JIA-LM Flashcards
what is JIA also know as/previously
Synonyms:
- Juvenile Rheumatoid Arthritis (JRA) = USA
- Juvenile Chronic Arthritis (JCA)= Europe
- Juvenile Idiopathic Arthritis (JIA)= International League of Rheumatology
tHERE IS NO single best what for JIA
a) Characteristic Appearance
b) Diagnostic Criterion
c) Treatment
d) Uniform prognosis
SO pauci has less than 5 hot joints but how many are in the subdivisions of mono and oligo?
mono=1
oligo=2-4
extended pauci has how many hot joints
5-6
what about poly arthritis?
6+
what is a hot joint?
•Any swelling or effusion, increased warmth, and/or painful limited movement with or without tenderness in one or more joints lasting more than 6 weeks.
What is the age demographic of JIA
<9 y/o
What is the ACR Classification Criteria for the Diagnosis of JIA.
Lets break it down
AGE
How many joints involved
Duration
TYpe of onset of disease during the first 6 months
Exclusion of what
- Age at onset < 17 years
- Arthritis in 1 or more joints
- Duration of disease > 6 weeks
- Type of onset of disease during the first 6 mos
- Polyarthritis
- Pauciarthritis
- Systemic Disease
5.Exclusion of other forms of juvenile arthritis
What are the 3 main types of JIA
What are the % occurances of each?
Systemic 10-15
Pauciarticuar (oligoarticular) 50%+
Polyarticular 30-40%
What are some characteristics of growing pains
Yeah I know, so let me give you categories to guess from, ya dummies.
Age
Distribution of pain
Time of Day for pain
Exercise induced?
DO they grow ok?
How do you treat?
- Age: 6-13 yrs of age
- Distribution: Lower extremities
–Pain localized to thighs, calves & shins (not joints)
- Pain most frequent late in day or night (not morning)
- Correlation with strenuous exertion is variable
- Normal growth & development
- Treatment: heat, massage & analgesics
So lets talk about systemic JIA also known as what……………………. Still’s disease
What are some signs and symptoms that are systemic?
–Malaise, fever, rash, adenopathy, hepatosplenomegaly, serositis, hepatitis, DIC, anemia
MSK sym of Still’s
–Arthritis, myalgia, arthralgias
AOO for still’s?
thats age of onset for all you slow people
there is a thing about sex and age so try to get that too.
–Usually < 5 yoa
- If < 5 yoa F=M
- If > 5yoa F>M
Describe the fever in Systemic JIA.
Spiking, crazy all over the place up and down what the heck.
What is this?
fusiform or spindle form fingers from systemic JIA
Describe the rash in systemic JIA? Note that pruritis is unsual
color
shape
size
distribution
- Evanescent (transient) and intermittent
- Salmon pink
- Circumscribed macular
- Size: 2-6 mm or greater, Often confluent
- Chest
- Axilla
- thighs & upper arms
What will the ESR be in Systemic JIA?
CBC would show what?
Dr Told said all your inflammatory markers should be up.
- ESR - high
- CBC
–Anemia
–Leukocytosis (inc. polys
–Thrombocytosis
What is the IgM RF and ANA in systemic JIA?
What confers a worse prognosis on Systemic JIA?
If the disease persist what could happen
youger the age of onset.
Amyloidosis in some children
So you have this kid with Still’s how do you manage the disease?
Splinting
PT/OT
NSAIDS
Steroids in what cases? Severe, right on Steve
DMARDS with questional benefit.
What age and sex are common in pauci articular JIA
<6
F>M
What are some clinical features of pauci?
How many and which joints
how do they grow?
etc
- Joints:
- Early growth abnormalities
- Chronic uveitis w/in 5 yrs (asymptomatic)
–Occurs in 1/3 of cases
What leads to the early growth abnormalities in pauci JIA?
Generalized inflammation
What are the lab findings in Pauci articular JIA
Pay special attention to RF and ANA
ESR
CBC
RF
ANA
- ESR - increased or wnl
- CBC:
–Hb/Hct - wnl
–WBC - wnl
–Platelets - wnl
- RF - neg
- ANA - freq + (40-75%)
Long term prognosis of Pauci JIA is good and early detection and treatment is important but what are some complications
–Alteration in growth of affected limb
–2/3 develop iridocyclitis in both eyes
Dr Told said blindness is common
How do you manage Classic Pauci?
WHich one is very important and you must know.
- Splinting
- PT & OT
- NSAIDs
- Local corticosteroid injections
–controversial
•Frequent ophthalmologic assessment & tx- this is the important one
SO we talked about classic pauci immune JIA
WHat is type II?
Juvenile Spondyloarthropathy
So Type 1 was 6 y/o or less, what is the characteristics of type II pauci JIA?
•Age: > 9 years, M > F
WHat are some clinical feature of type II pauci
- Peripheral arthritis primarily in lower extremities
- Enthesopathies
- Acute iritis
- Sacroiliac pain in some
- Axial disease in some
What is this
Enthesopathy
What is the notable lab finding in pauci type II, try to get this one but if you want the others too
- ESR - wnl to high
- CBC - wnl
- RF - neg
- HLA-B27 + must know this one
What % of kids will develop •spondylitis, hip and cervical problems in type II pauci JIA
1/3, otherwise prognosis is good
Here is the management for type II pauci
What should you note about one of these treatments?
- PT & OT
- Posture training
- NSAIDs
- ? Anti-TNF agents
- Local corticosteroid injections
- Hip arthroplasty
- Ophthalmologic examination & follow-up
Be careful in kids with the steroid injections.
Type III pauci is psoriatic, what are some characteristics of it?
- Age: ~8 years, F>M
- Family History of psoriasis
- Rarely systemic
What are some clinical features of type III pauci
- Occasional severe destructive arthritis
- Dactylitis (swollen/inflamed digit)
- Asymmetric peripheral joints
- Psoriatic rash, nail pitting/onycholysis
- Flexor tenosynovitis
ESR, CBC, RF, ANA in type III pauci?
- ESR - varies with # of joints, may be high
- CBC: Hb/Hct - (+/-) low, WBC - (+/-) inc
- RF - neg
- ANA - ? +
What is the course and prognosis of type II pauci?
- Young onset (+/-) associated with iritis
- Remitting & relapsing, even into adulthood
- Occasionally severely destructive
- Occasionally spondylitis develops
How would you manage type III pauci
Its basically the same except for 1 thing I hope you think of.
- PT & OT
- Splinting
- NSAIDs
- May require immunosuppression- this the the main difference –Methotrexate (MTX)
- Biologic Agents
–anti-TNF, IL-1ra
Polyarticular JIA has 2 subtypes what are they and severity?
How many joints are involved?
- RF (+) à adolescent, severe, similar to adults
- RF (–) à milder disease
>5 joints
Polyarticular JIA RF neg is in •Any age, occasionally < 1 year, F > M what are the clinical features?
Joints
ROM
fever
is anything enlarged
- Can affect any joint (K, W, A, PIP & DIP)
- Reduced neck & TMJ ROM
- Flexor tenosynovitis
- +/- low grade fever
- Mild lymphadenopathy & hepatosplenomegaly
What is the ESR
CBC
RF
ANA
in polyarticular JIA RF neg
one of these you must know
- ESR - increased, must know this one
- CBC:
–Anemia
–Mild leukocytosis
–Thrombocytosis
- RF - negative
- ANA - occas. positive
Recurrant episodes of polyarticular RF neg JIA cause what
Progressive deformaties
course otherwise if variable but generally good
How do you manage RF neg?
- Splinting to prevent deformity
- PT/OT to maintain & improve joint & muscle function
- NSAIDs
- DMARDs
- Anti-TNF Agents
Characteristics of RF pos
age/sex
•> 8 years @ onset, F > M
What are some clinical features of RF pos
JOints
Skin finding
Vessel thing
- Polyarthritis of any joint (small joints of W, H, A, F; K & H early)
- Rheumatoid nodules
- Vasculitis - uncommon & late
Why should you be careful with HVLA in RF pos?
cervical sublaxation
ondontoid process erosion
What will be your lab test results for RF pos
ESR
CBC
RF
ANA
HLA-DR4
X rays
- ESR – increased
- CBC: moderate anemia
- RF – positive, high titre
- ANA – may be positive
- HLA-DR4 – frequently positive
- X-rays – early erosive changes
What is the course and prognosis of RF pos
•Persistent - serious joint destruction & poor function
What are some additional long term hazards of RF pos
- C1-C2 subluxation
- aortic insufficiency & amyloidosis
Management of RF pos
- Splinting
- PT & OT
- NSAIDs
- DMARDs (MTX)
- Biologic Agents (anti-TNF)
- Surgical intervention
What are some treatments of JIA?
Hint I am really looking for the ones that are FDA approved in children*
- NSAIDs*
- Gold*
- Antimalarials
- Sulfasalazine*
- Methotrexate
- Azathioprine
- Anti-TNF Agents (Etanercept)*
- Steroids
- Adjunctive treatments
Overall in management of JIA what should you really watch
The eye, duh WATCH that eye
