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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