Paeds rheum

Question 1

List 4 non infective causes of a fever lasting > 5 days

Answer 1

• Kawasaki disease
• Still’s disease
• Rheumatic fever
• Leukaemia

Question 2

What is another name for systemic JIA?

Answer 2

Still’s disease

Question 3

Child B presents with a salmon pink rash, fevers and joint pain. What’s the likely diagnosis?

Answer 3

Still’s disease

Question 4

What is a complication of Still’s disease? What is the key investigation finding?

Answer 4

Macrophage activation syndrome. Low ESR

Question 5

What 3 key things are required for diagnosing JIA?

Answer 5

• Arthritis without a cause
• Lasting more than 6 wks
• In someone < 16 years old

Question 6

How many joints need to be affected to diagnose polyarticular JIA?

Answer 6

5 or more joints

Question 7

Oligoarticular JIA:
1. How many joints are affected?
2. Typical gender and age?
3. What features is classical associated?

Answer 7

1. 4 or less
2. Girls under 6
3. Anterior uveitis

Question 8

Enthesitis related arthritis:
- Typical gender and age?
- What is this the ‘paediatric version of’?
- What gene is it commonly associated with?

Answer 8

1. Boys over 6
2. Seronegative spondyloarthropathy
3. HLA B27

Question 9

Give 4 types of medication used to manage JIA + examples

Answer 9

1. NSAIDs (ibuprofen)
2. Steriods (prednisolone/hydrocortisone)
3. DMARDs (methotrexate/sulfasalazine/leflunomide)
4. Biologics (infliximab/adalimumab)

Question 10

What gender and age does Kawasaki typically affect?

Answer 10

Boys under 5

Question 11

What type of vasculitis is Kawasaki?

Answer 11

Systemic, medium vessel vasculitis

Question 12

What is the management of Kawasaki

Answer 12

High dose aspirin and IVIG

Question 13

What is the role of IVIG in Kawasaki?

Answer 13

To prevent coronary artery aneurysms

Question 14

Why is aspirin normally avoided in children?

Answer 14

Risk of Reye syndrome - swelling in the liver and brain

Question 15

What are typical features of Kawasaki disease?

Answer 15

• Fever > 5 days
• Widespread erythematous maculopapular rash
• Desquamation on palms and soles
• Strawberry tounge
• Conjunctivitis
• Lymphadenopathy

Question 16

What is Henoch-Schonlein Purpura?

Answer 16

An IgA vasculitis

Question 17

What parts of the body are affected in HSP?

Answer 17

The skin, kidneys and GI tract

Question 18

What are classic features of HSP?

Answer 18

• Purpura
• Joint pain
• Abdominal involvement
• IgA nephritis

Question 19

What is the management of HSP?

Answer 19

• Supportive
• Monitor BP for hypertension
• Urine dipstick to monitor renal involvement

Question 20

How many patients have a reoccurrence of HSP within 6 months?

Answer 20

1/3rd

Question 21

What is the most common type of Ehlers-Danlos syndrome?

Answer 21

Hypermobile Ehlers-Danlos

Question 22

What type of Ehlers-Danlos is associated with abnormal wound healing?

Answer 22

Classical Ehlers-Danlos

Question 23

What score can be used to assess hyper mobility? What do you get points for?

Answer 23

Beighton Score (one point score for each side of body, max nine):
- Palms flat on floor with straight legs
- Elbows hyperextended
- Knees hyperextended
- Them can bend to forearm
- Little finger hyperextends past 90 degrees

Question 24

What is rheumatic fever?

Answer 24

An autoimmune condition in response to strep infection in which antibodies against the bacteria also attack the joints, heart and skin

Question 25

What criteria is used to diagnose rheumatic fever?

Answer 25

Jones criteria

Question 26

According to the Jones Criteria, which is required to diagnoses rheumatic fever

Answer 26

• Evidence of recent strep infection and…
• Two major criteria or…
• One major and two minor criteria

Question 27

What are major features according to Jones Criteria?

Answer 27

JONES
- Joint arthritis
- Organ inflammation
- Nodules
- Erythema marginatum rash
- Sydenham chorea

Question 28

What are minor features according to Jones Criteria?

Answer 28

FEAR
- Fever
- ECG changes
- Arthralgia without arthritis
- Raised inflammatory markers

Question 29

Which valvular heart disease is most likely to be caused by rheumatic fever?

Answer 29

MS

Question 30

What is the follow up for HSP?

Answer 30

BP and early morning dipstick at..
- 1 and 2 weeks
- 1, 3 and 6 months

Question 31

What would bloods show in JIA?

Answer 31

• Raised inflammatory markers (ESR/CRP)
• Negative ANA and rheumatoid factor

Question 32

What part of the skin is spared in HSP?

Answer 32

Trunk is normally spared (from rash)