juvenile idiopathic arthritis

Question 1

what is JIA

Answer 1

a group of systemic inflammatory disorders that occur in those <16 years old

Question 2

what type of disorder is JIA

Answer 2

an autoimmune disorder (genetics and environmental factors also play a part)

Question 3

what is the criteria for diagnosis of JIA

Answer 3

1) be <16 years old
2) duration of > 6 weeks
3) have joint swelling and 2 of: warmth, tenderness, pain/limited movement of a joint

Question 4

when can you identify exactly what type of JIA someone has

Answer 4

after 6 months of them having the disease

Question 5

what are the 3 main types of JIA

Answer 5

1) pauciarticular
2) polyarticular
3) systemic onset

Question 6

what is the most common type of JIA

Answer 6

pauciarticular (type 1)

Question 7

what is the most severe type of JIA

Answer 7

systemic onset

Question 8

what are the 3 different types of pauciarticular

Answer 8

type 1, 2 and 3

Question 9

what is the most common type of pauciarticular JIA

Answer 9

type 1

Question 10

discribe type 1 pauciarticular JIA

Answer 10

• onset before the child is 5 years old
• more common in girls
• LL joints affected - present with limp
• knee is the most common joint
• +ANA result in most
• chronic uveitis and irregular iris complications

Question 11

describe type 2 pauciarticular JIA

Answer 11

• onset between 8-9 years
• more common in boys
• LL joint mainly affected - present with limp
• hips may become damaged requiring children to have a total hip replacement
• acute iridocyclitis can occur (inflammation of the iris and the ciliary body)

Question 12

describe type 3 pauciarticuar JIA

Answer 12

• can occur at any age
• most commonly girls affected
• asymmetrical upper and lower limb arthritis
• may present with dactylics (inflammation of the finger/toe due to bone infection)
• usually a family history of psoriasis and may go on to develop psoriasis
• may experience chronic iriocyclitis

Question 13

what are the 2 main types of polyarticualr arthritis

Answer 13

RF+ or RF-

Question 14

describe RF- polyarticular JIA

Answer 14

• usually girls (of any age)
• present with low grade fevers, hepatosplenomegaly, growth abnomalities
• all joints can be affected and it is usually symmetrical

Question 15

describe RF+ polyarticular JIA

Answer 15

• presents in late childhood (usually boys)
• present with low grade fever, weight loss, anaemia, nodules
• very similar to adult RA but in children
• erosions can be seen on x-ray

Question 16

describe systemic onset JIA

Answer 16

• this is the least common but most severe from of JIA
• also known as STILLs disease
• boys and girls are affected equally
• usually experience evening fevers that go away in the morning
• usually have a rash on the trunk or thighs - may be brought on by scratching (Koebner’s phenomenon)
• may have lymphadenopathy, hepatosplenomegaly, abdominal pain, pericarditis, pleural effusion or pulmonary fibrosis

Question 17

what serum marker is usually elevated in JIA

Answer 17

ESR - but not an indicator of the severity of disease

Question 18

what is the 1st line management of JIA

Answer 18

1) simple pain relief, NSAIDs and steroid injections

Question 19

what is the 2nd line management of JIA

Answer 19

1) methotrexate (suppresses the immune system)
2) anti-TNF therapy
3) IL-1 R-antagonists (refractory systemic arthritis)
4) IL-6 antagonists (refractory systemic disease)

Question 20

why is there limited use of systemic steroids

Answer 20

due to the serious side effects - osteoporosis, infections and growth abnormalities

Question 21

when are steroids used in JIA

Answer 21

when children are undergoing surgery, used in systemic JIA and to manage serious disease complications of all types

Question 22

what surgical treatments can be helpful in JIA

Answer 22

• joint replacement surgery

- synovectomy