Juvenile Idiopathic Arthritis

Question 1

Whats makes an arthritis JIA?

Answer 1

Systemic Inflammatory disorders

Has to be <16yrs old

Question 2

What is required for us to diagnose JIA?

Answer 2

Onset <16yrs
Duration >6wks
Symptoms:
Joint Swelling OR 2 of:
- Tenderness
- Painful/limited ROM
- Warmth

Question 3

There are 3 major subtypes of JIA (only determinable after 6 months)?

Answer 3

Pauciarticular (55%) - <4 joints
Polyarticular (25%) - >4 joints
Systemic Onset (20%) - Still's Disease

Question 4

There are 3 subtypes of Pauciarticular JIA (Type 1, 2 & 3). Here’s some characteristics of 1

Answer 4

• 1-3yrs so tend to present with unusual gait rather than complaining of pain.
• More common in F
• Most commonly in the LL
• 50% asymptomatic

Question 5

What are the subtypes of Polyarticular JIA?

Answer 5

RF -ve (15%)

RF +ve (10%

Question 6

What sets polyarticular apart from pauciarticular (other than more joints)?

Answer 6

Potential Systemic Symptoms:

• Low grade fever
• Malaise &weight loss
• Hepato-splenomegaly- RF -VE
• Mild Anaemia
• Growth abnormalities- IN RF-VE
• Nodules- IN RF +VE

Can be complicated by vasculitis, Sjogren’s or pulm fibrosis

Question 7

Whats special about how Still’s Disease (Systemic onset JIA) presents?

Answer 7

Systemic features start first with arthritis not occuring till 3-12 months in.

Question 8

Who gets Still’s Disease?

Answer 8

1.5F:1M, mostly 4-6yrs old

Question 9

How does Still’s Disease Start?

Answer 9

Fever.
It rises daily in the afternoon/evening for atleast 2 weeks.
Look for a child toxic with fever that goes away in the morning

Question 10

50-75% of Still’s Patients get Abdominal symptoms such as…

Answer 10

Hepatosplenomegaly
Abdo Pain
Raised Transaminases

Question 11

How many stills patients get lymph symptoms?

Answer 11

50-75% geet non-tender Generalised Lymphadenopathy

Question 12

90% of Still’s Patients present with a rash, descirbe it?

Answer 12

Evanescent Salmon Red Eruption on their trunk/thighs along with the fever.
It can be brought on by scratching (aka Koebner’s Phenomenon)

Question 13

Very rarely Still’s Patients can get pulmonary symptoms like..

Answer 13

Pleural effusion

Pulm. Fibrosis

Question 14

Inflammation of Serous membranes can come with Still’s Disease, what types are common?

Answer 14

36% of patient’s get Polyserositis including pericarditis

Rarely they can get tamponade and myocarditis

Question 15

How do the actual arthritis symptoms appear in Still’s Disease?

Answer 15

Start 3-12months after the fever onsets

Wrist, ankle, knee, C-spine, hips & TMJ

Question 16

What tests can we do to diagnose and determine the subtype of JIA?

Answer 16

1) ANA (commonest in pauciarticular)
RF (subtype of polyarticular)
2) ESR/CRP (Most elevated in systemic onset)
3) FBC (May see anaemia, thrombocytosis & leucocytosis. Esp in polyarticular and Systemic onset)
4) Opthalmology screening for uveitis (mostly in pauciarticular)

Question 17

1st Line treatment of JIA?

Answer 17

SImple Pain Killers
NSAIDs
Local Steroids:
- IA in Pauciarticular JIA
- For Eye disease

Question 18

What if NSAIDs & Steroids fail?

Answer 18

Methotrexate

Question 19

What if NSAIDs, steroids and MTX fail?

Answer 19

Anti-TNFalpha - Infliximab

Question 20

How do we treat Refractory Systemic Arthritis?

Answer 20

IL-1 Receptor Antagonist - Anakinra

IL-6 Antagonist (Tocilizumab)

Question 21

When would we risk using systemic steroids?

Answer 21

Systemic JIA
Serious compliated JIA e.g. pericardial effusion, tamponade, vasculitis, severe anaemia or severe eye disease
Bridge between DMARDs
Surgery

Question 22

What are the risks of systemic steroids?

Answer 22

Osteoporosis
Infection
GRowth Abnormality

Question 23

So summarise the likely presentations of Systemic Onset JIA?

Answer 23

1st = Daily rising and falling fever
2nd:
- Generalised Lymphadenoapthy
- Evanescent Salmon red rash
- ~ serositis
- ~ Abdo pain, hepatosplenomegaly & raised transaminases

Then 3-12months later the arthritis starts

Question 24

There are 3 subtypes of Pauciarticular JIA (Type 1, 2 & 3).
type 2

Answer 24

• Can feature hip/back pain which can rarely progress to ankylosing spondylitis (enthesitis and sacroiliac joints involved)
• More common in boys
• 8-9 years

Question 25

There are 3 subtypes of Pauciarticular JIA (Type 1, 2 & 3). :
type 3

Answer 25

• Asymmetrical with psoriasis & Dactylitis

- At any age

Question 26

Differences between RF +VE and -VE

Answer 26

+VE

• Nodules
• -similar to RA: erosions present early on scan
• often presents in teens
• VE:
• hepato-splenomegaly
• often present early

Question 27

what are other treatments available?

Answer 27

REHABILITATION:
• Physiotherapy
• Occupational therapy

Surgery
• Synovectomy
• Reconstructive / joint replacement surgery

Question 28

what are the treatments for uveitis?

why is it important to screen?

Answer 28

Very common in JLA- 15-24% of cases

Treatment:
–	Steroids: Topical / intraocular / systemic 
–	Mydriatic & cycloplegic agents 
–	Methotrexate
–	MMF
–	Ciclosporin
–	Anti-TNF

Question 29

what are some of the complications of uveitis?

Answer 29

• Posterior Synechiae
• Cataract
• Band keratopathy
• Glaucoma
• Visual loss

Question 30

what are the localised and systemic growth failures associated with JLA?

Answer 30

Localised
• Leg length discrepancies
• Shortening of fingers, hands, forearms, toes, feet
• Micrognathia - jaw is undersize

Generalised 
•	related to severe systemic disease
•	short stature
•	delayed puberty
•	Systemic steroids