Juvenile idiopathic arthritis

Question 1

What is JIA?

What can it cause in young people as well?

Answer 1

Group of systemic inflammatory disorders affecting children below age of 16 years.
The most commonly diagnosed Rheumatic disease in children.
An important cause of disability and blindness.

Question 2

history and pathogenesis

• what may affect the immune response?

Answer 2

auto immune
- Etiopathogenesis is multi-factorial and different from that of adult RA.

• Strong subset-specific genetic markers may affect immune response.

Question 3

Most common cause for physical disability ?

Answer 3

JIA

Question 4

Etiology and Pathogenesis
Age of onset?
Duration of disease?
Presence of arthritis?

Answer 4

<16 years

> 6 weeks

Joint swelling or 2 of the following:
Painful or limited joint motion
Tenderness
Warmth

Question 5

Clinical subtypes of JIA

-most common subtype?

Answer 5

Pauciarticula

Question 6

most common subtype divided into

Answer 6

Rheumatoid factor positive and negative

Question 7

all subtypes are greater in females apart from what one?

Answer 7

enthesitis related

Question 8

Pauciarticular JIA (4 or less joints) - describe the 3 types - type 1

Answer 8

Majority of pauci (25%)
Age: before 5 years, peak 1-3 years
Girls: boys = 8:1

Question 9

Presentation of children with arthritis

Answer 9

• limp rather than pain

No constitutional manifestations

Question 10

Pauciarticular JIA (4 or less joints) - what joints are involvement? - type 1

Answer 10

Mainly LL joints

Knee> ankle> hand or elbow (hip very rare).

Question 11

What is positive in Pauciarticular JIA - type 1

• what is common that can be asymptomatic?

Answer 11

ve ANA in 40-75%

Chronic uveitis in 20% of cases (95% if female < 2years old)
Asymptomatic in 50%
Irregular iris due to posterior synechiae (get eyes looked at)

Question 12

Pauciarticular JIA - type 2

• what joints are affected?

Answer 12

More common in boys
can get acute episodes

Mainly LL joints: knee, ankle.
Hip can be affected early with rapid damage requiring THR early in life + enthesitis + many have sacroilliac joints and may evolve AS or spondyloarthritis
20% difficult to classify to particular spondyloarthropathy group.

Question 13

Pauciarticular JIA - type 2 - those who lack back problems called?

slightly older patients

Answer 13

into sero-ve enthesopathy arthropathy (SEA) syndrome who less frequently inherit HLA-B27

Question 14

Those with HLA-B27 + inflammatory back symptoms can be termed as having?

Answer 14

Juvenile Ankylosing spondylitis.

Question 15

Pauciarticular JIA - type 3 - features - what can it be associated with?

younger patients

Answer 15

constitutional rare
asymmetric UL and LL arthritis
dactylitis.

Arthritis can be very destructive
Family history of psoriasis in 40%
+/- nail pitting
These patients may develop psoriasis later in life

Question 16

Pauciarticular JIA - type 3 - what can they develop

Answer 16

Chronic iridocyclitis in 10-20%.

Question 17

Pauciarticular JIA - what can they go on to develop?

Answer 17

Extended oligoarthritis:

30% of these presenting with pauciarticular JIA can go on to more severe polyarticular course.

Question 18

WHta is dactylitis?

Answer 18

nail bed
linked with psoriasis
- fungal infections

Question 19

Polyarticular JIA (5 or more joints) - rheumatoid factor negative?

Answer 19

girls more than boys

- early age

Question 20

Polyarticular JIA (5 or more joints) - rheumatoid factor negative - presentation

Answer 20

Constitutional manifestations (low grade fever, malaise)
Hepato-splenomegaly
Mild anemia
Growth abnormalities

Symmetric large and small joints affection: knees, wrists, ankles, MCPs, PIPs, neck

Question 21

What is rare in Polyarticular JIA negative

Answer 21

Iridocyclitis rare.

Question 22

Polyarticular JIA (5 or more joints) - rheumatoid factor positive - presentation

Answer 22

later years

Presentation:
Constitutional manifestations (low grade fever, malaise, weight loss)
Anemia
Nodules.

Question 23

Systemic complications of Polyarticular JIA (5 or more joints) - rheumatoid factor positive

Answer 23

Can be complicated by sjogren’s, Felty or vasculitis, AR, pulmonary fibrosis, AAS, CTS.
Similar to adult RA but in a child
Erosions in x ray occur early

Question 24

Systemic onset JIA - what defines ?

Answer 24

Extra-articular features define the disease

Question 25

Systemic onset JIA - presentation

Answer 25

fever that rises daily and goes back to normal - presentationt for 2 weeks Rise to 39.5 C daily for at least 2 weeks Late in afternoon or evening and returns to normal or subnormal in the morning Child appears toxic with fever +/- chills but looks normal when fever goes away

Question 26

Systemic onset JIA - what is there a typical clinical sign of? GIVE features

Answer 26

``` RASH 90% Evanescent salmon red eruption On trunk and thighs Accompanies fever Can be brought by scratching (+ve Koebner’s phenomenon). ```

Question 27

Systemic features of Systemic onset JIA

Answer 27

``` Generalized lymphadenopathy Hepatosplenomegaly 50-75% Abdominal pain +/- transaminases - Polyserositis Pericarditis in 36% Tamponade and myocarditis rare ``` Rare Pleural effusion Pulmonary fibrosis

Question 28

Systemic onset JIA - what is the arthritis like when does it present? joints affected?

Answer 28

poly arthritis Within 3-12 months of onset of fever Wrists, knees, ankles, cervical spine, hips and TMJ.

Question 29

Important clinical sign

Answer 29

loss of hyper mobility

Question 30

What should you detect in children with arthritis?

Answer 30

jaw movement - jaw ma be pulled towards the affected side

Question 31

When are there higher levers of uveitis (2)

Answer 31

oligo-articular and extended oligo groups - related to ANA positivity in EOIJA

Question 32

Uveitis complications (3) lots of patients will develop it

Answer 32

potential blindness - can affect all groups early in the disease course - posterior synechiae - iris sticks onto the Lens - cataract - band keratopathy - glaucoma - eye surgery - Sicca syndrome - dry eyes

Question 33

Treatment and management of uveitis

Answer 33

screening!!! - all age groups treatment: - steroids : typical: intraoccular - mydriatic agents/cycloplegic agents - Methotrexate - MMF - Ciclosporin Anti-TNF

Question 34

1st line therapy? some features you must consider with NSAIDS

Answer 34

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

Question 35

2nd line therapy in children? what drugs do you use (1-4) when are these drugs used? when are these drugs rarely needed?

Answer 35

Methotrexate (pharmocokinetics is age related). (2) Anti-TNF Rx. (all 3): In methotrexate failure. (3) IL-1 R-antagonist (Anakinra) in refractory systemic arthritis. (4) IL-6 antagonist (Tocilizumab) for refractory systemic disease If no response to NSAIDs/ joint (steroid) injections. - Rarely needed in oligoarticular JIA

Question 36

Systemic steroids When are they used?

Answer 36

Limited indications due to serious side effects - Systemic JIA (control pain and fever) - Serious disease complications with any subtype e.g. periocordial effusion, tamponade, vasculitis, severe auto-immune anemia, severe aye disease - As a bridge between DMARDs - Children undergoing surgery

Question 37

What are the risks of systemic steroids?

Answer 37

of osteoporosis, infections, growth abnormalities

Question 38

Local steroids are used mainly in? how are they used?

Answer 38

- Oligo-articular JIA | - TOPICALLY for the Eye disease (ANA +ve oligo-articular disease).

Question 39

Surgical treatment (2)

Answer 39

- Synovectomy | - Reconstructive / joint replacement surgery

Question 40

Growth failure in JIA? the 2 types and features of both

Answer 40

localised - leg length discrepancies, shortening of fingers, hands, forearms, toes and feet, JAW - Mircrognathia (poor growth of the mandible) - receding chin Generalised - related to severe systemic - short stature - delayed puberty - systemic steroids

Question 41

Mircrognathia - often need (3)

Answer 41

orthodontic intervention receding chin - TMJ resistant = vigilant in MRI scanning, intra- ocular steroids to prevent growth failure

Question 42

What is not a diagnostic factor?

Answer 42

ANA - important to do it - helps to categorise their arthritis as much as possible