Juvenile Idiopathic Arthritis Flashcards
JIA formerly known as
juvenile rheumatiod arthritis
definition of JIA
arthritis of unknown etiology
lasting 6 weeks or more
occuring in kids before their 16th birthday
How many categories are there?
6 - depending on location and number of joints and presence or lack of inflamatory markers
1 Oligoarthritis
affects 1-4 joints during the first 6 months
can be persistant (1-4) or
extended (4+) after the first 6 months
earlier onset - usually around 4 years old
common in females
2 Polyarticular
affects 5 or more joints during the first 6 months
broken down in to RF negative or positive
RF negative Polyarticular
No rheumatoid factor present when tested
RF positive Polyarticular - when to repeat testing?
Which gender is more affected?
Age of onset?
Two positive tests- three months apart in first 6 months
more aggresive disease course
higher risk of joint deformities d/t errosive joints/RA
Common in females
onset 1-3 years and
around 12 years old
3 Enthesitis related arthritis
ERA presents with arthritis and enthesitis (infl of ligaments/tendons where they connect to the bone)
common in BOYS
usually has spine and sacroiliac joint involvement and back pain
can present with an acute painful red eye
4 systemic
intermittant fever, rash, arthritis, systemially ill with myalgia/arthralgia
This triad of fever/rash/arthritis doesnt have to appear at the same time
fever/rash may occur before arthritis pain
5 psoriatic arthritis
psoriasis and arthritis present
may have dactylsis (swelling of fingers)
onycholysis (nail pitting)
+ fam h/o psoriasis in first degree relative
6 undifferentiated arthritis
kids that meet criteria for 2+ types
OR
dont meet criteria for any type
JIA genetic causes
heterogenous disorder, enviornmentally induced in genetically predispositioned person
25-40% in monozygotic twins
siblings with 15-30 fold higher prevalence of dev JIA
Autoimmune & genetic cause of JIA
humoral and cell medicated immunity concerns
ILRA/CD25 and VTCN1 genes known for succeptability in kids
HLA-I and II leads to the dev of T cells and antigen presentation
activation of the humoral immune resp is seen in the prod of antinuclear antibodies (ANA) and serum IGGs such as IgM rheumatiod factor
enviornmental RF
maternal smoking
infection/trauma may initiate autoimmune reaction - T cells and pro-inflammatory cytokines
Bone health recomendations for prevention
Vit D, sun exposure, breastfeeding
Which gender is affected more?
females > males but depends on type
onset equal in both sets
Type more common in african americans?
polyarticular and RF+
Type more common in white children/european discent
Oligoarthritis
early referral
important for early aggressive treatment
onset in late teen years
related to progression to adult rheumatoid diesase
Disease progression
many never have another episode or may be in remission for months then reoccur
Indications for poor prognosis
systemic onset, positive RF factor, poor resp to therapy, bone erosion
< 4 joints - arthritis of hip or cervical spine
> 5 joints - arthritis of hip or cervical spine and RF +
Joint problems
osteopenia/osteoporosis, bony erosions, premature fusion of growth plates, sublaxated/unstable joints, epiphyseal overgrowth, eventual fusion/ankylosis
TMJ - bilateral can disrupt growth of mandible results in micrognathia and retrognathia
arthritis- limited mobility, decreased QOL, joint damage, psyc issues
What is enthesitis ?
infl of sites where ligaments or tendons insert into bone
knee, achilles tendon, greater trochanter, planter fascia insertion on feet or metatarsal heads
Uveitis
infl of the eyes - should be checked by optho dependig on type of JIA (ANA positive OJIA)
can result in cataracts, glaucoma, blindness if untreated
eye exam frequency for ANA positive OJIA and PJIA
onset < 7 years old
Q 3-4 months
ANA - antinuclear antibodies r/t autoimmune pathophys and humoral immune resp
eye exam frequency for ANA positive OJIA and PJIA
7 + years old
Q 6 months
eye exam frequency for ANA negative OJIA and PJIA
all ages
Q 6 months
antinuclear antibodies
related to autoimmune pathophysiology and humoral immune response
Macrophage Activation Syndrome and JIA (MAS)
Life threatning complication
persistant fever, cytopenias, fixed rash, elevated D-dimers and liver enzymes, low fibrinogen, decreased ESR rates, and elevated coagulopathy and triglycerides
Gelling and morning stiffness
gelling - joint stiffness after prolonged inactivity
stiffness - improves as the day goes on
Presentation in toddlers
may present with a lip, grumpy upon wakening,
* pain is not a primary symptom
arthritis and synovitis can be painless
JIA history clues
growth
gelling
morning stiffness/achiness
limping
swelling/warmth
JIA presenting s/s
Decreased ROM - observe activity, positions, gait, walking and running
Joint effustion/tenderness
leg legnth discrepancy - abnormal gait
malnutrition
jaw pain
Long term presentation
bony overgrowth - usually over knees
muscle weakness/atrophy
leg legnth discrepancy - affected leg will be longer due to overgrowth
micro or retrognathia (TMJ) - difficulty chewing or opening mouth
diagnosis
usually made clinically as all labs may be normal there is no definitive lab test
Olgoarthritis and labs
usually have negative lab markers
polyarthritis and labs
usually have elevated acute phase reactants and
anemia of chronic disease
Labs
CBC- exclude leukemia dx, check for anemia, bone pain closely resimbles malignancy
ESR/CRP- deteft infl and infection
Lyme titers- to r/o lyme
LFT- r/o hepatitis and check liver function
rheumatoid factor- identifies subtype
immunoflurescent test
gold standard for ANA test
positive ANA (anti-nuclear antibody) helps determine if the child is at risk for uveitis and determine if there is an auto-antibody problem
anti-ccp antibodies
check for, seen with more aggressive types of disease and may show before onset of s/s
Imaging
Xray- used initially to r/o traumatic/ortho issues
MRI- can be used to determine extent of joint involvement or dificult areas to see on x-ray
US- helpful to use in younger kids who wont sit still for imaging, less invasive
Immaging is unable to differentiate between underlying causes of arthritis, like malignancy, infl, infection
4 main goals of treatment
- suppress inflammation
- preserve/maximize joint function
- prevent joint deformities
- prevent blindness
NSAID use in JIA
use in those with low-disease activity with less than 4 joints, without poor prognosis or joint contracture
2 week trial warranted
lack of response to one NSAID does not confer response to all agents in the class- trial alternatve NSAID should be considered
Ibuprofen use, dosing and s/e
30-40 mg/kg/day in 3-4 divided doses
Max dose 2400mg/day
S/E abd pain, peptic ulcer disease, gastritis, thrombocytopenia, renal toxicity w long-term use
Naproxen use, dosing and s/e
10mg/kg/day in 2 divided doses
max dose 1000mg/day
For more moderate pain NSAIDs should be used in adjunct.
Labs before starting and twice yearly for daily use lasting > 3-4 weeks
CBC, LFT, serum creatine
Celexocib (Celebrex)
can be used if the patient has severe GI s/e from NSAIDS
NO ASPRIN- RYES
Topical OTC pain patches- OTC salonpas has 4% lidocaine
Non-biological DMARD
disease modifying antirheumatic drug =
Methotrexate
Prescribed by rheumatologist
s/e: GI- abd pain, nausea, hematologic, lover toxicity, oral ulcers
Systemic glucocorticoids
no longer recomended d/t adverse se and availability newer theraputic regimens
growth
may have slow growth esp from meds like steriods and inflammation
Diet
foods to lower inflammation
fiber rich foods
quinoa, sweet potatoes, beans
clean protein
salmon, grass fed beef
fruits/veg
folate rich, dark leafy greens, beets, berries, tomatoes, cherries, broc,
herbs/spices
ginger, rosemary, cinnamon
omega 3 rich
salmon, tuna, sardines
Calcium/Vit D
immune boosting and strong bone development
Diet foods to avoid
sugar, sat/trans fat, artificial ingredients, charred foods, all increase inflammation
safety risk
joint damage/risk of injury from sports
driving with uveitis
avoid live vaccines if
taking immunosuppressive drugs
should get PCV 13, prevnar and Flu
screening
TMJ, pericarditis, LFT, serum creat, NSAID monitoring, routine hearing
eye exam frequency
Q 3-4 months or Q6 months
dep on ANA status or type
puberty
may be late onset
Methotrexate - tetrogenic NSAIDS avoid if pregnant
resources
Kids health
american arthritis foundation
prognosis
onset in teen years may progress into adult life
wanes and completely subsides in 85% of patients
low impact sports to recommend
swimming (not for those with anemia or cardiac hx)- easy on joints,
Dance- low impact
tricycle or bike riding
yoga
