summary Juvenile Idiopathic Arthritis is a chronic autoimmune inflammatory arthritis lasting greater than 6 weeks in a patient younger than 16 years of age that typically presents with morning joint stiffness and ocular infections. Diagnosis is one of exclusion and presence of infection must be ruled out. One of the following must be present to confirm the diagnosis: rash, iridocyclitis, pericarditis, tenosynovitis, intermittent fever, presence of RF, C-spine involvement, morning stiffness. Treatment is usually immunomodulating drugs and frequent ophthalmologic exams. Epidemiology Demograpics female > male Anatomic location joint involvement ( knee > hand/wrist > ankle > hip > C-spine) Etiology Genetics HLA markers DR4 associated with polyarticular DR8, DR5, DR2.1 associated with pauciarticular RF- seropositive in <15% Diagnostic criteria a diagnosis of exclusion must rule out infection one of the following must be present to make diagnosis rash presence of RF iridocyclitis C-spine involvement pericarditis tenosynovitis intermittent fever morning stiffness Associated conditions C-spine involvement may lead to kyphosis, facet ankylosis, and atlantoaxial subluxation Ocular involvement typically consists of iridocyclitis, a type of anterior uveitis frequently indolent and requires immediate ophthalmologic evaluation for slit lamp examination can lead to rapid loss of vision if untreated increase risk with positive ANA titer Stills disease acute-onset JRA with multiple joint involvement, fever, rash, and splenomegaly infection must be ruled out male = female usually presents at age 5-10 years Classification Onset early onset denotes onset before teens late onset denotes onset during teens or later Classification of Juvenile Rheumatoid Arthritis Polyarticular (30%) Hand/wrist involvement most common Deformity is wrist ulnar deviation and flexion with MCP stiffness in extended, swollen & radially deviated digits position 60% remission rate > 5 joints involved Small joint involvement (hand, wrist) Symmetric findings F>M Pauciarticular (50%)(oligoarticular) Most common type Early-onset associated with iridiocyclitis in 50% and chronic uveitis Obtain ophthalmology consult; requires frequent ophthalmologic exams (q4 mth if ANA+; q6 mth if ANA-) Girls are affected four times more often than boys in early-onset Peak age 2-3 yrs Late-onset seen more frequent in boys Typical finding is a limp that improves during the day Best prognosis for long term remission (70%) < 5 joints involved Large joint involvement (knees, ankles) Asymmetric findings (leg length discrepancy, involved limb ends up LONGER) M:F =1:4 Age 2-3y Systemic (20%) Includes Stills disease Poorest prognosis systemic symptoms (rash, fever, multiple joint involvement) anemia, high WBC, elevated ESR, CRP, platelets Hepatosplenomegaly, lymphadenopathy, pericarditis M=F, age 5-10y Presentation Symptoms morning stiffness and joint pain visual changes fever Physical exam rash iridocyclitis (can lead to rapid loss of vision if untreated) Imaging Radiographs often negative at presentation juxta-articular, late osteopenia and joint destruction can be seen if disease progressive obtain flexion-extension neck radiographs to rule out atlantoaxial instability Studies Laboratory rheumatoid factor RF seropositive in <15% associated with higher incidence of chronic, active, and progressive disease often results in more destructive DJD more likely to progress into adult RA RF seronegative is more common ANA ANA positive is diagnostic basic serology values often normal and are not diagnostic Treatment Nonoperative immunomodulating drugs (DMARDs) and frequent ophthalmologic exams indications first line of treatment medications DMARDs (disease modifying antirheumatic drugs) new class of medications that have had significant impact on outcomes includes but not limited to etanercept TNF inhibitor rituximab chimeric monoclonal antibody against CD20 on B cell surface azathioprine purine synthesis inhibitor high dose aspirin/NSAIDs salicylates are now used less frequently secondary to DMARD success occasionally, gold may be used intra-articular steroid injections frequent ophthalmologic exams slit-lamp examination twice yearly if ANA(-), every 4 months if ANA(+) progressive iridocyclitis can lead to rapid loss of vision if untreated Operative synovectomy epiphysiodesis indications LLD (affected leg typically longer in oligoarticular disease) corrective osteotomies indications extremity deformity deferred until skeletal maturitity arthrodesis and arthroplasty indications for severe disease Prognosis 50% patients symptoms resolve without sequelae 25% are slightly disabled 25% have crippling arthritis or blindness best prognosis pauciarticular > polyarticular > systemic
QUESTIONS 1 of 8 1 2 3 4 5 6 7 8 Previous Next (OBQ18.52) A 5-year-old girl presents with an 8-week history of pain and swelling in the right knee, right shoulder, and left elbow after returning from summer camp. She denies any antecedent trauma, fevers, or rashes. Antibiotics prescribed by her primary care doctor have provided no significant relief, but she reports feeling better at the end of the day. Labs reveal a negative rheumatoid factor. Which of the following is most commonly associated with her diagnosis? QID: 212948 Type & Select Correct Answer 1 Cardiomegaly 7% (106/1612) 2 Facial nerve palsy 31% (503/1612) 3 Uveitis 58% (928/1612) 4 Lower limb hemihypertrophy 1% (15/1612) 5 Meningitis 3% (46/1612) L 2 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic (OBQ16.189) In which of the following clinical scenarios would an urgent ophthalmology consultation be warranted to mitigate potential irreversible complications of the primary pathology? QID: 8951 Type & Select Correct Answer 1 A 4-year old male with proportionate dwarfism secondary to an autosomal recessive mutation resulting in L-alpha iduronidase deficiency. 9% (147/1659) 2 A 5-year old male with proportionate dwarfism secondary to an X-linked recessive mutation resulting in sulpho-iduronate-sulphatase deficiency 11% (178/1659) 3 A 6-year old female with 2 months of persistent left knee swelling and associated stiffness, intermittent fever, and elevated ESR. 39% (640/1659) 4 A 7-year old male with developmental delay, dolichostenomelia, and positive urine nitroprusside test secondary to a cystathionine b-synthase deficiency. 17% (287/1659) 5 A 10-year old developmentally normal male with dolichostenomelia, generalized ligamentous laxity, and pecrus carinatum. 23% (388/1659) L 5 Question Complexity A Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ10.269) Which of the following statements is FALSE regarding juvenile idiopathic arthritis(JIA)? QID: 3252 Type & Select Correct Answer 1 To meet diagnostic criteria, persistent arthritis must occur in any joint for greater than 6 weeks before the age of 16 years 10% (300/3066) 2 Radiographic evaluation may be unremarkable 2% (74/3066) 3 Cervical involvement may lead to atlantoaxial instability 8% (233/3066) 4 A patient with suspected JIA should undergo slit lamp examination by an ophthalmologist 5% (154/3066) 5 Definitive diagnosis of JIA is confirmed by serologic evaluation 75% (2300/3066) L 2 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review Tested Concept Review Full Topic This is an AAOS Self Assessment Exam (SAE) question. Orthobullets was not involved in the editorial process and does not have the ability to alter the question. If you prefer to hide SAE questions, simply turn them off in your Learning Goals. (SAE07PE.33) What is the peak period of onset in children with pauciarticular juvenile rheumatoid arthritis? QID: 6093 Type & Select Correct Answer 1 Before age 2 years 0% (0/261) 2 Between the ages of 2 and 4 years 22% (57/261) 3 Between the ages of 4 and 8 years 38% (98/261) 4 Between the ages of 8 and 12 years 33% (86/261) 5 During adolescence 7% (18/261) N/A Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ04.226) A 4-year-old female is brought by her parents in regard to a right sided limp that improves during the day and has been present for two months. She is found to have a right knee effusion and associated soft-tissue swelling with no redness or warmth. Lab work reveals negative Rheumatoid factor, a positive low titer ANA and a normal WBC. Radiographs are normal for her age. What additional work up does she need? QID: 1331 Type & Select Correct Answer 1 Skeletal survey 5% (104/1892) 2 MRI of the pelvis 4% (75/1892) 3 Clotting factor levels 16% (294/1892) 4 Ophthalmology evaluation 68% (1278/1892) 5 Bone scan 7% (137/1892) L 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review Tested Concept Review Full Topic
All Videos (0) Podcasts (1) Basic Science⎪Juvenile Idiopathic Arthritis Basic Science - Juvenile Idiopathic Arthritis Listen Now 21:42 min 4/28/2020 253 plays 0.0 (0)