Revision - JIA Flashcards
What is juvenile idiopathic arthritis (JIA)?
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.
When can JIA be diagnosed?
When there is arthritis without any other cause lasting >6 weeks in a patient <16 y/o
How long does arthritis have to be present for for diagnosis of JIA?
6 weeks
What are the 3 key features of JIA?
1) joint pain
2) swelling
3) stiffness
What are the 5 key subtypes of JIA?
1) Systemic JIA
2) Polyarticular JIA
3) Oligoarticular JIA
4) Enthesitis related arthritis
5) Juvenile psoriatic arthritis
What is the most common subtype of JIA?
Oligoarticular JIA AKA pauciarticular
Common signs & symptoms of JIA?
1) Arthritis:
- joint pain & stiffness
- swollen joints, warm to touch
2) Systemic:
- fatigue
- uveitis
- salmon pink rash
- high, spiking fever
- lymphadenopathy
- weight loss
How many joints are affected in oligoarticular JIA?
4 or less
Normally it only affects a single joint (monoarthritis)
Who does oligoarticular JIA typically occur in?
Girls under the age of 6
What is a classic associated feature with oligoarticular JIA?
Anterior uveitis - refer to ophthalmology
Can a fever be present in JIA?
Yes
What eye features may be present in JIA?
Anterior uveitis - pain, redness, vision loss
Which antibody is often positive in JIA (especially oligoarticular)?
ANA
Management of JIA?
1) NSAIDs
2) Steroids
3) Dmards etc
What score is used to assess for hypermobility and support the diagnosis of Ehlers-Danlos?
The Beighton score
What cardiac abnormalities are patients with Ehlers-Danlos prone to?
Mitral regurg and aortic root dilatation
Describe GI features typically seen in HSP?
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.
What is the most common GI complication in HSP?
Intussusception
How can HSP affect the kidneys?
Can affect the kidneys in 50% of patients, causing IgA nephritis:
- micro or macroscopic haematuria
- proteinuria
- nephrotic syndrome or renal failure
How do you know if HSP has caused nephrotic syndrome?
If more than 2+ of protein on dipstick
Will also have some oedema
Management of HSP?
1) Normally supportive e.g. paracetamol
2) Steroids: for nephrotic-range proteinuria
Which medication increases the production of HbF?
Hydroxycarbamide (aka hydroxyurea)
What causes a normal Hb dip in healthy term babies around 6-9 weeks of age?
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.
What is a key risk factor for anaemia in neonates?
Prematurity
Worldwide, what is a common cause of blood loss causing chronic anaemia & iron deficiency?
Helminth infection, with roundworms, hookworms or whipworms.
This can be very common in developing countries and those living in poverty.
Where is iron mainly absorbed?
Duodenum & jejunum
What medications can interfere with iron absorption?
Medications that reduce the stomach acid, such as PPIs (lansoprazole and omeprazole)
What conditions can affect iron absorption?
Coeliac disease or Crohn’s (inflammation of duodenum or jejunum)
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?
1) supplementation with iron
2) acute liver damage (lots of iron stored in liver)
When is oral iron unsuitable?
Where malabsorption is the cause of the anaemia
