R/O - Rheumatic & vasculitis diseases in children

Question 1

what is JIA (juvenile idiopathic arthritis)?

Answer 1

• hyperplasia of synovial lining
• edema
• hyperemia

Question 2

• morning stiffness
• rheumatoid nodules
• limp / refusal to bear weight
• deformity
• joint edema, erythema, warmth

Answer 2

JIA

Question 3

polyarthritis is arthritis in how many joints?

Answer 3

5 or more

Question 4

oligoarthritis - what should you look for?

Answer 4

uveitis

Question 5

ANA positivity occurs in what type of arthritis?

Answer 5

oligoarthritis

Question 6

what joints are affected in polyarthritis?

Answer 6

all large joints

Question 7

what joints are affected in oligoarthritis?

Answer 7

knees / ankles

Question 8

Rh factor is (sometimes, 10%) positive in what type of arthritis?

Answer 8

poly

Question 9

if patient is ANA positive, __________ is more common and therefore requires a ___________ exam

Answer 9

• uveitis

- eye exam

Question 10

ANA - worry about:

Answer 10

uveitis

Question 11

• swelling
• limited ROM
• tenderness
• increased heat

patient 16 yo or younger

Answer 11

JIA

Question 12

what is the treatment for JIA?

Answer 12

• multidisciplinary team (PCP, social services, PT, rheumo, ophtho, nurse, family)
• NSAIDs (first line)
• immunosuppressive (DMARDs) - delay progress
• steroids
• joint injections

Question 13

JIA is worse if positive for:

Answer 13

Rh factor

Question 14

psoriatic arthritis is arthiritis with 2 or more of:

Answer 14

• dactylitis
• nail piting / oncycholysis
• family history of psoriasis

Question 15

treatment for psoriatic arthritis requires:

Answer 15

DMARDs

Question 16

what is the enthesis?

Answer 16

site of attachment of muscle or ligament to bone

Question 17

• SI joint tenderness
• HLA B27 positive
• acute uveitis
• oligo or polyarthritis

boy 8 yo or older

Answer 17

enthesitis related JIA

Question 18

neonatal lupus is caused by:

Answer 18

maternal transfer of antibodies

Question 19

what are the symptoms of neonatal lupus?

Answer 19

• hematologic: thrombocytopenia
• cardiac: complete heart block
• liver: hepatomegaly, hepatitis

Question 20

children with neonatal lupus and cardiac complications require what therapy?**

Answer 20

permanent pacemaker

Question 21

henoch schonlein purpura is immune mediated via what Ig?

Answer 21

IgA

Question 22

• non thrombocytopenic purpura
• vasculitis
• 2-10 yo

Answer 22

HSP

Question 23

what is diagnostic for HSP?

Answer 23

palpable purpura

Question 24

are the palpable purpura from HSP symmetric or asymmetric?

Answer 24

symmetric

Question 25

what are a few GI complications from HSP?

Answer 25

- intussusception | - bleeding

Question 26

what are the renal complications in HSP?

Answer 26

- nephritis (asymptomatic hematuria, possible proteinuria) - HTN - progressive renal failure (rare) - acute insufficiency (rare)

Question 27

what is the treatment for HSP?

Answer 27

- supportive | - steroids (mostly for ab pain)

Question 28

what is the triad of HUS?

Answer 28

- microangiopathic hemolytic anemia - thrombocytopenia - renal insufficiency

Question 29

most cases of HUS are caused by:

Answer 29

shiga toxin producing straings E. coli 0157:H7

Question 30

- abdominal pain followed by diarrhea that becomes bloody - possible fever (low grade) - petechia - oliguria - fatigue, pallor - edema / HTN - seizure / coma

Answer 30

HUS

Question 31

what meds should be AVOIDED in HUS?

Answer 31

- antimotility - abx - platelet transfusions

Question 32

what is cure / treatment for HUS?

Answer 32

- NO cure!! - symptomatic care and watchful waiting - PREVENTION