Introduction A persistent autoimmune inflammatory arthritis lasting > 6 weeks in a patient younger than 16 years of age previously known as juvenile rheumatoid arthritis Epidemiology joint involvement ( knee > hand/wrist > ankle > hip > C-spine) female > male 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 Prognosis 50% patients symptoms resolve without sequelae 25% are slightly disabled 25% have crippling arthritis or blindness best prognosis pauciarticular > polyarticular > systemic Classification Onset early onset denotes onset before teens late onset denotes onset during teens or later Classification of Juvenile Rheumatoid Arthritis Polyarticular (30%) > 5 joints involved small joint involvement (hand, wrist) symmetric findings F>M 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 Pauciarticular (50%) (oligoarticular) < 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 most common type early-onset associated with iridiocyclitis in 50% and chronic uveitis. obtain opthalmology consult; requires frequent ophthalmologic exams (q4mth if ANA+; q6mth 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 day best prognosis for long term remission (70%) Systemic (20%) systemic symptoms (rash, fever, multiple joint involvement) anemia, high WBC, elevated ESR, CRP, platelets hepatosplenomegaly, lymphadenopathy, pericarditis M=F, age 5-10y includes Stills disease poorest prognosis 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
QUESTIONS 1 of 4 1 2 3 4 Previous Next 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)? Review Topic QID: 3252 1 To meet diagnostic criteria, persistent arthritis must occur in any joint for greater than 6 weeks before the age of 16 years 10% (254/2632) 2 Radiographic evaluation may be unremarkable 2% (61/2632) 3 Cervical involvement may lead to atlantoaxial instability 8% (209/2632) 4 A patient with suspected JIA should undergo slit lamp examination by an ophthalmologist 5% (119/2632) 5 Definitive diagnosis of JIA is confirmed by serologic evaluation 75% (1985/2632) ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 5 (OBQ04.226) A 4-year-old female is brought by her parents in regards 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? Review Topic QID: 1331 1 Skeletal survey 5% (79/1485) 2 MRI of the pelvis 4% (55/1485) 3 Clotting factor levels 14% (211/1485) 4 Ophthalmology evaluation 69% (1030/1485) 5 Bone scan 7% (108/1485) ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 4