Juvenile/Idiopathic Arthritis

Question 1

Juvenile idiopathic arthritis (JIA) is a chronic autoimmune disease that affects children. It is characterised by joint inflammation, pain, and stiffness that can lead to joint damage and disability if left untreated. What is the estimated incidence of JIA?

1 - 2 cases per 100,000
2 - 20 cases per 100,000
3 - 200 cases per 100,000
4 - 2000 cases per 100,000

Answer 1

1 - 2 cases per 100,000

More common in females

Question 2

What age does Juvenile idiopathic arthritis (JIA) typically present?

1 - 0-12 months
2 - 5-10
3 - 6-15
4 - 10-25

Answer 2

3 - 6-15

Question 3

Juvenile idiopathic arthritis (JIA) is diagnosed where there is arthritis without any other cause, lasting how long?

1 - >2 weeks in <16
2 - >6 weeks in <16
3 - >12 weeks in <16
4 - >12 months in <16

Answer 3

2 - >6 weeks in <16

Question 4

Which of the following is NOT a key clinical feature of Juvenile idiopathic arthritis (JIA)?

1 - joint pain
2 - swelling
3 - fragility fractures
4 - stiffness

Answer 4

3 - fragility fractures

Question 5

There are 5 main types of Juvenile idiopathic arthritis (JIA). Systemic JIA, also known as Still’s disease is a systemic illness that can occur throughout childhood in boys and girls. It is an idiopathic inflammatory condition. Which of the following are typical features of this condition?

1 - Subtle salmon-pink rash
2 - High swinging fevers
3 - Enlarged lymph nodes
4 - Weight loss
5 - Joint inflammation and pain
6 - Splenomegaly
7 - Muscle pain
8 - Pleuritis and pericarditis
9 - all of the above

Answer 5

9 - all of the above

Question 6

In Systemic Juvenile idiopathic arthritis (JIA), are anti-nuclear antibodies and RF typically positive or negative?

Answer 6

• both are negative

Question 7

In Systemic Juvenile idiopathic arthritis (JIA), all of the following are commonly raised, EXCEPT which one?

1 - ESR
2 - WBCs
3 - CRP
4 - platelets
5 - ferritin

Answer 7

2 - WBCs

Question 8

In Systemic Juvenile idiopathic arthritis (JIA), a key complication is macrophage activation syndrome (MAS). This is due to a severe activation of the immune system with a massive inflammatory response. It presents with all of the following EXCEPT which feature?

1 - acutely unwell child
2 - disseminated intravascular coagulation
3 - anaemia
4 - thrombocytopenia
5 - bleeding
6 - non-blanching rash
7 - high ESR

Answer 8

7 - high ESR

Typically presents with a low ESR
MEDICAL EMERGENCY

Question 9

If a child presents with a salmon-pink rash, fevers, joint pain and fevers for more than 5 days, which of the following non-infective differentials should be considered?

1 - Kawasaki disease
2 - Still’s disease (Systemic Juvenile idiopathic arthritis (JIA)
3 - Rheumatic fever
4 - Leukaemia
5 - all of the above

Answer 9

5 - all of the above

Question 10

Polyarticular Juvenile idiopathic arthritis (JIA) involves inflammatory arthritis in 5 or more joints. Does this typically present symmetrical or asymmetrically?

Answer 10

• symmetrical
  Typically both sides are affected

This is the RA in children

Question 11

Polyarticular Juvenile idiopathic arthritis (JIA) involves inflammatory arthritis in 5 or more joints, commonly symmetrically. Is this confined to small or large joints?

Answer 11

Can affect all joints including small joints of hands and feet to hips and and knees

Question 11

Polyarticular Juvenile idiopathic arthritis (JIA) involves inflammatory arthritis in 5 or more joints, commonly symmetrically. Systemic symptoms are not common, but can present with all of the following EXCEPT which one?

1 - high swinging fever
2 - anaemia
3 - reduced growth

Answer 11

1 - high swinging fever
Typically causes a mild fever

Systemic symptoms are typically mild

Question 11

Polyarticular Juvenile idiopathic arthritis (JIA) involves inflammatory arthritis in 5 or more joints, commonly symmetrically. This is often referred to as the RA of children. Is RF always positive?

Answer 11

• no

Most children are negative (seronegative)
However, some children are RF positive (seropositive) and are typically older

Question 11

Oligoarticular Juvenile idiopathic arthritis (JIA) is the most common form of JIA, typically affecting 4 joints or less. Does this commonly affect more or multiple joints?

Answer 11

• Commonly just one joint
  Typically called monoarthritis and affects the knee or ankle

BUT can affect 4 or less

Question 12

Oligoarticular Juvenile idiopathic arthritis (JIA) is the most common form of JIA, typically affecting 4 joints or less. Does this occur more commonly in boys or girls?

Answer 12

• girls

Commonly aged <6 years old

Question 13

Oligoarticular Juvenile idiopathic arthritis (JIA) is the most common form of JIA, typically affecting 4 joints or less. Which is the classic associated feature of this?

1 - lung fibrosis
2 - anterior uveitis
3 - finger clubbing
4 - mouth and genital ulcers

Answer 13

2 - anterior uveitis

Patients should be referred to ophthalmologist for treatment

Question 14

Oligoarticular Juvenile idiopathic arthritis (JIA) is the most common form of JIA, typically affecting 4 joints or less. Do patients with this condition have any systemic symptoms?

Antinuclear antibodies are often positive, however rheumatoid factor is usually negative.

Answer 14

• no

Inflammatory markers are mildly raised or normal

Question 15

Oligoarticular Juvenile idiopathic arthritis (JIA) is the most common form of JIA, typically affecting 4 joints or less. Inflammatory markers can be mild elevated or normal. Is anti-nuclear antibodies or RF positive?

Answer 15

• anti-nuclear antibodies are commonly positive
• RF is typically negative

Question 16

Entheses is the anatomical junction where connective tissue (e.g. ligament, tendon, joint capsule, bursa or a combination thereof) attaches to bone. Enthesitis-Related Arthritis is arthritis causing inflammation of the entheses. Is this more common in boys or girls?

Answer 16

• Boys aged >6 years old

Commonly referred to as the paediatric version of seronegative spondyloarthropathy group of conditions that affect adults, such as:

• ankylosing spondylitis
• psoriatic arthritis
• reactive arthritis
• inflammatory bowel disease-related arthritis

Question 17

Entheses is the anatomical junction where connective tissue (e.g. ligament, tendon, joint capsule, bursa or a combination thereof) attaches to bone. Enthesitis-Related Arthritis is arthritis causing inflammation of the entheses. Which of the following is commonly positive in Enthesitis-Related Arthritis?

1 - HLA-B27
2 - RF
3 - HLA-DQ2
4 - HLA-DR3

Answer 17

1 - HLA-B27

Question 18

Entheses is the anatomical junction where connective tissue (e.g. ligament, tendon, joint capsule, bursa or a combination thereof) attaches to bone. Enthesitis-Related Arthritis is arthritis causing inflammation of the entheses. When examining a patient with this, all of the following should be looked for EXCEPT which one (or at least the least likely to be present)?

1 - ankylosing spondylitis
2 - anterior uveitis
3 - osteoarthritis
4 - psoriasis
5 - IBD

Answer 18

3 - osteoarthritis

Question 19

Entheses is the anatomical junction where connective tissue (e.g. ligament, tendon, joint capsule, bursa or a combination thereof) attaches to bone. Enthesitis-Related Arthritis is arthritis causing inflammation of the entheses. Which of the following are commonly affected?

1 - Interphalangeal joints in the hand
2 - Wrist
3 - greater trochanter of femur
4 - Quadriceps insertion at the ASIS
5 - Quadriceps and patella tendon insertion around the patella
6 - Base of achilles
7 - Metatarsal heads on the base of the foot
8 - all of the above

Answer 19

8 - all of the above

Question 20

Juvenile Psoriatic Arthritis is another form of Juvenile idiopathic arthritis (JIA) commonly associated with psoriasis. The pattern of joint involvement varies. Does this always present with symmetrical or asymmetrical joints affected?

Answer 20

• Both
• symmetrical polyarthritis affecting the small joints similar to rheumatoid

OR

• asymmetrical arthritis affecting the large joints in the lower limb

Question 21

Juvenile Psoriatic Arthritis is another form of Juvenile idiopathic arthritis (JIA) commonly associated with psoriasis. The pattern of joint involvement varies. Which of the following are features that should be examined if this diagnosis is suspected?

1 - Plaques of psoriasis on the skin
2 - Pitting of the nails (nail pitting)
3 - Onycholysis, separation of the nail from the nail bed
4 - Dactylitis, inflammation of the full finger
5 - Enthesitis, inflammation of the entheses
6 - all of the above

Answer 21

6 - all of the above

Question 22

The management of all Juvenile idiopathic arthritis (JIA) should come under a rheumatologist. Which of the following is typically 1st line?

1 - NSAIDs, such as ibuprofen
2 - Steroids, either oral, intramuscular or intra-artricular in oligoarthritis
3 - Disease modifying anti-rheumatic drugs (DMARDs), such as methotrexate, sulfasalazine and leflunomide
4 - Biologic therapy, such as the tumour necrosis factor inhibitors etanercept, infliximab and adalimumab

Answer 22

1 - NSAIDs, such as ibuprofen

Steroids can be effective during a flare up

DMARDs are long term medications when NSAIDs become infective or if joint damage is suspected

Biologics are used when at least 2 DMARDs have been tried