Juvenile Idiopathic Arthritis Flashcards

1
Q

what is JIA?

A

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

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2
Q

what is the criteria for diagnosis of JIA?

A
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
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3
Q

what are the subtypes of JIA?

A

pauciarticular
polyarticular
systemic onset

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4
Q

what sex is JIA most common in?

A

females

except enthesitis related JIA

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5
Q

what joints/how many does pauciarticular JIA affect?

A

4 joints or less

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6
Q

what are the features of type 1 pauciarticular JIA?

A

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

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7
Q

what are the features of type II pauciarticular uveitis?

A

mainly affects lower limbs
can affect the hip, enthesitis, sacroiliac joints and may develop ankylosing spondylitis / spondyloarthritis
acute iridocyclitis

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8
Q

what is juvenile ankylosing spondylitis

A

if a patient has type II pauciarticular JIA, HLA B27 ve+ plus back involvement they are termed as having juvenile ankylosing spondylitis

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9
Q

what are the features of type III pauciarticular JIA?

A

asymmetric upper and lower limb involvement
dactylitis
family history of psoriasis - may develop psoriasis later on
chronic iridiocycilitis

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10
Q

what are the 2 subtypes of polyarticular JIA?

A

RF negative

RF positive

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11
Q

at what age of onset if RH ve- polyarticular JIA most common?

A

can occur at any age in childhood

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12
Q

what are the features of RF ve- polyarticular JIA?

A
constitutional manifestations (fever, malaise)
hepatosplenomegaly 
mild anaemia
growth abnormalities
symmetric small and large joints
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13
Q

what is the age onset of RF ve+ polyarticular JIA?

A

late childhood (teens 12-16yrs)

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14
Q

what is the presentation of RH ve+ polyarticular JIA?

A

constitutional features (low grade fever, malaise)
anaemia
nodules
similar to RA in adult but in a child

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15
Q

what conditions can complicate RF ve+ polyarticular JIA?

A
sjogrens 
feltys
aortic regurgitation 
pulmonary fibrosis 
acute aortic syndrome 
carpal tunnel syndrome
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16
Q

what is the age of onset for pauciarticular JIA?

A

type 1: < 5 yrs
type 2: > 8/9yrs
type 3: any age

17
Q

what genes are involved with pauciarticular JIA?

A

type 1: ANCA ve+

type 2: HLA B27

18
Q

in what type of JIA do you find dactylitis?

A

type III pauciarthritis

19
Q

what are the features of systemic onset (stills) disease?

A

persistent high spiking fevers
salmon coloured rash
joint pain / arthritis

20
Q

what are other extra articular features of systemic onset JIA (stills) ?

A
pulmonary:
- pleural effusion
- pulmonary fibrosis
stomach;
- hepatosplenomegaly
- abdominal pain 
lymphadenopathy 
rash 
heart;
- pericarditis
- myocarditis
- tamponade
21
Q

what characteristic of the fever in stills disease?

A

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

22
Q

what are the complications of uveitis in JIA?

A

glaucoma
blindness
posterior synechiae
band keratopathy

23
Q

what are the treatment options for uveitis in patients with JIA?

A
steroids
methotrexate
adriatic and cyclopegic agents
cyclosporin 
anti TNF alpha
24
Q

what is the treatment for JIA?

A

1st line; NSAIDS or joint steroid injections

2nd line:

  • methotrexate
  • anti TNF alpha
  • IL-1 R antagonist
  • IL-6 antagonist

surgery

25
Q

when are steroids used to treat JIA ?

A

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+)

26
Q

what are the surgical treatment options for JIA?

A

synovectomy

reconstruction / joint replacement

27
Q

what are the 2 main complications from JIA?

A

anterior uveitis = blindness

localised or generalised growth failure

28
Q

in what types of JIA is ANCA positive?

A

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