Juvenile Idiopathic Arthritis Flashcards
what is JIA?
JIA is an autoimmune rheumatological condition that affects the joints in children as well as systemically
it is multifactorial and the aetiology is different to that of adult arthritis
what is the criteria for diagnosis of JIA?
age of onset < 16 yrs duration > 3 months presence of arthritis: joint swelling or 2 of the following: - painful or limited joint movement - tenderness - warmth
what are the subtypes of JIA?
pauciarticular
polyarticular
systemic onset
what sex is JIA most common in?
females
except enthesitis related JIA
what joints/how many does pauciarticular JIA affect?
4 joints or less
what are the features of type 1 pauciarticular JIA?
present with limp rather than pain (as they are so young)
irregular iris due to posterior synechae
chronic uveitis in 20 %
asymptomatic in 50%
ANA positive
mainly affects the lower limb joints
what are the features of type II pauciarticular uveitis?
mainly affects lower limbs
can affect the hip, enthesitis, sacroiliac joints and may develop ankylosing spondylitis / spondyloarthritis
acute iridocyclitis
what is juvenile ankylosing spondylitis
if a patient has type II pauciarticular JIA, HLA B27 ve+ plus back involvement they are termed as having juvenile ankylosing spondylitis
what are the features of type III pauciarticular JIA?
asymmetric upper and lower limb involvement
dactylitis
family history of psoriasis - may develop psoriasis later on
chronic iridiocycilitis
what are the 2 subtypes of polyarticular JIA?
RF negative
RF positive
at what age of onset if RH ve- polyarticular JIA most common?
can occur at any age in childhood
what are the features of RF ve- polyarticular JIA?
constitutional manifestations (fever, malaise) hepatosplenomegaly mild anaemia growth abnormalities symmetric small and large joints
what is the age onset of RF ve+ polyarticular JIA?
late childhood (teens 12-16yrs)
what is the presentation of RH ve+ polyarticular JIA?
constitutional features (low grade fever, malaise)
anaemia
nodules
similar to RA in adult but in a child
what conditions can complicate RF ve+ polyarticular JIA?
sjogrens feltys aortic regurgitation pulmonary fibrosis acute aortic syndrome carpal tunnel syndrome
what is the age of onset for pauciarticular JIA?
type 1: < 5 yrs
type 2: > 8/9yrs
type 3: any age
what genes are involved with pauciarticular JIA?
type 1: ANCA ve+
type 2: HLA B27
in what type of JIA do you find dactylitis?
type III pauciarthritis
what are the features of systemic onset (stills) disease?
persistent high spiking fevers
salmon coloured rash
joint pain / arthritis
what are other extra articular features of systemic onset JIA (stills) ?
pulmonary: - pleural effusion - pulmonary fibrosis stomach; - hepatosplenomegaly - abdominal pain lymphadenopathy rash heart; - pericarditis - myocarditis - tamponade
what characteristic of the fever in stills disease?
spike fever up to 39.5 each day
mainly in the late afternoon/evenings but settles in the morning
child appears toxic with chills etc during fever but are fine when fever is away
what are the complications of uveitis in JIA?
glaucoma
blindness
posterior synechiae
band keratopathy
what are the treatment options for uveitis in patients with JIA?
steroids methotrexate adriatic and cyclopegic agents cyclosporin anti TNF alpha
what is the treatment for JIA?
1st line; NSAIDS or joint steroid injections
2nd line:
- methotrexate
- anti TNF alpha
- IL-1 R antagonist
- IL-6 antagonist
surgery
when are steroids used to treat JIA ?
intra articular steroids mainly used to treat oligoarticular JIA
used to treat severe disease complications i.e. pericarditis
used to treat eye disease in oligoarticular JIA (ANCA ve+)
what are the surgical treatment options for JIA?
synovectomy
reconstruction / joint replacement
what are the 2 main complications from JIA?
anterior uveitis = blindness
localised or generalised growth failure
in what types of JIA is ANCA positive?
mainly pauciarticular/oligoarticular
to a lesser degree also present in polyarticular JIA
negative in systemic and enthesitis
ANCA isn’t diagnostic but if absent doesn’t rule it out either
