Juvenile Idiopathic Arthritis

Question 1

what is JIA?

Answer 1

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

Question 2

diagnosis of JIA

Answer 2

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

Question 3

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

Answer 3

pauciarticular 55%
polyarticular 25%
systemic onsent 20%

Question 4

what is pauciarticular JIA?

Answer 4

4 or less joints

Question 5

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

Answer 5

polyarticular JIA

Question 6

type 1 pauciarticular JIA: age of onset

Answer 6

<5

peak 1-3

Question 7

type 1 pauciarticular JIA: M:F

Answer 7

1:8

Question 8

type 1 pauciarticular JIA: presentation

Answer 8

limp rather than pain

no constitutional manifestations

Question 9

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

Answer 9

25%

Question 10

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

Answer 10

40-75

Question 11

type 1 pauciarticular JIA: joints affected

Answer 11

knee > ankle > hand or elbow

rarely hip

Question 12

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

Answer 12

20%

95% if female < 2

Question 13

type 1 pauciarticular JIA: is asymptomatic in how many?

Answer 13

50%

Question 14

type 1 pauciarticular JIA: what causes the irregular iris?

Answer 14

poster synechiae

Question 15

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

Answer 15

15%

Question 16

type 3 pauciarticular JIA: age

Answer 16

any age during childhood

Question 17

type 3 pauciarticular JIA: M”F

Answer 17

1:4

Question 18

type 3 pauciarticular JIA: presentation

Answer 18

consitutional
asymmetric UL and LL arthritis
dactylitis

Question 19

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

Answer 19

psoariasis

Question 20

type 3 pauciarticular JIA: chronic iridocyclitis develops what %

Answer 20

10-20

Question 21

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

Answer 21

15%

Question 22

type 2 pauciarticular JIA: age

Answer 22

8-9

Question 23

type 2 pauciarticular JIA: M:F

Answer 23

7:1

Question 24

type 2 pauciarticular JIA: presentation

Answer 24

consitutional rare

limp due to LL

Question 25

type 2 pauciarticular JIA: joints

Answer 25

knee ankle hip

Question 26

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

Answer 26

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

Question 27

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

Answer 27

juvenile ankylosing spondylitis

Question 28

type 2 pauciarticular JIA: acute iridocylitis in what %

Answer 28

10-20%

Question 29

define polyarticular JIA

Answer 29

5 or more joints

Question 30

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

Answer 30

15%

Question 31

RF -ve polyarticular JIA: age

Answer 31

any but often early

Question 32

RF -ve polyarticular JIA: M:F

Answer 32

1:9

Question 33

RF -ve polyarticular JIA: joints affected

Answer 33

``` symmetric large and small joints knees wrists ankles MCPs PIP neck ```

Question 34

RF -ve polyarticular JIA: presenation

Answer 34

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

Question 35

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

Answer 35

10%

Question 36

RF +ve polyarticular JIA: age

Answer 36

late childhood 12-16

Question 37

RF +ve polyarticular JIA: M:F

Answer 37

1:7

Question 38

RF +ve polyarticular JIA: presentation

Answer 38

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

Question 39

RF +ve polyarticular JIA: can be complicated by

Answer 39

``` Sjogren's Felty vasculitis AR pulmonary fibrosis AAS CTS ```

Question 40

systemic onset JIA =

Answer 40

STILLs disease

Question 41

STILL's disease: makes up what % of JIA

Answer 41

20%

Question 42

STILL's disease: age

Answer 42

4-6 but throughout childhood

Question 43

STILL's disease: M:F

Answer 43

1:1.5

Question 44

STILL's disease: what defines the disease?

Answer 44

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

Question 45

STILL's disease: fever

Answer 45

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

Question 46

STILL's disease: rash

Answer 46

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

Question 47

STILL's disease: lymph nodes

Answer 47

50-75% generalised lymphadenopathy non-tender

Question 48

STILL's disease: abdominal

Answer 48

hepatosplenomegaly 50-70% abdo pain +/- transaminases

Question 49

STILL's disease: serositis

Answer 49

polyserositis pericarditis in 36% tamponade and myocarditis rare

Question 50

STILL's disease: pulmonary

Answer 50

rare pleural effusion pulmonary fibrosis

Question 51

STILL's disease: arthritis

Answer 51

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

Question 52

management of JIA: 1st line therapy

Answer 52

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

Question 53

management of JIA: 2nd line therapy

Answer 53

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)

Question 54

non-drug treatment of JIA

Answer 54

PT OT synovectomy reconstructive/joint replacement surgery