Pediatric Rheumatology Flashcards
Dx of JIA
Need arthritis for at least 6 weeks in a child less than 16 y/o when other causes ruled out
JIA complications
Persists into adult
Uveitis is serious
Uncontrolled leads to growht probs
JIA pathogenesis
Iiodpathic
Genetic,
immunological (altered immunity or immune dysregulation)
environment (infection, trauma, stress, perinatal/prenatal exposures)
Genetic factors of JIA
PTPN22, IL2RA/CD25
HLA associations
Down syndrome (trisomey 21) Turner syndrome (45,XO) Velocardiofacial syndrome (del 22q11.2)
Uveitis and JIA
Young female (think toddler) oligo-JIA and patients who are ANA+ are most at risk
Sometimes prior to onset of arthritis
Usually asymptomatic
Can get postierior synechiae, cataracts, band keratopathy, glaucoma, loss of vision
What is uveitis?
Mostly in the anterior chamber in JIA
Get inflammation of anterior chamber…iris develops adhesions to the lens…can even have damage to ciliary body
Uveitis managment of JIA
Need regular slit lamp exams to screen…depends n subtype and other factors
How does JIA present
Joint swelling, pain (typically not in JIA), morning stiffness
Limp, revert to infantile movement patterns, refuse to perform activities, troulbe keeping up with peers
Exam on JIA
Inflammatory signs
Grwoth distrubance….increased growht of long bones and premature fusion of grwoth plates in small bones
Systemic JIA dx
DIagnosis is any number of joints with a fever more than 2 weeks that is daily for 3 days and accompanied by
Rash
LAD
Hepato/splenomegaly
Serositis
sJIA epidemiolgy
Onset before 5y/o
Males and females equal
Autoinflammatory dz
Bloodwork with inflammation but ABs negative
sJIA complications
Rash is most common…migratory and transient and exhibits koebner phenoone…might look like urticaria and if you touch or scratch, rahs will appear
No uveitis normally
Growth retardation/macrophage activation syndrome…maybe amyloidosis
Oligo-JIA dx
Arthrtis in less than 4 joints during the 6 mos of dz
Persistent - never more than 4 joints
Extended - more than 4 joints affected after the first 6 months of dz
Oligo-JIA epi and other dx
More in females
Around 1-2 y/o
Usually lower extremity involvement
Most ANA positive
Uveitis in about 20%
POly-JIA dx
Arthritis affecting more than 5 joints during first 6 mos of the disease
RF- neg
RF-pos - test is positive more than 2 times more than 3 months apart during 1st 6 months of the dz
POly-JIA epi
MOre in females…especially in RF positive
PlyJIA neg onset and epi
1-3 y/o onset, or later childhood/adolescence
Mild systemic faetures
May be asymmetric
All RF neg, ANA+ on 50%
15% uveitis
Poly-JIA RF positive onset and epi
9-11 y/o
Clinically similar to RA
Aggressive nad may be symmetric
All RF+, CCP+ in 75%, ANA+ in 75%
Uveitis uncmmon
Infection diff dx
Viruses - parvo B19, EBV, CMV…tend to be more acute
Bacteria - septic arth, neisseria gonorrhoeae, TB
Transient synovitis
Often preceded by an URI
More in boys 3-10 y/o
Pain can be sudden/gradual and lasts about 6 days
Unilateral hip pain is most common
Transient synovitis dx
ESR and WBC normal
Joint fluid normal or slight increase in WBC
X-ray/US/MRI may show joint effusion…x-ray cannot rule out
Manage with NSAIDs and rest
Systemic dz dx
Malignancy
IBD
SLE
Sarcoidosis
Vasculitis - look for HSP - palpable purpura, ab pain, renal dx, periarticular soft tissue swelling
JIA management
NSAIDs Steroids DMARDs Biologics PT/OT
Uveitis screening
JIA outcomes in general
50% into adults
50% without diability
sJIA outcomes
50% monocyclic
50% have a persistent disease course
Not much death
