Bronchiectasis Core Conference - PACCM @...
Transcript of Bronchiectasis Core Conference - PACCM @...
12/8/16
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Non-CFBronchiectasis
ClinicalFeatures OtherSuggestiveFactors• Difficulttotreatasthma• NonsmokersdiagnosedwithCOPD• PseudomonasaeruginosaorNTMinsputum
• Chroniccoughandsputum• Hemoptysis• Dyspnea• Weightloss• Recurrentpulmonaryinfections• Recurrentrhinosinusitis• Wheezing• Fatigue
Pathophysiology
DifferentialDiagnosis
Etiologybasedondisease location
First excludecystic fibrosis• Sweat test: <40normal, 40–
60borderline, >60abnormal• CFTRmutation analysis (most
commongenes) or fullsequencing
• Nasal PD, semen analysis
Diagnosis ofPCD• Nasal nitric oxide testing canbeused asascreening test – levels will be low• Carinal ornasalbiopsy with electron microscopy examination ofcilia is the
gold standard fordiagnosis• Nasal scrapings at specialized researchcenters• Genetic testing –mutations in the DNAI1 andDNAH5 genes account forup to
30%patients, extended genetic testing encompasses 70%patients
Antibiotics=cornerstoneof treatment
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ManagementPearls• DNAse:patientstreatedfor24weeks(BIDdosing)hadincreasednumberofpulmonaryexacerbationsandgreaterdecline inFEV1– nottypicallyused• Corticosteroids:notroutinelyusedunlesstheyarepartofthetreatment forunderlyingcause(ABPA)• Antibioticsduringanexacerbation• Typicallychosenbasedonpriorculturedata• ConsiderchoosingsomethingwithactivityagainstPAifnopriorculturedata• BritishThoracicSocietyrecommendscombinationantibioticsforMDRPA• Durationofantibioticsisnotclearcutbut2weeksistypicallyrecommended
PrimaryCiliaryDyskinesia
Absence of inner andouter dynein arms
Kartagener’ssyndrome
• Triad ofbronchiectasis,rhinosinusitis, andsitusinversus• Accounts forabout50%ofpatients withPCD
Bronchiolar Disorders
• Diffusepanbronchiolitis• Idiopathicinflammatorydisease• MostwellrecognizedinJapan• Coexistingsinusitis• Treatmentwithmacrolides
• Constrictivebronchiolitis• Postviral• GVHD
• Diameteroftrachea>3cm• Diameterofrightmainbronchus>2.4cm• Diameterofleftmainbronchus>2.3cm• Inefficientcoughmechanismleadstoimpairedmucociliaryclearance
Tracheobronchomegaly(Mounier Kuhn Syndrome)
• malformationsaffectingthefingernailsandtoenails• Pleuraleffusions,recurrentsinopulmonary infections,bronchiectasis• lymphedema
Cartilage deficiency(Williams-Campbell)
• absenceofcartilageinsubsegmental bronchi• Leadstodistalairwaycollapse
Yellow Nail Syndrome
Young’s Syndrome• Bronchiectasis,sinusitis,andobstructiveazoospermia• NeedtoexcludecysticfibrosisandPCD
Job’s Syndrome• Autosomaldominanthyper-IgE syndrome• triadofeosinophilia,eczema,andrecurrentskinandpulmonaryinfections
References• Guidelines fornon-CFbronchiectasisBritish ThoracicSocietyGuidelineGroup
• McShanePJ,etal. Non-cysticfibrosis bronchiectasis. AmJRespir Crit CareMed.2013
• Altenburg J,etal. Effectofazithromycinmaintenance treatmentoninfectiousexacerbationsamongpatients withnon-cystic fibrosisbronchiectasis: theBAT randomizedcontrolled trial. JAMA.2013
• Serisier DJ,MartinML,McGuckinMA, etal. Effectoflong-term, low-doseerythromycinonpulmonaryexacerbationsamongpatients withnon-cysticfibrosis bronchiectasis: theBLESSrandomizedcontrolled trial. JAMA.2013
• O’Donnell AE,BarkerAF, I lowite JS,etal. Treatmentofidiopathicbronchiectasis withaerosolized recombinanthumanDNaseI. Chest1998
• Scheinberg,etal. Apilot studyof thesafetyandefficacyoftobramycinsolution for inhalation inpatientswith severebronchiectasis. Chest.2005