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PEMSoft:HENOCHSCHONLEINPURPURA(HSP)

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HENOCHSCHONLEINPURPURA(HSP)
GENERALINFORMATION
Description
HenochSchonleinpurpura(HSP)isanacutesystemicimmunemediatedsmallvessel
vasculitisresultinginpalpablepurpurawithoutthrombocytopeniainmostlyinchildren
Nonthrombocytopenicpalpablepurpuraand1of
Abdominalpain
Acutearthritisorarthralgia
Renalinvolvement(proteinuriaorhematuria)
ImmunoglobulinA(IgA)predominantdepositsonbiopsy(usuallyskinorkidney)
Selflimitedcourse,butwithpersistentrenalsequelaeinsomepatients
Mostcommonvasculitisofchildhood
Diagnosisisbasedupontheclinicalmanifestationsofthedisease
Alsocalled
Allergicpurpura
Allergicvasculitis
Anaphylactoidpurpura
Autoimmunepurpura
Henoch'spurpura
Nonthrombocytopenicpurpura
SchonleinHenochpurpura
Clinicalpresentation
Rash
Presentingsymptomin75%andeventuallyoccursin100%ofcases
Lesionsappearincropslasting3to10days
Morphology
Oftenbeginsaserythematous,macular,orurticarialwhealsonlegs
Symmetric(bothsides)ingravityorpressuredependentareas
Maximalonlowerlimbsandbuttocksespeciallyintoddlers
Face,trunk,andupperextremitiesinnonambulatorychildren
Mayincludemacules,papules,vesicles,bullae,nodulesandurticaria
Rashinitiallyblanchesthendevelopsintononblanchingpalpablepurpura
Lesionsmaybepainfulorassociatedwithfeverorangioedema
Localangioedema
Scalp,eyelids,lips,periorbital,hands,feet,back,scrotumandperineum
Morecommonatage<3yearsbutcanoccuratanyage
Edemamayprecedetheskinlesions
Scrotalpainmimickingtesticulartorsionmaybethepresentingsymptom
Gastrointestinal(GI)involvement
GIsymptomsin50to67%
Colickydiffuseabdominalpain+/vomiting+/bloodydiarrhea
GIsymptomsmayprecedetherashin15to35%
GIsymptomswithoutanyrashhavebeendescribed
Arthritis/arthralgia
Presentin75%andpresentingcomplaintin10to15%
Differentialdiagnosiswidewhenjointcomplaintsprecedetherash
Mainlylargejointsespeciallykneesandankles
Serouseffusionsresolvequicklybutmayrecur
Noresidualjointdeformity
Nephritis
Microscopichematuria+/proteinuriain60to70%butonly1%goontoendstage
renaldisease
SeeHYPERTENSION
Othersystems
Pulmonaryinvolvementrare,butifpresentmayhaveralesonlungexamination
Alteredlevelofconsciousness,convulsions,andfocalneurologicaldeficits
BASICSCIENCE
Causes
Causeisunknown
Hypothesesincludegeneticpredispositioncombinedwithantigenicstimulus(suchas
infections,drugs,toxins)thattriggersdisease
Pathogenesis
ImmunemediatedvasculitiswithIgAimmunecomplexdepositionresultingin
leukohistiocyticsmallvesselvasculitisinskinandaffectedorgan
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Vesselwallsofarterioles,capillariesandespeciallypostcapillaryvenulesaffectedwith
neutrophilicinfiltrateandfibrinoidnecrosis
ImmunofluorescenceshowsdepositionofIgA,C3andfibrin(alsoIgGinrenalvessel
walls)
SeveralstudiesreportelevatedIgAandIgAimmunecomplexes,IgAanticardiolipin
antibodiesandtransforminggrowthfactorbeta
OnlyIgA1isfoundintheinflammatoryinfiltrates,notIgA2
Triggers
Exposuretoantigenintheformofinfectiousagent,drug,chemical,foreignor
endogenoussubstanceortoxinincludinginsectbites
Approximatelyhalfofcasesareprecededbyanupperrespiratoryinfection
Oftenpoststreptococcalbutmanyotheragentsimplicatedeg,Bartonellaspecies,
hepatitisA,Haemophilusspecies,parainfluenza,Staphylococcalinfection,
Mycoplasma
EPIDEMIOLOGY
Whoismostaffected
Peakincidenceatage4to7years
Mostareage<10years
Recentstudiesshowequalincidencemalesandfemales
LessfrequentlyinblackchildrencomparedtowhiteorAsianchildren
Incidence
Overallincidenceabout10to30per100,000childrenage<17yearsdependingon
geographicregion
Annualincidence2027per100,000childrenand12per100,000adultsinScotland
from1995to2007(Penny2010)
BasedonreviewofhospitaldischargediagnosesfromScottishMorbidityDatabase
Estimatedannualincidence20.4per100,000childreninUnitedKingdom(Gardner
Medwin2002)
Highestincidence(70.3per100,000)inchildrenaged46years
Annualincidenceof12.9per100,000children<17yearsoldinTaiwanfrom1999to
2002(Yang2005)
Annualincidenceestimatedat10.2per100,000children<17yearsoldinCzech
Republicfrom1997to1999(Dolezalova2004)
Annualincidenceof6.1per100,000childrenaged018years,including14.9per
100,000childrenaged36yearsinNetherlandsin2004(Aalberse2007)
Seasonality
Morecommoninwinterandcoolermonths
Riskfactorsfortheeventualdevelopmentofchronicrenalfailure
Over4ageyearsatonset
Renalinvolvementin50%ofolderchildrenbutinonly25%<age2years
Acutenephritic/nephroticatthetimeofdiagnosis
50%ofthesechildrenmayprogresstochronicrenalinsufficiency
Persistentpurpuriclesionsoriftherearebloodystools
Severegastrointestinalsymptoms
Renalfailureatthetimeofdiagnosis
LowfactorXIIIactivity
Greaterthan50%crescentsoninitialrenalbiopsy
DIAGNOSTICSTUDIES
Makingthediagnosis
HSPisprimarilyaclinicaldiagnosisbasedonacuteonsetofpurpuricrashnotrelatedto
thrombocytopeniaoverthelowerlimbsandbuttocksusuallyinassociationwithsome
combinationofabdominalpain,jointinvolvement,angioedemaandhematuria+/
proteinuria
Checkbloodpressure
Testingoverview
Bloodtestsnotsuggestiveofseriousinfectionorcoagulationdisorder
Laboratoryfinding
Completebloodcount(CBC)nonspecific
Thrombocytosis,leukocytosisandeosinophilia
ESRandCRPmaybeelevated
Coagulationprofile
Plateletcount,PTandPTTallnormal
Chemistrypanelincludingelectrolytes,bloodureanitrogen,andcreatinine
SerumIgAelevatedinabout50%
ConsiderantistreptococcalDNaseBandantihyaluronidasetodetectrecentinfection
Peaksat4to6weekslastingupto4months
Normalurineatpresentationin66%ofchildrenwithHSP(Narchi2005)
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SeeHEMATURIA
SeePROTEINURIA
Imaging
Obtainabdominalultrasoundifintussusceptionsuspected
Considerrenalultrasoundifrenalimpairment
Considerscrotalultrasoundifseverescrotalpainpresent
Histology
Punchbiopsyofskinlesionifthereisdoubtaboutdiagnosis
HSPshowsleukoclasticangitiswithIgAimmunofluorescence
NoIgAimmunofluorescenceinacutehemorrhagicedema
Renalbiopsyifnephritissuspected
SeeRENALBIOPSY
Rangesfrompatchymesangialproliferationtocrescenticglomerulonephritis
ImmunofluorescenceshowsIgAdepositioninthemesangium
MoreseverecasesshowIgG,fibrin,C3,andproperdinintheglomeruli
DIFFERENTIALDIAGNOSIS
Nonblanchingrashpresent
SeeACUTEHEMORRHAGICEDEMA
Autoimmunedisease
SeeSLE
Antiphospholipidantibodieseg,anticardiolipinantibodiessuggestautoimmunecause
SeeIMMUNETHROMBOCYTOPENICPURPURA
SeeCOAGULOPATHY
SeePLATELETDYSFUNCTION&THROMBOCYTOPENIA
Infections
SeeMENINGOCOCCEMIA
SeeROCKYMOUNTAINSPOTTEDFEVER
SeeVASCULITIS
SeePOLYARTERITISNODOSA
SeeKAWASAKIDISEASE
Rashabsentordelayed
Abdominalpresentation
SeeACUTEABDOMEN
Jointpresentation
SeeARTHRITIS
Renalpresentation
SeeNEPHRITIS
TREATMENT
Treatmentoverview
Treatanyetiologicagentorcause
Supportivemanagementunlesssevereskinororganinvolvement
Adequatehydrationandblanddiet
Paincontrol(avoidNSAIDSifsignificantrenaldisease)
Restandelevationofextremitiesmaypreventpurpuraduringactivephaseofdisease
Lesionsmayrecurasactivitylevelincreases
Steroids
Commonlyusedfortheshorttermreliefofpainanddiscomfort
Steroidsreducethedurationjointpain(Ronkainen2006)
Usefulforsevereabdominalpain(somesmallstudyevidence)
Painfuledemaandscrotalswelling
Severearthralgia
Steroidscommonlygivenformarkedproteinuriaorimpairedrenalfunctionbutno
evidenceforsignificantbenefit
MetaanalysisofMedlineandtheCochraneControlledTrialsRegistersuggeststhat
steroidsdonotreducepersistentrenaldisease(Hahn2015)
Dosage
Prednisone/prednisolone1mg/kginshorttaperingregime
Ceasingwithouttaperingmayresultinrecurrenceofabdominalpain
SeeSTEROIDS
Nephritis
EarlymanagementinconjunctionwithaNephrologistmayhelptomitigatefurtherrenal
involvement
Noevidencethatsteroidspreventrenalfailure
Controversyastowhethersteroidspreventmildnephritis
Considermethylprednisolonepulsesinseverecases
Plasmapheresis,cyclophophamideorazathioprine,urokinase,cyclosporinhavebeen
usedinseveresteroidresistantcaseseg,crescenticnephritis
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Persistentproteinuriaoftentreatedwithangiotensinconvertingenzymeinhibitors
(ACEI)orangiotensinreceptorblockers
KidneyDisease:ImprovingGlobalOutcomes(KDIGO)recommendationbasedon
extrapolationfromstudiesinchildrenandyoungadultswithimmunoglobulinA(IgA)
nephropathy
Longtermefficacyunknown
Severegastrointestinalinvolvement
Earlyuseofcorticosteroidsassociatedwithdecreasedneedforabdominalsurgeryor
endoscopyinchildrenhospitalizedforHSP(Weiss2010)
PlasmaexchangetherapyhasbeenusedsuccessfullyforsevereGImanifestationsbased
oncasereports
Renalconsultation
Renalconsultantinvolvementrecommended,especiallywithprogressivedeteriorationor
lackofresponsetosteroids
COMPLICATIONS
Acutecomplications
Gastrointestinal(GI)complications
GIarethemostseriousacutecomplications
GIsymptomsandguaiacpositivestoolisfoundinjustoverhalfofcases
Usuallywithin8daysaftertherashbutintervalcanbemuchlonger
Submucosalhemorrhageandedemacauseabdominalpain,nausea,vomiting,and
transientparalyticileus
Purpuriclesionsmaybeseenonendoscopyinthedescendingduodenum,stomach,
andcolonandtheterminalileumalsomaybeinvolved
Submucosaledema,ulceration,andspasmoftheileumandjejunummaybeseenin
smallbowelseries
SignificantanemiamaydevelopfromGIbleeding
Intussusceptionincidenceestimatesvariesfrom0.5to3.5%ofallcasesofHSP
Limitedtothesmallbowelinmajoritywhereasnormalintussusceptionistypically
ileocolic
SeeINTUSSUSCEPTION
RareGImanifestations
SeePANCREATITIS
SeeCHOLECYSTITIS
SeePROTEINLOSINGENTEROPATHY
Massivegastrointestinalhemorrhageisrareasisbowelischemiaandperforation
Diagnosticstudies
Useultrasoundbecausecontrastenemascannotdetectileoilealintussusception
Renalcomplications
91%haverenalinvolvementwithin6weeksafterpresentation
Raremorethan6monthsafteronsetofHSP
Morecommoninolderchildren
RecurrentgrosshematuriawithURIinfections
Depressedcomplementlevelsinonethirdofpatients
Renalinvolvementmaydevelopwithoutspecificsymptomsorclearsigns
SeePROGNOSIS
Othercomplications
Rarecentralnervoussystem(CNS)involvement,usuallytransient
SeeSEIZURES
Headache,focalneurologicdeficits,ataxia,CNSbleeds,paresisandcoma
Alsoraremononeuropathies
Hepatosplenomegalyandadenopathy
SeeTESTICULARTORSION
Associatedwithscrotaledema
Orchitis
Cardiacinvolvementrare
Pulmonary
Mildinterstitialchanges
Rarepulmonaryhemorrhage
Eyeinvolvementrare
Keratitis,uveitis
Associateddisorders
SeeDIABETESMELLITUS
SeeHEMOPHILIA
SeeINTUSSUSCEPTION
PROGNOSIS
Renalimpairment
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Isolatedhematuriaandproteinuriawithoutrenalinsufficiencycompletelyrecovers
Mostselfresolvingoveraperiodof6to8weeks
94%completerecoveryby18months
Acutenephritisand/ornephroticsyndromeoccursin6%7%ofchildrenwithHSP
Amongpatientswithrenalcomplications(KDIGO2012)
91%developsymptoms6weeksfromdiseaseonset
97%developsymptomswithin6months
13%progresstoendstagerenaldisease
Significantrenaldiseasemorelikelyifhematuriaandproteinuriaatonset,nephritic
rangeproteinuria,elevatedserumcreatinine,hypertension
SomewhoappeartorecovermaydevelopsignificantrenaldiseasemanyyearslaterE
Recurrence
Recursinapproximatelyonethirdusuallywithinfourmonthsoftheinitialepisode
Milderandshorterthantheinitialepisodebutmorecommonifinitialepisodesevere
eg,elevatedESRorCRPindicatingacuteinflammationoruseofsteroidsneeded
Furtherrelapsesoccurinaboutonethirdoverthesubsequentyear
DISPOSITION
Admittohospital
Severeabdominalorjointpain
Vomitingcausingdehydration
Significanthypertension
Renaldysfunction
Gastrointestinalhemorrhage
Orchitis
Inabilitytoambulate
Outpatientmanagement
Ifdoesnotmeetcriteriaabove,reliablecaretakers
Followup
Followcloselytomonitorforseveresymptoms
Bloodpressure(BP)surveillanceandurinalysis(UA)for12monthsafteranacute
episodeofHSPtolookfordelayedrenalinsufficiency
CheckBPandUAweeklyfor4weeks
ThencheckBPandUAeveryotherweekduringweeks5to12ofillness
ThencheckBPandUAat6andat12monthspostdiagnosis
Ifmicroscopichematuriaat12months,continuetomonitorannually
Ifurinalysiscompletelynormal,maystopmonitoring
EDUCATION/PREVENTION
NopreventativemeasuresforHenochSchonleinpurpura
TIPS
Classicrashisnottheinitialpresentingsigninaboutonequarterofpatients
Somechildrenpresentwithisolatedabdominalsymptomsorarthritis
Diagnosismayonlybecomeapparentwhentherashdevelopsdaystoweekslater
Considerotherorgansystemsevenifinvolvementislimitedtotheskin
Goodprognosisifnorenalinvolvement
Renalbiopsynotindicativeoffuturecomplications
Checkbloodpressureateachfollowupvisit
ACUTEHEMORRHAGICEDEMAOFINFANCY(AHEI)
Description
Benign,selflimitedleukocytoclasticvasculitiswithoutIgAimmunoflourescence
AHEIhadbeenconsideredavariantofHSPbutnowisrecognizedasadistinctentity
becauseofthelackofGIinvolvement
LackstheIgAdepositionseeninHSP
Cause
Causeunknownbutoftenprecededbyrespiratorytractinfection,immunizationsor
medicationeg,antibiotics
SeeHYPERSENSITIVITYANGIITIS
Epidemiology
Rare
YoungeragethanHSP
Usuallychildren4monthstoage3yearswitharecenthistoryofarespiratoryillness
4timesmorecommonamongmaleinfantsthanamongfemales
Clinicalpresentation
Characterizedbythetriadoffever,edema,androsette,annularortargetpurpura
primarilyovertheface,ears,andextremitiesinanontoxicinfant
Developrapidly,with/withoutpremonitorysymptoms(fever,irritability,fatigue)
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Patientotherwisenontoxicappearing
Lesionsresolveoverseveraldaysonlytobefollowedbyanothercropoffreshlesions
Cutaneousfindingsaredramaticbothinappearanceandrapidonsetover24to48
hours
Infiltrated,tenderpurpuricswellingonface,ears,armsandlegswithsparingofthe
trunk
Rosette,annularortargetshapedpurpura
Maystartasredtopurpleurticarialplaques
Mayhavesharpbordersandmaybetargetoidinappearance
Purpuramayinvolvethescrotum
PurpuraoftheumbilicuscanbemistakenfortheCullensign
Vesiclesorbullaemaybepresentinupto10%ofcases
Resolveoverseveraldaysonlytobefollowedbyanothercropoffreshlesions
Associatedsuddensymmetricedemaandswellingoftheface,handsandfeet
Acraledemainvolvingthedorsumofthehandsandfeetfrequentlyextendsproximally
uptheextremities
NoneofthesystemicinvolvementoftenseenonHSP
Diagnosticstudies
Makingthediagnosis
AHEIisprimarilyaclinicaldiagnosisbasedonacuteonsetofdramaticpurpuricrashnot
relatedtothrombocytopeniainanontoxicyoungchild
Testingoverview
Bloodtestsnotsuggestiveofseriousinfectionorcoagulationdisorder
Laboratoryfindings
Completebloodcount(CBC)nonspecific
Mayhavemildleukocytosis
ESRandCRPmaybeelevated
Coagulationprofile
Plateletcount,PTandPTTallnormal
Chemistrypanelincludingelectrolytes,bloodureanitrogen,andcreatinine
Liverfunctiontestsmaybeelevated
C3andC4levelsarenormal
Biopsyfindingswillbeconsistentwithleukocytoclasticvasculitisofthedermalvessels
withfibrinoidnecrosis
Differentialdiagnosis
Recurrentbruising
SeeCHILDMALTREATMENT
Recurrentswelling
SeeHEREDITARYANGIONEUROTICEDEMA
Complications
Residualhyperpigmentationorscarring
Gastrointestinalbleeding
SeeINTUSSUSCEPTION
SeeNEPHRITIS
Prognosis
PrognosisbetterthanHSP
Eruptionsusuallyremitspontaneouslywithin60days
Resolvewithin21daysin80%
Treatment
Noeffectivetherapy
Systemicsteroidsdonotappeartoalterthediseasecourse
EVIDENCE
EvidencesupportedbyDynaMedSystematicLiteratureSurveillance
HahnD,HodsonEM,WillisNS,CraigJC.Interventionsforpreventingandtreatingkidney
diseaseinHenochSchonleinPurpura(HSP).CochraneDatabaseSystRev.2015Aug
78:CD005128
Shorttermprednisonetreatmentmaynotreduceriskofpersistentkidneydiseasein
childrenwithHenochSchonleinpurpurabasedonCochranesystematicreviewof13
randomizedtrialsevaluatinginterventionsforpreventionortreatmentofkidney
diseasein1,403patientswithHenochSchonleinpurpura
5trialscomparedprednisonetoplaceboorsupportivetreatment
3trialsenrolledpatientswithexistingkidneydisease
Prednisoneregimensincludedprednisone1mg/kg/dayorallyfor1014days(3
trials)andprednisone2mg/kg/dayorallyfor7days(2trials)
Mostregimensincludedsteroidtaperingovernext12weeksafterinitial714days
oftreatment
Resultsforpersistentkidneydiseaselimitedbyconfidenceintervalincluding
clinicallymeaningfuldifferences
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Comparingprednisonetoplaceboorsupportivetreatment
Nosignificantdifferencesinpersistentkidneydiseaseat612months(riskratio
0.74,95%CI0.421.32)inanalysisof5trialswith746children
Nosignificantdifferencesinseverekidneydiseaseinanalysisof2trialswith418
children
Nosignificantdifferencesinhematuria,proteinuria,orhypertensionat8year
followupin1trialwith138children
Noseriousadverseeffectsin2trialswith211children
WeissPF,KlinkAJ,LocalioR,etal.Corticosteroidsmayimproveclinicaloutcomesduring
hospitalizationforHenochSchonleinpurpura.Pediatrics2010Oct126(4):67481
Earlyuseofcorticosteroidsassociatedwithdecreasedneedforabdominalsurgeryor
endoscopyinchildrenhospitalizedforHSPbasedonretrospectivecohortstudyof
1,895childrenadmittedtohospitalfornewonsetHSP
42%receivedcorticosteroid2days(early)
8%receivedcorticosteroidsduringdays34(midinitiation)
7%receivedcorticosteroids>4days(lateinitiation)
Earlycorticosteroidusecomparedtolateinitiationornocorticosteroidsassociated
withdecreasedriskof
Abdominalsurgery(hazardratio[HR]0.39,95%CI0.170.91)
Endoscopy(HR0.27,95%CI0.130.55)
Abdominalimaging(HR0.5,95%CI0.290.88)
Reductioninendoscopyandabdominalimagingremainedsignificantafteradjustment
forsevereillnessduringday12
GUIDELINES
Internationalguidelines
KidneyDiseaseImprovingGlobalOutcomes(KDIGO)clinicalpracticeguidelineon
glomerulonephritiscanbefoundatKDIGO2012JunPDForatNationalGuideline
Clearinghouse2013Jan28:38244
UnitedStatesguidelines
AmericanSocietyforApheresis(AFSA)guidelineontheuseoftherapeuticapheresisin
clinicalpracticecanbefoundinJClinApher2013Jul28(3):145
AustralianandNewZealandguidelines
RoyalChildren'sHospitalMelbourne(RCHM)clinicalpracticeguidelineonHenoch
SchonleinpurpuracanbefoundatRCHMSep2012
REFERENCES
TopicsupportedbyDynaMedSystematicLiteratureSurveillance
AalberseJ,DolmanK,RamnathG,PereiraRR,DavinJC.HenochSchonleinpurpurain
children:anepidemiologicalstudyamongDutchpaediatriciansonincidenceand
diagnosticcriteria.AnnRheumDis2007Dec66(12):164850
AmitaiY,GillisD,WassermanD,KochmanRH.HenochSchonleinpurpuraininfants.
Pediatrics.1993Dec92(6):8657
BlumanJ,GoldmanRD.HenochSchnleinpurpurainchildren:Limitedbenefitof
corticosteroids.CanFamPhysician.2014Nov60(11):10071010
ChartapisakW,OpastirakuS,WillisNS,CraigJC,HodsonEM.Preventionandtreatment
ofrenaldiseaseinHenochSchonleinpurpura:asystematicreview.ArchDisChild.2009
Feb94(2):1327
CiceroM.Rashdecisions:acutehemorrhagicedemaofinfancyina7montholdboy.
PediatrEmergCare.2008Jul24(7):5012
DolezalovaP,TelekesovaP,NemcovaD,HozaJ.Incidenceofvasculitisinchildreninthe
CzechRepublic:2yearprospectiveepidemiologysurvey.JRheumatol2004
Nov31(11):22959
DudleyJ,SmithG,LlewelynEdwardsA,BaylissK,PikeK,TizardJ.Randomised,double
blind,placebocontrolledtrialtodeterminewhethersteroidsreducetheincidenceand
severityofnephropathyinHenochSchonleinPurpura(HSP).ArchDisChild2013
Oct98(10):75663
FioreE,RizziM,SimonettiGD,GarzoniL,BianchettiMG,BettinelliA.Acutehemorrhagic
edemaofyoungchildren:aconcisenarrativereview.EurJPediatr2011
Dec170(12):150711
FotisL,NikorelouS,LariouMS,DelisD,StamoyannouL.Acutehemorrhagicedemaof
infancy:afrighteningbutbenigndisease.ClinPediatr(Phila)2012Apr51(4):3913
GardnerMedwinJM,DolezalovaP,CumminsC,SouthwoodTR.IncidenceofHenoch
Schnleinpurpura,Kawasakidisease,andrarevasculitidesinchildrenofdifferent
ethnicorigins.Lancet2002Oct19360(9341):1197202
GedaliaA.HenochSchnleinpurpura.CurrRheumatolRep.2004Jun6(3):195202
JennetteJC,FalkRJ.Smallvesselvasculitis.NEnglJMed.1997Nov20337(21):1512
23
KarremannM,JordanAJ,BellN,etal.Acutehemorrhagicedemaofinfancy:reportof4
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casesandreviewofthecurrentliterature.ClinPediatr(Phila).2009Apr48(3):3236
KawasakiY,SuzukiJ,SakaiN,etal.Clinicalandpathologicalfeaturesofchildrenwith
HenochSchoenleinpurpuranephritis:riskfactorsassociatedwithpoorprognosis.Clin
Nephrol.2003Sep60(3):15360
KidneyDisease:ImprovingGlobalOutcomes(KDIGO)GlomerulonephritisWorkGroup.
KDIGOClinicalPracticeGuidelineforGlomerulonephritis.KidneyIntSuppl(2011).2012
Jun2(2):142
LanzkowskyS,LanzkowskyL,LanzkowskyP.HenochSchonleinpurpura.PediatrRev.
1992Apr13(4):1307
McCarthyHJ,TizardEJ.Clinicalpractice:DiagnosisandmanagementofHenoch
Schnleinpurpura.EurJPediatr.2010Jun169(6):64350
NarchiH.Riskoflongtermrenalimpairmentanddurationoffollowuprecommendedfor
HenochSchonleinpurpurawithnormalorminimalurinaryfindings:asystematic
review.ArchDisChild.2005Sep90(9):91620
OliveiraJA,LopesL,FragaA,SoaresdeAlmeidaL,guasB,SiborroAzevedoA.Acute
hemorrhagicedemaofinfancy:ararecauseofpurpuricexanthema.JPediatr2015
Feb166(2):49898.e1
PennyK,FlemingM,KazmierczakD,ThomasA.AnepidemiologicalstudyofHenoch
Schnleinpurpura.PaediatrNurs2010Dec22(10):305
RiganteD,CandelliM,FedericoG,etal.Predictivefactorsofrenalinvolvementor
relapsingdiseaseinchildrenwithHenochSchnleinpurpura.RheumatolInt.2005
Jan25(1):458
RonkainenJ,KoskimiesO,AlaHouhalaM,etal.EarlyprednisonetherapyinHenoch
Schnleinpurpura:arandomized,doubleblind,placebocontrolledtrial.JPediatr2006
Aug149(2):2417
SaulsburyFT.Clinicalupdate:HenochSchonleinpurpura.Lancet.2007Mar
24369(9566):9768
TingTV,HashkesPJ.Updateonchildhoodvasculitides.CurrOpinRheumatol.2004
Sep16(5):5605
TrapaniS,MicheliA,GrisoliaF,etal.HenochSchnleinpurpurainchildhood:
epidemiologicalandclinicalanalysisof150casesovera5yearperiodandreviewof
literature.SeminArthritisRheum.2005Dec35(3):14353
WeissPF,FeinsteinJA,LuanX,BurnhamJM,FeudtnerC.EffectsofCorticosteroidon
HenochSchoenleinPurpura:ASystematicReview.Pediatrics.2007Nov120(5):1079
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May44(5):61822
SEEALSO
SeeABDOMINALPAIN
SeeARTHRITIS
SeeBLEEDING
SeeCHRONICRENALFAILURE
SeeCOAGULOPATHY
SeeDISSEMINATEDINTRAVASCULARCOAGULATION
SeeEPISTAXIS
SeeHEMATURIA
SeeHEMOPHILIAA
SeeHEMOPHILIAB
SeeIDIOPATHICTHROMBOCYTOPENICPURPURA(ITP)
SeePLATELETDYSFUNCTION&THROMBOCYTOPENIA
SeeVASCULITIS

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