Revision - JIA

Question 1

What is juvenile idiopathic arthritis (JIA)?

Answer 1

A chronic autoimmune disease that affects children.

Characterised by joint INFLAMMATION, pain, and stiffness that can lead to joint damage and disability if left untreated.

Question 2

When can JIA be diagnosed?

Answer 2

When there is arthritis without any other cause lasting >6 weeks in a patient <16 y/o

Question 3

How long does arthritis have to be present for for diagnosis of JIA?

Answer 3

6 weeks

Question 4

What are the 3 key features of JIA?

Answer 4

1) joint pain
2) swelling
3) stiffness

Question 5

What are the 5 key subtypes of JIA?

Answer 5

1) Systemic JIA
2) Polyarticular JIA
3) Oligoarticular JIA
4) Enthesitis related arthritis
5) Juvenile psoriatic arthritis

Question 6

What is the most common subtype of JIA?

Answer 6

Oligoarticular JIA AKA pauciarticular

Question 7

Common signs & symptoms of JIA?

Answer 7

1) Arthritis:
- joint pain & stiffness
- swollen joints, warm to touch

2) Systemic:
- fatigue
- uveitis
- salmon pink rash
- high, spiking fever
- lymphadenopathy
- weight loss

Question 8

How many joints are affected in oligoarticular JIA?

Answer 8

4 or less

Normally it only affects a single joint (monoarthritis)

Question 9

Who does oligoarticular JIA typically occur in?

Answer 9

Girls under the age of 6

Question 10

What is a classic associated feature with oligoarticular JIA?

Answer 10

Anterior uveitis - refer to ophthalmology

Question 11

Can a fever be present in JIA?

Answer 11

Yes

Question 12

What eye features may be present in JIA?

Answer 12

Anterior uveitis - pain, redness, vision loss

Question 13

Which antibody is often positive in JIA (especially oligoarticular)?

Answer 13

ANA

Question 14

Management of JIA?

Answer 14

1) NSAIDs

2) Steroids

3) Dmards etc

Question 15

What score is used to assess for hypermobility and support the diagnosis of Ehlers-Danlos?

Answer 15

The Beighton score

Question 16

What cardiac abnormalities are patients with Ehlers-Danlos prone to?

Answer 16

Mitral regurg and aortic root dilatation

Question 17

Describe GI features typically seen in HSP?

Answer 17

Colicky abdominal pain which may be severe enough to mimic an acute abdomen.

Bloody stools or melena.

N&V also occurs in about half of patients.

Question 18

What is the most common GI complication in HSP?

Answer 18

Intussusception

Question 19

How can HSP affect the kidneys?

Answer 19

Can affect the kidneys in 50% of patients, causing IgA nephritis:

• micro or macroscopic haematuria
• proteinuria
• nephrotic syndrome or renal failure

Question 20

How do you know if HSP has caused nephrotic syndrome?

Answer 20

If more than 2+ of protein on dipstick

Will also have some oedema

Question 21

Management of HSP?

Answer 21

1) Normally supportive e.g. paracetamol

2) Steroids: for nephrotic-range proteinuria

Question 22

Which medication increases the production of HbF?

Answer 22

Hydroxycarbamide (aka hydroxyurea)

Question 23

What causes a normal Hb dip in healthy term babies around 6-9 weeks of age?

Answer 23

1) High oxygen delivery to the tissues caused by the high Hb levels at birth cause negative feedback.

2) Production of EPO by the kidneys is suppressed.

3) Subsequently there is reduced production of Hb by the bone marrow.

i.e. The high oxygen results in lower Hb production.

Question 24

What is a key risk factor for anaemia in neonates?

Answer 24

Prematurity

Question 25

Worldwide, what is a common cause of blood loss causing chronic anaemia & iron deficiency?

Answer 25

Helminth infection, with roundworms, hookworms or whipworms.

This can be very common in developing countries and those living in poverty.

Question 26

Where is iron mainly absorbed?

Answer 26

Duodenum & jejunum

Question 27

What medications can interfere with iron absorption?

Answer 27

Medications that reduce the stomach acid, such as PPIs (lansoprazole and omeprazole)

Question 28

What conditions can affect iron absorption?

Answer 28

Coeliac disease or Crohn’s (inflammation of duodenum or jejunum)

Question 29

Two things can increase the values in iron studies (except TIBC, which will be low), giving the impression of iron overload.

What are these two things?

Answer 29

1) supplementation with iron

2) acute liver damage (lots of iron stored in liver)

Question 30

When is oral iron unsuitable?

Answer 30

Where malabsorption is the cause of the anaemia