Pediatric Rheum Flashcards
milestones in peds to look for
weight and height retradtation
localized growth abnormalities
delayed secondary sex characteristics
going forward than regressing
chief compplaint in kids with arthritis
not pain- usually parents notice change ina ctivity
how to evalute a patient
limited ROM–>swelling/tenderness?–>symmetry?
benign juvenile hypermotility syndrome
some have painw with this and others dont
JI-JA overview
chronic arthritis persisting in >1 joint for >6 weeks at age <16 with exlcusion of other etio
pauci-oligo articular age 2-5 years
F»M
knee, ankle, wrist
ANA +
most severe
pauci-oligo articular 6-10 years
F=M
pauci-oligo articular age 11-18 years
M»F
lower limb, enthesitis, acute uveitis
enthetitis
inflammation where ligaments or tendons join to bones
polyarticular 2-5 years old
F>M
small and large joints with tenosynovitis
polyarticular 6-10 years
F>M
polyarticular 11-18 years
F»M
symmetric
RF+
erosive, like adult RA
systemic all ages groups
M=F
fever, rash,v araible arthritis, hematologic elevated ESR/CRP, auto ab -
lab values in JIA
high platelets
Iga deficient in 7-10%
anti CCP antibody is occasionally seen in kids
pathogenesis of synovitis
villous hyperplasia of synovium w/ hypervascularity and infiltration by lymphocytes and macrophages
main presentation JIA
younger boy or girls with less than 5 joints
main symptom
synovitis
other symptoms
synovitis, joint contracture, +/- erosions, deformity, and functional limitations
systemic onset JIA shows
rash +/i enlarged lymph nodes
rash very diagnostic for JIA
rash/fever last 2-5 hours and the arthritis resolves as it does
more severe rash in JIA
koebner phenomenom- linear skn lesiosn at site of trauma
epiphyseal overgrowth
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)
cspine may be associated with
micrognathia (lower jaw smaller than normal)
highest risk of uveitis
kids <7 with + ANA, oligoarticular
uveitis is usally
asymptomatic–>if undetected, blindness- keratopathy, cataracts
tx uveritis
ocular steroids
tx of JIA
NSAIDs 1st line- covers all signs and symptoms in 30%
refractory cases- 1 injection of sterioids–>stop dz
systemic JIA
mTx, lefunomide, then biologics if they dont work
percentage that goes into remission in adulthood with JIA
65% pauciarticualr
percentage pauciarticular contineu to have active arthritis
50%
population most likely to have active arthritis in adulthood
16 year old girls with + RF
what do you need to exclude before dx pauci aritcualr 2-5 years
lyme arthritis
m&m with systemic onset by age 15
14%, but 80% will achieve adult remission
MC raynaud’s disease
ADD meds
how do you tell primary and secondary raynaud’s
+ANA and nailfold path
causes of secondary raynaud’s
CT disease
occlusive arteriole
vascular injury-frostbite, trauma
drugs and toxins
drugs and toxins
b-blockers
vinyl chloride
ampetamine
ritalin/concerta
chillblain’s is like
reverse raynauds==> venules instead of arterioles (pool blood in toes vs fingers)
*mostly benign
how does chillblain’s come about
infectious skin condition presenting secondar to exposure to cold as PRURITIC and or painful erythematous to violaceous papules/plaques/nodules
autoimmune diseases kids get
raynauds morphea neonatal lupus vasculitis HSP kawaski's
other name for morphea
linear scleroderma
tx for morphea
mild lesions do not require or respond to tx (may regress spontaneously)
–>lesions on face or joint line, treat aggressively (MTX)
how do you get neonatal lupus
mother with active SLE can pass Ro and La abs to baby via placenta
what does neonatal lupus present as
reversible skin rash, leukopenia, cytopenia
big issue with neonatal lupus
irreversible heart bock
management of neonatal lupus
cleck SLE mom for these antibodies
if yes, give dexamethasone
2 most common forms of vasculitis in children
Henoch schonlein Purpura
kawaski disease
when does HSP present
generally after age 3 (slight male predominance)
what vessel does HSP infiltrate
capilarries and pre and post cap vessels
what is HSP often preceded by
URI
pathogenesis of HSP
IgA immune complexes
skin findings of HSP
urticaria (hives) to purpura
joint clinical presentation of HSP
transient asymmetric arthritis (almost NEVER involves the hips)
clinical presentation of GI in HSP
colicky abdominal pain, bloody diarrhea, melena, intususception
renal clinical presentation in HSP
micro/gross hematuria, proteinura, htn, nephrotic syndrome–>renal failure
tx HSP
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)
kawaski’s dz aka
mucocutaneous LN syndrome
kawaski’s disease presentation
generalized systemic vasculitis, but has predilection for coronary arteries–>aneurysms in 20-25% of untreated patients with mortality of 2%
tx kawaskis
Aspirin + iViG –>reduces aneurysms to 5%
2 proposed mechanisms of kawaski’s
polyclonal superantigen response oligoclonal response (IgA)
dx criteria of kawaski’s
fever + 4/5
bilateral conjunctival injection polymorphous erythema rash oropharyngeal involvement- STRAWBERRY TONGUE cervical lymphadenopathy extremity involvmeny
extremity involvment of kawaski’s includes
edema of hands and feet
erythema of palms or soles
cutaneous desquamation
transverse nailg rooves
incmplete/atypical KD
fever >5 days and <4 cardinal signs
to confirm dx of KD or atypical KD
echo
