21 - Juvenile idiopathic arthritis Flashcards
define JIA?
Group of systemic inflammatory disorders affecting children below age of 16 years
what can JIA lead to?
disability and blindness
what type of disease is JIA?
auto-immune
which 3 factors affect the pathogenesis of JIA?
genetic, environmental and immunological
age of onset (diagnosis)?
<16 years
duration of disease (diagnosis)?
> 6 weeks
describe the presence of arthritis to make a diagnosis?
joint swelling or 2 of the following -
1 - painful/ limited joint motion, 2 - tenderness, 3 - warmth
M:F type 1 JIA?
F>M (8:1)
describe type 1 JIA?
peak age - 1-3 years, irregular iris due to posterior synechiae
describe type 2 JIA?
after age 8-9, M:F = 7:1
describe type 3 JIA?
any age during childhood, M:F = F>M (4:1)
what is the most common type of JIA?
pauciarticular JIA (4 joints or less)
what is the 2nd most common type of JIA?
polyarticular JIA (5+ joints)
describe RF -ve polyarticular JIA?
any age, often early, F:M = 9:1
describe RF +ve polyarticular JIA?
late childhood (teens, 12-16 years), F:M = 7:1
what is the least common type of JIA?
systemic onset JIA
how is systemic onset JIA AKA?
Still’s disease
what defines still’s disease?
extra-articular features - start early and disappear after 2-5 years
age affected by Still’s disease?
throughout childhood (4-6 years)
M:F Still’s Disease?
F>M = 1.5:1
how does fever present in Still’s disease?
rises to 39.5 degrees C daily for at least 2 weeks - late in afternoon/ evening and returns to normal in morning
how does a rash present in Still’s disease?
over 90% patients, evanescent salmon red eruption on trunk and thighs
what phenomenon can be seen with the rash in Still’s disease?
+ve Koebner’s phenomenon - rash brought on by scratching
what is 1st line therapy for JIA?
simple pain killers - NSAIDs
what should 2nd line treatment be for JIA?
joint (steroid) injections
what is the role of topical steroids in JIA?
rehabilitation - physiotherapy and occupational therapy
surgical treatment in JIA?
1 - synovectomy, 2 - reconstructive/ joint replacement surgery