Juvenile Idiopathic Arthritis Flashcards

1
Q

JIA formerly known as

A

juvenile rheumatiod arthritis

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2
Q

definition of JIA

A

arthritis of unknown etiology

lasting 6 weeks or more

occuring in kids before their 16th birthday

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3
Q

How many categories are there?

A

6 - depending on location and number of joints and presence or lack of inflamatory markers

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4
Q

1 Oligoarthritis

A

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

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5
Q

2 Polyarticular

A

affects 5 or more joints during the first 6 months

broken down in to RF negative or positive

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6
Q

RF negative Polyarticular

A

No rheumatoid factor present when tested

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7
Q

RF positive Polyarticular - when to repeat testing?

Which gender is more affected?

Age of onset?

A

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

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8
Q

3 Enthesitis related arthritis

A

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

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9
Q

4 systemic

A

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

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10
Q

5 psoriatic arthritis

A

psoriasis and arthritis present

may have dactylsis (swelling of fingers)

onycholysis (nail pitting)

+ fam h/o psoriasis in first degree relative

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11
Q

6 undifferentiated arthritis

A

kids that meet criteria for 2+ types

OR

dont meet criteria for any type

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12
Q

JIA genetic causes

A

heterogenous disorder, enviornmentally induced in genetically predispositioned person

25-40% in monozygotic twins

siblings with 15-30 fold higher prevalence of dev JIA

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13
Q

Autoimmune & genetic cause of JIA

humoral and cell medicated immunity concerns

A

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

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14
Q

enviornmental RF

A

maternal smoking

infection/trauma may initiate autoimmune reaction - T cells and pro-inflammatory cytokines

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15
Q

Bone health recomendations for prevention

A

Vit D, sun exposure, breastfeeding

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16
Q

Which gender is affected more?

A

females > males but depends on type

onset equal in both sets

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17
Q

Type more common in african americans?

A

polyarticular and RF+

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18
Q

Type more common in white children/european discent

A

Oligoarthritis

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19
Q

early referral

A

important for early aggressive treatment

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20
Q

onset in late teen years

A

related to progression to adult rheumatoid diesase

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21
Q

Disease progression

A

many never have another episode or may be in remission for months then reoccur

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22
Q

Indications for poor prognosis

A

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 +

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23
Q

Joint problems

A

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

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24
Q

What is enthesitis ?

A

infl of sites where ligaments or tendons insert into bone

knee, achilles tendon, greater trochanter, planter fascia insertion on feet or metatarsal heads

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25
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
26
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
27
eye exam frequency for ANA positive OJIA and PJIA 7 + years old
Q 6 months
28
eye exam frequency for ANA negative OJIA and PJIA all ages
Q 6 months
29
antinuclear antibodies
related to autoimmune pathophysiology and humoral immune response
30
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
31
Gelling and morning stiffness
gelling - joint stiffness after prolonged inactivity stiffness - improves as the day goes on
32
Presentation in toddlers
may present with a lip, grumpy upon wakening, \* pain is not a primary symptom arthritis and synovitis can be painless
33
JIA history clues
growth gelling morning stiffness/achiness limping swelling/warmth
34
JIA presenting s/s
Decreased ROM - observe activity, positions, gait, walking and running Joint effustion/tenderness leg legnth discrepancy - abnormal gait malnutrition jaw pain
35
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
36
diagnosis
usually made clinically as all labs may be normal there is no definitive lab test
37
Olgoarthritis and labs
usually have negative lab markers
38
polyarthritis and labs
usually have elevated acute phase reactants and anemia of chronic disease
39
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
40
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
41
anti-ccp antibodies
check for, seen with more aggressive types of disease and may show before onset of s/s
42
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
43
4 main goals of treatment
1. suppress inflammation 2. preserve/maximize joint function 3. prevent joint deformities 4. prevent blindness
44
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
45
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
46
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
47
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
48
Non-biological DMARD disease modifying antirheumatic drug = Methotrexate
Prescribed by rheumatologist s/e: GI- abd pain, nausea, hematologic, lover toxicity, oral ulcers
49
Systemic glucocorticoids
no longer recomended d/t adverse se and availability newer theraputic regimens
50
growth
may have slow growth esp from meds like steriods and inflammation
51
Diet
foods to lower inflammation
52
fiber rich foods
quinoa, sweet potatoes, beans
53
clean protein
salmon, grass fed beef
54
fruits/veg
folate rich, dark leafy greens, beets, berries, tomatoes, cherries, broc,
55
herbs/spices
ginger, rosemary, cinnamon
56
omega 3 rich
salmon, tuna, sardines
57
Calcium/Vit D
immune boosting and strong bone development
58
Diet foods to avoid
sugar, sat/trans fat, artificial ingredients, charred foods, all increase inflammation
59
safety risk
joint damage/risk of injury from sports driving with uveitis
60
avoid live vaccines if
taking immunosuppressive drugs should get PCV 13, prevnar and Flu
61
screening
TMJ, pericarditis, LFT, serum creat, NSAID monitoring, routine hearing
62
eye exam frequency
Q 3-4 months or Q6 months dep on ANA status or type
63
puberty
may be late onset Methotrexate - tetrogenic NSAIDS avoid if pregnant
64
resources
Kids health american arthritis foundation
65
prognosis
onset in teen years may progress into adult life wanes and completely subsides in 85% of patients
66
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