Juvenile Idiopathic Arthritis

Question 1

Whats makes an arthritis JIA? [2]

Answer 1

Systemic inflammatory disorders

Has to be <16yrs old

Question 2

Diagnostic criteria for JIA [3 components]

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 which are only determinable after 6 months)?

Answer 3

Pauciarticular (55%) - <5 joints
Polyarticular (25%) - >4 joints
Systemic Onset (20%) - Still's Disease

Question 4

There are 3 subtypes of Pauciarticular JIA (Type 1, 2, 3). How does Type 1 present?
Gender, age, sites
2 features

Answer 4

Female dominant
Peaks at 1-3yrs.
Limp due to LL arthritis
20% also get chronic uveitis

Question 5

How can we test for Type 1 Pauciarticular JIA? [1]

Answer 5

40-75% are ANA +ve

Question 6

How does Type 2 Pauciarticular JIA present?
Gender and demographic
2 features
NB Watch out for hip or back involvement, while rare it can progress rapidly to AS (worth testing for HLA-B27)

Answer 6

Pre-school boys >8 yo

Limp due to LL arthritis

10-20% get Acute Iridocyclitis

Question 7

How Does Type 3 Pauciarticular JIA present?
Gender, age
4 features

Answer 7

Female Predominant
Any age during childhood

Asymmetric UL and LL arthritis
Dactylitis
\+/- Nail pitting
Psoriasis
40% have FH/o Psoriasis, develops later in life

Question 8

What are the subtypes of Polyarticular JIA? [2]

NB in both iridocyclitis is rare

Answer 8

RF -ve (15%)

RF +ve (10%

Question 9

How does RF -ve Polyarticular JIA present?
Gender 
Main presenting complaint
Constitutional manifestations [2]
Signs [3]

Answer 9

Female predominant, any age

•Symmetric large and small joint effusions
•Constitutional manifestations
	§ Low grade fever
	§ Malaise
• Hepato-splenomegaly
• Mild anemia
• Growth abnormalities

Question 10

How does RF +ve Polyarticular JIA present?
Gender, age
Main presenting complaint +
Systemic sx [4]
Complications [5]
X-ray signs

Answer 10

Female mostly, 12-16yrs

5 or more joints + systemic features:

• Anaemia
• Low grade fever
• Malaise
• Rheumatoid Nodules

Can be complicated by:

• Secondary Sjogren’s
• Atlanto-Axial subluxation
• Pulm. Fibrosis
• Felty’s disease
• Cubital tunnel syndrome

• X-ray erosion seen early

Question 11

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

Answer 11

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

Question 12

Who gets Still’s Disease?

Answer 12

1.5 F:M, mostly 4-6yrs old

Question 13

Still’s disease fever [2]

Answer 13

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

Question 14

50-75% of Still’s Patients get Abdominal features such as… [3]
2 symptoms and 1 sign

Answer 14

Hepatosplenomegaly
Abdo Pain
~Raised Transaminases

Question 15

Lymphadenopathy problems in 50-75% of still’s patients:

2 features

Answer 15

Non-tender and generalized

Question 16

Describe Still’s rash (90%) [1]

Name the phenomenon associated with this rash

Answer 16

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

Question 17

Pulmonary sx in Still’s patients are rare [2]

Answer 17

Pleural effusion

Pulm. Fibrosis

Question 18

Inflammation of Serous membranes can come with Still’s Disease, what type is most common?
Give 2 rare complications assoc with inflammation of serous membranes

Answer 18

36% of patient’s get Polyserositis including pericarditis

Rarely they can get tamponade and myocarditis

Question 19

How do the actual arthritis symptoms appear in Still’s Disease? (Name 6 sites)

Answer 19

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

Question 20

Many types of JIA can present with Eye symptoms such as uveitis, these are often asymptomatic but can lead to blindness, how do we respond?

Answer 20

Every JIA patient gets regular Ophthalmology Screenings (PArticularly those with Pauciarticular JIA)

Question 21

1st Line treatment of JIA? [3]
Treatment for pauciartuclar
Treatment for polyarticular

Answer 21

Simple analgesia
NSAIDs
Intra-articular steroids for single joint involvement
Systemic steroids for polyarticular

Question 22

What if NSAIDs and local steroids fail? [4] options

Answer 22

Methotrexate
Anti-TNF - infliximab
IL-1R antagonist
IL-6 antagonist tocilizumab

Question 23

How do we treat Refractory Systemic Arthritis?

Answer 23

IL-1 Receptor Antagonist - Anakinra

IL-6 Antagonist (Tocilizumab)

Question 24

When would we risk using systemic steroids? [3]

- Serious disease complications [5]

Answer 24

Systemic JIA to control pain and fever

Bridge between DMARDs

Surgery

Serious disease complications in JIA e.g. pericardial effusion, tamponade, vasculitis, severe anaemia or severe eye disease

Question 25

What are the risks of systemic steroids?

Answer 25

Osteoporosis
Infection
Growth abnormality

Question 26

Major difference between pauciarticular and polyarticular JIA in presentation

Answer 26

Rare constitutional manifestations in pauci vs

poly

Question 27

Surgical management of JIA involves [2]

Answer 27

• Surgical mx
○ Synovectomy
Reconstructive/joint replacement surgery

Question 28

Examinations [2]
Investigations
No specific tests for JIA 
But what lab investigations can you consider [4]
What imaging techniques can you do [2]

Answer 28

pGALS as screening measure and pREMS
ANA
HLA-B27
Inflammatory markers
RF
X-ray, MRI

Question 29

Management approach
Do’s [2]
Dont’s [3]

Answer 29

Do - intensify treatment, remain active
Don't:
- Advise rest
- Splints, crutches, wheelchairs
- Accept less than normal function