Juvenile Idiopathic Arthritis (Juvenile RA)

Question 1

What is Juvenile RA?

Answer 1

An inflammatory arthritis in children

Question 2

How many different forms can it present in?

Answer 2

3

Question 3

What are the three different presentations in JRA?

Answer 3

pauciarticular, polycarticular, systemic onset

Question 4

What is its speculated pathogenesis?

Answer 4

dysregulation of immune response

Question 5

Early nonspecific clinical features?

Answer 5

morning stiffness, irritability, assumption of posture, refusal to walk, fatigue, low grade fever, anorexia

Question 6

What Polyarticular JRA resembles?

Answer 6

Adult RA

Question 7

How many joints are involved in Polyarticular JRA?

Answer 7

5 or more joints

Question 8

What is the pattern of involvement in Poly. JRA?

Answer 8

symmetric or asymmetric, hand involvement like adult RA

Question 9

What joints are commonly involved in JRA?

Answer 9

arthritis of apophyseal joints of spine with stiff neck (check range of motion)

Question 10

Another joint can be involved?

Answer 10

Temporal mandibular joint -> micrognathia

Question 11

How many joints are involved in Oligoarticular JRA?

Answer 11

less than 5 joints

Question 12

What are the joints primarily involved?

Answer 12

knees, ankles, wrists

Question 13

If one joint is involved? It usually is:

Answer 13

Knee

Question 14

How many types in Oligoarticular JRA?

Answer 14

2 types

Question 15

Type I typically present in:

Answer 15

Female, less than 5 years old, >50% ANA +, Uveitis 15-20%

Question 16

Type II typically present in:

Answer 16

Male, late childhood onset, 90% HLA 27+, - RF and - ANA

Question 17

What is the hallmark for Systemic onset JRA?

Answer 17

Quotidian (daily) fever, chills no rigor

Question 18

What makes diagnostic in JRA?

Answer 18

Rash (migratory erthematous macuoles) + fever

Question 19

The rash can be induced by:

Answer 19

rubbing or scratching the skin -> Koebner phenomena

Question 20

What are the extra-articular manifestations of JRA?

Answer 20

pericarditis, myocarditis, chronic uveitis, iridocyclitis, blindness, brachydactly (short fingers), TMJ->microganthia

Question 21

Diganostic criteria includes?

Question 22

Special lab studies and results?

Answer 22

RF (only 20%+), ANA (40%+), CBC (normaocytic/hypchromic anemia and leukocytosis)

Question 23

X-rays result show what following features?

Answer 23

soft tissue swelling, premature closing of epiphysis, marginal erosion, narrowing of cartilage, antlantoaxial subluxation

Question 24

Treatment of JRA?

Answer 24

pain and inflammation relief: NSAIDs, corticosteroids, DMARD (methotrexate, TNF inhibitors)
physical therapy

Question 25

50% of JRA is

Answer 25

Pauciarthritis

Question 26

75% of ANA is

Answer 26

Pauciarthritis

Question 27

Prominent extra-articular involvement

Answer 27

Systemic onset JRA