Pediatric Rheum

Question 0

milestones in peds to look for

Answer 0

weight and height retradtation
localized growth abnormalities
delayed secondary sex characteristics
going forward than regressing

Question 1

chief compplaint in kids with arthritis

Answer 1

not pain- usually parents notice change ina ctivity

Question 2

how to evalute a patient

Answer 2

limited ROM–>swelling/tenderness?–>symmetry?

Question 3

benign juvenile hypermotility syndrome

Answer 3

some have painw with this and others dont

Question 4

JI-JA overview

Answer 4

chronic arthritis persisting in >1 joint for >6 weeks at age <16 with exlcusion of other etio

Question 5

pauci-oligo articular age 2-5 years

Answer 5

F»M
knee, ankle, wrist
ANA +
most severe

Question 6

pauci-oligo articular 6-10 years

Answer 6

F=M

Question 7

pauci-oligo articular age 11-18 years

Answer 7

M»F

lower limb, enthesitis, acute uveitis

Question 8

enthetitis

Answer 8

inflammation where ligaments or tendons join to bones

Question 9

polyarticular 2-5 years old

Answer 9

F>M

small and large joints with tenosynovitis

Question 10

polyarticular 6-10 years

Answer 10

F>M

Question 11

polyarticular 11-18 years

Answer 11

F»M
symmetric
RF+
erosive, like adult RA

Question 12

systemic all ages groups

Answer 12

M=F

fever, rash,v araible arthritis, hematologic elevated ESR/CRP, auto ab -

Question 13

lab values in JIA

Answer 13

high platelets
Iga deficient in 7-10%
anti CCP antibody is occasionally seen in kids

Question 14

pathogenesis of synovitis

Answer 14

villous hyperplasia of synovium w/ hypervascularity and infiltration by lymphocytes and macrophages

Question 15

main presentation JIA

Answer 15

younger boy or girls with less than 5 joints

Question 16

main symptom

Answer 16

synovitis

Question 17

other symptoms

Answer 17

synovitis, joint contracture, +/- erosions, deformity, and functional limitations

Question 18

systemic onset JIA shows

Answer 18

rash +/i enlarged lymph nodes
rash very diagnostic for JIA
rash/fever last 2-5 hours and the arthritis resolves as it does

Question 19

more severe rash in JIA

Answer 19

koebner phenomenom- linear skn lesiosn at site of trauma

Question 20

epiphyseal overgrowth

Answer 20

inflammation at joint stimulates growth of its spiphyseal plate–>affected limb become slonger than the other side until its epiphyseal plate closes early–>ok limb over grows it
(smaller joint is the involved joint)

Question 21

cspine may be associated with

Answer 21

micrognathia (lower jaw smaller than normal)

Question 22

highest risk of uveitis

Answer 22

kids <7 with + ANA, oligoarticular

Question 23

uveitis is usally

Answer 23

asymptomatic–>if undetected, blindness- keratopathy, cataracts

Question 24

tx uveritis

Answer 24

ocular steroids

Question 25

tx of JIA

Answer 25

NSAIDs 1st line- covers all signs and symptoms in 30% | refractory cases- 1 injection of sterioids-->stop dz

Question 26

systemic JIA

Answer 26

mTx, lefunomide, then biologics if they dont work

Question 27

percentage that goes into remission in adulthood with JIA

Answer 27

65% pauciarticualr

Question 28

percentage pauciarticular contineu to have active arthritis

Answer 28

50%

Question 29

population most likely to have active arthritis in adulthood

Answer 29

16 year old girls with + RF

Question 30

what do you need to exclude before dx pauci aritcualr 2-5 years

Answer 30

lyme arthritis

Question 31

m&m with systemic onset by age 15

Answer 31

14%, but 80% will achieve adult remission

Question 32

MC raynaud's disease

Answer 32

ADD meds

Question 33

how do you tell primary and secondary raynaud's

Answer 33

+ANA and nailfold path

Question 34

causes of secondary raynaud's

Answer 34

CT disease occlusive arteriole vascular injury-frostbite, trauma drugs and toxins

Question 35

drugs and toxins

Answer 35

b-blockers vinyl chloride ampetamine ritalin/concerta

Question 36

chillblain's is like

Answer 36

reverse raynauds==> venules instead of arterioles (pool blood in toes vs fingers) *mostly benign

Question 37

how does chillblain's come about

Answer 37

infectious skin condition presenting secondar to exposure to cold as PRURITIC and or painful erythematous to violaceous papules/plaques/nodules

Question 38

autoimmune diseases kids get

Answer 38

``` raynauds morphea neonatal lupus vasculitis HSP kawaski's ```

Question 39

other name for morphea

Answer 39

linear scleroderma

Question 40

tx for morphea

Answer 40

mild lesions do not require or respond to tx (may regress spontaneously) -->lesions on face or joint line, treat aggressively (MTX)

Question 41

how do you get neonatal lupus

Answer 41

mother with active SLE can pass Ro and La abs to baby via placenta

Question 42

what does neonatal lupus present as

Answer 42

reversible skin rash, leukopenia, cytopenia

Question 43

big issue with neonatal lupus

Answer 43

irreversible heart bock

Question 44

management of neonatal lupus

Answer 44

cleck SLE mom for these antibodies | if yes, give dexamethasone

Question 45

2 most common forms of vasculitis in children

Answer 45

Henoch schonlein Purpura | kawaski disease

Question 46

when does HSP present

Answer 46

generally after age 3 (slight male predominance)

Question 47

what vessel does HSP infiltrate

Answer 47

capilarries and pre and post cap vessels

Question 48

what is HSP often preceded by

Answer 48

URI

Question 49

pathogenesis of HSP

Answer 49

IgA immune complexes

Question 50

skin findings of HSP

Answer 50

urticaria (hives) to purpura

Question 51

joint clinical presentation of HSP

Answer 51

transient asymmetric arthritis (almost NEVER involves the hips)

Question 52

clinical presentation of GI in HSP

Answer 52

colicky abdominal pain, bloody diarrhea, melena, intususception

Question 53

renal clinical presentation in HSP

Answer 53

micro/gross hematuria, proteinura, htn, nephrotic syndrome-->renal failure

Question 54

tx HSP

Answer 54

usually self-limiting and resoloves without sequelae | tx- supportive-hydration and time, meds only if severe disease or if theres suspcision of complx (renal dz)

Question 55

kawaski's dz aka

Answer 55

mucocutaneous LN syndrome

Question 56

kawaski's disease presentation

Answer 56

generalized systemic vasculitis, but has predilection for coronary arteries-->aneurysms in 20-25% of untreated patients with mortality of 2%

Question 57

tx kawaskis

Answer 57

Aspirin + iViG -->reduces aneurysms to 5%

Question 58

2 proposed mechanisms of kawaski's

Answer 58

``` polyclonal superantigen response oligoclonal response (IgA) ```

Question 59

dx criteria of kawaski's

Answer 59

fever + 4/5 ``` bilateral conjunctival injection polymorphous erythema rash oropharyngeal involvement- STRAWBERRY TONGUE cervical lymphadenopathy extremity involvmeny ```

Question 60

extremity involvment of kawaski's includes

Answer 60

edema of hands and feet erythema of palms or soles cutaneous desquamation transverse nailg rooves

Question 61

incmplete/atypical KD

Answer 61

fever >5 days and <4 cardinal signs

Question 62

to confirm dx of KD or atypical KD

Answer 62

echo