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Diffuse panbronchiolitis in an Australian aborigine James Brown  Graham Simpson Department Diffuse panbronchiolitis in an Australian aborigine James Brown  Graham Simpson Department

Diffuse panbronchiolitis in an Australian aborigine James Brown Graham Simpson Department - PDF document

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Diffuse panbronchiolitis in an Australian aborigine James Brown Graham Simpson Department - PPT Presentation

Correspondence James Brown Department of Thoracic and Sleep Medicine Cairns Base Hospital PO Box 902 Cairns 4870 Queensland Australia Email drjamesibrowngmailcom Received 18 December 2013 Revised 29 December 2013 Accepted 06 January 2014 Respirology ID: 34135

Correspondence James Brown Department

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DiffusepanbronchiolitisinanAustralianaborigineJamesBrown&GrahamSimpsonDepartmentofThoracicandSleepMedicine,CairnsBaseHospital,Cairns,Queensland,AustraliaKeywordsAustralianaborigine,bronchiolitis,erythromycin,macrolide.CorrespondenceJamesBrown,DepartmentofThoracicandSleepMedicine,CairnsBaseHospital,POBox902,Cairns,4870Queensland,Australia.E-mail:drjamesibrown@gmail.comReceived:18December2013;Revised:29December2013;Accepted06January2014RespirologyCaseReports2014;2(2):doi:10.1002/rcr2.50 revealeddiffusecoarsecracklesandscatteredexpiratoryrhonchi.Therewerenosignsofrightheartfailure.HischestradiographatpresentationshowedadiffusebilateralnodularinÞltrate.AhighresolutionCTscanofthechestisshowninFigure1.Twocuts,onejustbelowthelevelofthecarina(left)andonetowardsthelungbases,showadiffusenodularinÞltratewithextensivetree-in-budchange.Thereiscentrilobularbronchiectasiswithbronchialwallthickening,especiallyinthelowerzones.DetailedlungfunctiontestsareshowninTable1.Theinitialtestsshowevidenceofasevereobstructiveventila-torydefectwithhyperinßation.Thegastransferwasmildlyimpaired,butcorrectedtonormalforlungvolumes.TheresultsoflaboratoryinvestigationsareoutlinedinTable2.ThecoldagglutininandrheumatoidfactorlevelswerebothsigniÞcantlyelevated.Treatmentwithlow-doseerythromycinfor6monthsimprovedhissymptomsandhischestradiographreturnedtonormal.Repeatdetailedlungfunctionhadimproveddramatically(Table1). Figure1.High-resolutioncomputedtomographyscanofthethorax(twocuts).Table1.Detailedlungfunctiontests. UnitReferencePre-erythromycinPost-erythromycinSpirometryLiters2.961.20(40)2.12(72)FVCLiters3.782.08(55)3.16(84)/FVC%765867LungVolumeTLCLiters6.515.53(85)5.51(85)VCLiters3.932.08(53)3.16(81)RVLiters2.403.45(144)2.35(97)RV/TLC%396243FRCPLLiters3.47DLCOmL/mmHg/min25.816.7(64)28.2(110)DLCOAdjmL/mmHg/min25.816.7(64)28.2(110)VALiters2.964.79DLCO/VAmL/mmHg/min/L5.175.63(109)5.90(115)diffusingcapacityforcarbonmonoxide,DLCOAdjdiffusingcapacityforcarbonmonoxideadjustedforalveolarvolume,FEVforcedexpiratoryvolumein1sec,FRCPLfunctionalresidualcapacity,FVCforcedvitalcapacity,RVresidualvolume,TLCtotallungcapacity,VAalveolarvolume,VCvitalcapacity.Table2.Laboratoryinvestigations. TestResultColdagglutinintiter128Htiter(Rheumatoidfactor138IU/mL(ANAnegativeANCAnegativeHLAanalysis(ClassIserology)A24;A34;B56;B60;Bw6;Cw1;Cw3IgGlevel13.3g/L(7.0Ð16.0)anti-nuclearantibody,ANCAant-nuclearcytoplasmicanti-body,HLAhumanleukocyteantigen.J.Brown&G.SimpsonDPBinanAustralianaborigine©2014TheAuthors.RespirologyCaseReportspublishedbyJohnWiley&SonsLtdonbehalfofTheAsianPaciÞcSocietyofRespirology WepresenttheÞrstcaseofDPBinanindigenousAustral-ianman.Theclinical,serological,andradiologicalcriteriaforthediagnosisofDPBarefulÞlled.Inaddition,therewasresolutionofsymptomsandsigniÞcantimprovementinbothchestradiographandlungfunctiontestsaftertreat-mentwithlow-dosemacrolide.Withregardtoothersino-bronchialsyndromesthatneedexclusioninthiscase.YoungÕssyndromeisassociatedwithobstructiveazoospermia.Ourpatientisaparent.GoodÕssyndromerequiresthepresenceofathymomaandimmunodeÞciency.Ourpatienthasnormalimmunoglobu-linlevelsandnothymomaevidentonCTscanningofthethorax.HumanT-lymphotropicvirus(HTLV)infectionhasbeenassociatedwithbronchiectasisinindigenouspatientsinCentralAustralia.HTLV-1serologywasnonreactiveinthiscase.Althoughformalnasalbrushingandgeneticstudieswerenotperformed,primaryciliarydyskinesiadoesnotrespondtomacrolidetherapy.AgeneticsusceptibilitytothediseasehasbeenshowninpatientsintheFarEast.Thisisintheformofanassociationwithhumanleukocyteantigen(HLA)classIantigens.HLA-B54antigensaremorecommonlyobservedintheJapanese,Chinese,andKoreanswiththecondition[2,5].Thisparticulargenotypewasnotseeninourpatient.Chronicrespiratorydiseaseandbronchiectasisiscommonamongaborigines.TheetiologyremainsunclearandDPBmayexplainthis.DisclosureStatementsNoconßictofinterestdeclared.Appropriatewritteninformedconsentwasobtainedforpublica-tionofthiscasereportandaccompanyingimages.References1.BrownJ,andSimpsonG.2012.DiffusepanbronchiolitisinMelanesians.TSANZPosterAbstracts.Respirology2.KeichoN,andHijikataM.2011.Geneticpredispositiontodiffusepanbronchiolitis.Respirology16:581Ð588.3.CrosbiePAJ,andWoodheadMA.2009.Long-termmacrolidetherapyinchronicinßammatoryairwaydiseases.Eur.Resp.J.4.KanohS,andRubinBK.2010.Mechanismsofactionandapplicationofmacrolidesasimmunomodulatorymedications.Clin.Micro.Rev.23:590Ð615.5.SugiyamaY,KudohS,MaedaH,etal.1990.AnalysisofHLAantigensinpatientswithdiffusepanbronchiolitis.Am.Rev.Resp.Dis.141:1459Ð1462.J.Brown&G.SimpsonDPBinanAustralianaborigine©2014TheAuthors.RespirologyCaseReportspublishedbyJohnWiley&SonsLtdonbehalfofTheAsianPaciÞcSocietyofRespirology