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P86 Pediatrics
Rheumatology
Toronto Notes 2019
Kawasaki Diagnostic Criteria: “CREAM” Conjunctival injection
Rash
Edema of hands and feet
Adenopathy Mucosal changes
Vasculitides
HENOCH-SCHÖNLEIN PURPURA
• mostcommonvasculitisofchildhood,peakincidence4-10yr,M:F=2:1 • vasculitisofsmallvessels
• oftenhavehistoryofURTI1-3wkbeforeonsetofsymptoms
Clinical Presentation
• clinicaltriad:1)palpablepurpura,2)abdominalpain,3)arthritis
• skin:palpable,non-thrombocytopenicpurpurainlowerextremitiesandbuttocks,edema,scrotal
swelling
• joints:arthritis/arthralgiainvolvinglargejointsassociatedwithpainfuledema
• GI:abdominalpain,GIbleeding,intussusception
• renal:microscopichematuria,IgAnephropathy,proteinuria,HTN,renalfailurein<5%
Investigation
• urinalaysis(blood,proteincreatinineratio),serum(urea/electrolytes,creatinine,albumin,elevatedIgA) • skin/renalbiopsy–IgAdeposition
• ultrasound–interssusception/perforation,testicularpain/swelling
• ruleoutotherautoimmuneconditions/vasculitides
Management
• mainlysupportive(e.g.elevationforedema)
• anti-inflammatorymedicationsforjointpain,corticosteroidsforselectpatients
• monitorforproteinonurinalysisandhypertensioneverymonthfor6mo,checkingforrenaldisease,
which may develop late (immunosuppressive therapy if severe)
Prognosis
• self-limited,resolveswithin4wk
• recurrenceinaboutone-thirdofpatients
• long-termprognosisdependentonseverityofnephritis
KAWASAKI DISEASE
• acutevasculitisofunknownetiology(likelytriggeredbyinfection)
• medium-sizedvasculitiswithpredilectionforcoronaryarteries
• mostcommoncauseofacquiredheartdiseaseinchildrenindevelopedcountries • peakage:3mo-5yr;Asians>Blacks>Caucasians
Diagnostic Criteria
• feverpersisting≥5dAND≥4ofthefollowingfeatures
1. bilateral, non-exudative conjunctival injection
2. oral mucous membrane changes (fissured lips, strawberry tongue, injected pharynx) 3. changes of the peripheral extremities
◆ acute phase: extremity changes including edema of hands and feet or erythema of palms or soles
◆ subacute phase: periungual desquamation
4. polymorphous rash
5. cervical lymphadenopathy >1.5 cm in diameter (usually unilateral)
• exclusionofotherdiseases(e.g.scarletfever,measles)
• atypicalKawasakidisease:feverpersisting≥5dand2-3oftheabovecriteria
■ further evaluation dictated by CRP, ESR, and supplemental laboratory criteria
Management
• initialtherapy:IVIG(2g/kg)andhigh(anti-inflammatory)doseofASA
• onceafebrile>48h:low(anti-platelet)doseofASAuntilplateletsnormalize,orlongerifcoronary
artery involvement
• IVIgwithin10dofonsetreducesriskofcoronaryaneurysmformation
• baseline2D-Echoandfollow-upperiodic2D-Echo(usuallyat2,6wk)
Complications
• coronaryarteryvasculitiswithaneurysmformationoccursin20-25%ofuntreatedchildren,<5%if receive IVIg within 10 d of fever
• 50%ofaneurysmsregresswithin2yr
• anticoagulationformultipleorlargecoronaryaneurysms
• riskfactorsforcoronarydisease:male,age<1or>9yr,fever>10d,AsianorHispanicethnicity,
thrombocytopenia, hyponatremia