is pauciarticular JIA?
4 or less joints
30% of those with pauciarticular JIA will go on to develop what?
polyarticular JIA
type 1 pauciarticular JIA: age of onset
<5
peak 1-3
type 1 pauciarticular JIA: M:F
1:8
type 1 pauciarticular JIA: presentation
limp rather than pain
no constitutional manifestations
type 1 pauciarticular JIA: makes up what % of JIA?
25%
type 1 pauciarticular JIA: ANA are +ve in what %
40-75
type 1 pauciarticular JIA: joints affected
knee > ankle > hand or elbow
rarely hip
type 1 pauciarticular JIA: chronic uveitis develops in how many?
20%
95% if female < 2
type 1 pauciarticular JIA: is asymptomatic in how many?
50%
type 1 pauciarticular JIA: what causes the irregular iris?
poster synechiae
type 3 pauciarticular JIA: makes up what % of JIA?
15%
type 3 pauciarticular JIA: age
any age during childhood
type 3 pauciarticular JIA: M”F
1:4
type 3 pauciarticular JIA: presentation
consitutional
asymmetric UL and LL arthritis
dactylitis
type 3 pauciarticular JIA: 40% have a FHx of?
psoariasis
type 3 pauciarticular JIA: chronic iridocyclitis develops what %
10-20
type 2 pauciarticular JIA: makes up what % of JIA?
15%
type 2 pauciarticular JIA: age
8-9
type 2 pauciarticular JIA: M:F
7:1
type 2 pauciarticular JIA: presentation
consitutional rare
limp due to LL
type 2 pauciarticular JIA: joints
knee
ankle
hip
type 2 pauciarticular JIA: what can happen if the hip is affected
hip can be affect early with rapid damage requiring THR early in life _ enthesitis + many have sacroiliac joints and may evolve to AS or spondyloarthorpathy
type 2 pauciarticular JIA: those with HLA-27 _ back involvement will be classified as what instead?
juvenile ankylosing spondylitis
type 2 pauciarticular JIA: acute iridocylitis in what %
10-20%
define polyarticular JIA
5 or more joints
RF -ve polyarticular JIA: makes up what % of JA
15%
RF -ve polyarticular JIA: age
any but often early
RF -ve polyarticular JIA: M:F
1:9
RF -ve polyarticular JIA: joints affected
symmetric large and small joints knees wrists ankles MCPs PIP neck
RF -ve polyarticular JIA: presenation
constitutional manifestations - low grade fever, malise
hepato-splenomegaly
mild anaemia
growth abnormalitis
RF +ve polyarticular JIA: makes up what % of JIA
10%
RF +ve polyarticular JIA: age
late childhood 12-16
RF +ve polyarticular JIA: M:F
1:7
RF +ve polyarticular JIA: presentation
constitutional symptoms - low grade fever, malaise, weight loss
anaemia
nodules
RF +ve polyarticular JIA: can be complicated by
Sjogren's Felty vasculitis AR pulmonary fibrosis AAS CTS
systemic onset JIA =
STILLs disease
STILL’s disease: makes up what % of JIA
20%
STILL’s disease: age
4-6 but throughout childhood
STILL’s disease: M:F
1:1.5
STILL’s disease: what defines the disease?
extra articular features - start early disappear after 2-5 years
STILL’s disease: fever
rise to 39.5 daily for at least 2 weeks
late in afternoon or evening and returns to normal or sunormal in the morning
child appears toxic with fever +/- chills, but normal without
STILL’s disease: rash
90% evanescent salmon red eruption on trunk and thighs acompanies fever can be brought by stratchin
STILL’s disease: lymph nodes
50-75%
generalised lymphadenopathy
non-tender
STILL’s disease: abdominal
hepatosplenomegaly
50-70%
abdo pain
+/- transaminases
STILL’s disease: serositis
polyserositis
pericarditis in 36%
tamponade and myocarditis rare
STILL’s disease: pulmonary
rare
pleural effusion
pulmonary fibrosis
STILL’s disease: arthritis
75%
within 3-12 months of onset of fever
wrists, knees, ankles, c-spine, TMJ, hips
management of JIA: 1st line therapy
o Simple pain killers o NSAIDs § Difference between adults and children in half life § Can control disease § Doses § Same compounds only
management of JIA: 2nd line therapy
o If no response to NSAIDs/joint steroid injections
o Rarely needed in oligoarticular JIA
o Methotrexate (pharmacokinetics is age related)
o Anti-TNF Rx (all 3) in methotrexate failure
o IL-1 R-antagonist (Anakinra) in refractory systemic arthritis
o IL-6 antagonist (Tocilizumab) for refractor systemic disease
o Systemic steroids
§ Limited indications due to serious side effects
§ Used in
• Systemic JIA to control pain and fever
• Serious disease complications with any subtype e.g. pericardial
effusion, tamponade, vasculitis, severe auto-immune anaemia,
severe eye disease
• As a bridge between DMARDs
• Children undergoing surgery
§ Risk of osteoporosis, infections, growth abnormalities
o Local steroids
§ Intra-articular mainly in oligo-articular JIA
§ Eye disease (ANA +ve oligo-articular disease)
non-drug treatment of JIA
PT
OT
synovectomy
reconstructive/joint replacement surgery
