Juvenile Idiopathic Arthritis Flashcards

1
Q

what is JIA?

A

JIA is a group of systemic inflammatory disorders affecting children below the age of 16.

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2
Q

diagnosis of JIA

A
<16, smyptoms > 6weeks
presence of arthritis = joint swelling or 2 of:
painful or limited joint motion
tenderness
warmth
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3
Q

after 6 months, 3 major subtypes of JIA can be identified. Name them and their relative %

A

pauciarticular 55%
polyarticular 25%
systemic onsent 20%

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4
Q

what is pauciarticular JIA?

A

4 or less joints

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5
Q

30% of those with pauciarticular JIA will go on to develop what?

A

polyarticular JIA

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6
Q

type 1 pauciarticular JIA: age of onset

A

<5

peak 1-3

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7
Q

type 1 pauciarticular JIA: M:F

A

1:8

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8
Q

type 1 pauciarticular JIA: presentation

A

limp rather than pain

no constitutional manifestations

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9
Q

type 1 pauciarticular JIA: makes up what % of JIA?

A

25%

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10
Q

type 1 pauciarticular JIA: ANA are +ve in what %

A

40-75

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11
Q

type 1 pauciarticular JIA: joints affected

A

knee > ankle > hand or elbow

rarely hip

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12
Q

type 1 pauciarticular JIA: chronic uveitis develops in how many?

A

20%

95% if female < 2

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13
Q

type 1 pauciarticular JIA: is asymptomatic in how many?

A

50%

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14
Q

type 1 pauciarticular JIA: what causes the irregular iris?

A

poster synechiae

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15
Q

type 3 pauciarticular JIA: makes up what % of JIA?

A

15%

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16
Q

type 3 pauciarticular JIA: age

A

any age during childhood

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17
Q

type 3 pauciarticular JIA: M”F

A

1:4

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18
Q

type 3 pauciarticular JIA: presentation

A

consitutional
asymmetric UL and LL arthritis
dactylitis

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19
Q

type 3 pauciarticular JIA: 40% have a FHx of?

A

psoariasis

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20
Q

type 3 pauciarticular JIA: chronic iridocyclitis develops what %

A

10-20

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21
Q

type 2 pauciarticular JIA: makes up what % of JIA?

A

15%

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22
Q

type 2 pauciarticular JIA: age

A

8-9

23
Q

type 2 pauciarticular JIA: M:F

A

7:1

24
Q

type 2 pauciarticular JIA: presentation

A

consitutional rare

limp due to LL

25
Q

type 2 pauciarticular JIA: joints

A

knee
ankle
hip

26
Q

type 2 pauciarticular JIA: what can happen if the hip is affected

A

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

27
Q

type 2 pauciarticular JIA: those with HLA-27 _ back involvement will be classified as what instead?

A

juvenile ankylosing spondylitis

28
Q

type 2 pauciarticular JIA: acute iridocylitis in what %

A

10-20%

29
Q

define polyarticular JIA

A

5 or more joints

30
Q

RF -ve polyarticular JIA: makes up what % of JA

A

15%

31
Q

RF -ve polyarticular JIA: age

A

any but often early

32
Q

RF -ve polyarticular JIA: M:F

A

1:9

33
Q

RF -ve polyarticular JIA: joints affected

A
symmetric large and small joints
knees
wrists
ankles
MCPs
PIP
neck
34
Q

RF -ve polyarticular JIA: presenation

A

constitutional manifestations - low grade fever, malise
hepato-splenomegaly
mild anaemia
growth abnormalitis

35
Q

RF +ve polyarticular JIA: makes up what % of JIA

A

10%

36
Q

RF +ve polyarticular JIA: age

A

late childhood 12-16

37
Q

RF +ve polyarticular JIA: M:F

A

1:7

38
Q

RF +ve polyarticular JIA: presentation

A

constitutional symptoms - low grade fever, malaise, weight loss
anaemia
nodules

39
Q

RF +ve polyarticular JIA: can be complicated by

A
Sjogren's
Felty
vasculitis 
AR
pulmonary fibrosis
AAS
CTS
40
Q

systemic onset JIA =

A

STILLs disease

41
Q

STILL’s disease: makes up what % of JIA

A

20%

42
Q

STILL’s disease: age

A

4-6 but throughout childhood

43
Q

STILL’s disease: M:F

A

1:1.5

44
Q

STILL’s disease: what defines the disease?

A

extra articular features - start early disappear after 2-5 years

45
Q

STILL’s disease: fever

A

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

46
Q

STILL’s disease: rash

A
90%
evanescent salmon red eruption
on trunk and thighs
acompanies fever
can be brought by stratchin
47
Q

STILL’s disease: lymph nodes

A

50-75%
generalised lymphadenopathy
non-tender

48
Q

STILL’s disease: abdominal

A

hepatosplenomegaly
50-70%
abdo pain
+/- transaminases

49
Q

STILL’s disease: serositis

A

polyserositis
pericarditis in 36%
tamponade and myocarditis rare

50
Q

STILL’s disease: pulmonary

A

rare
pleural effusion
pulmonary fibrosis

51
Q

STILL’s disease: arthritis

A

75%
within 3-12 months of onset of fever
wrists, knees, ankles, c-spine, TMJ, hips

52
Q

management of JIA: 1st line therapy

A
o Simple pain killers
o NSAIDs
§ Difference between adults and children in half life
§ Can control disease
§ Doses
§ Same compounds only
53
Q

management of JIA: 2nd line therapy

A

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)

54
Q

non-drug treatment of JIA

A

PT
OT
synovectomy
reconstructive/joint replacement surgery