BRONCHIECTASIS

by dr shams rehan

Definition Bronchiectasis means permanent dilatation of bronchi. It can ensue whenever bronchial obstruction is accompanied by infection in the collapsed portion of lung distal to the obstruction

ClassificationCongenitalCiliary dysfunction syndrome Ciliary dyskinesia syndrome or immotile cilia syndrome Kartagener syndromeYoungs syndromeAlpha-1 antitrypsin deficiencyCystic fibrosisPulmonary sequestrationMarfans syndromeImmunodeficiencyPrimaryHypogammaglobulinemiaSecondaryCaused by cancer (CLL), chemotherapy, immune modulation AcquiredPost-infectious conditionsBacteria (pseudomonas, haemophilus)Mycobacterium tuberculosisAspergillus speciesVirus (adenovirus, measles, influenza, HIV)

ClassificationAcquired (contd)Diseases causing pulmonary fibrosisCFASarcoidosisAllergic alveolitisBronchial adenoma or CASequelae of toxic inhalation or aspirationChlorineOverdose (heroin)Foreign bodyRheumatic conditionsRheumatoid arthritisSLESjgrens syndromeRelapsing polychondritisOtherInflammatory bowel disease (chronic ulcerative colitis or crohns disease)Yellow nail syndrome (yellow nails and lymphedema)

TypesCylindrical or tubular bronchiectasis: Uniformly dilated tubes that end abruptly at the point that smaller bronchi are obstructed by secretionsVaricose bronchiectasis: The affected bronchi have irregular beaded pattern of dilatation that resemble varicose veinsSaccular (cystic) Bronchiectasis: The bronchi have a ballooned appearance at the periphery, ending in blind sacs without recognizable bronchial structures distal to that

Pathology

Clinical featuresSymptomsAsymptomaticCough Sputum productionHemoptysisDyspneaMalaise, fatigueFever WheezingSignsNo signsDigital clubbingFeverCoarse crepitationsCollapseFibrosis

Kartagener syndromeDefects in ciliary ultrastructure & motility, which ultimately impairs ciliary beating and mucociliary clearance in upper & lower respiratory tracts, paranasal sinuses & middle ear. Clinical presentationTriad Rhinosinus abnormalitiesSitus inversusBronchiectasisInfertilty

Youngs syndromeChronic sinusitisBronchitisBronchiectaissObstructive azospermia

Cystic fibrosisHereditary disorder in which activity of chloride channel known as cystic fibrosis transmembrane conductance regulator (CFTR) is reduced

Pulmonary disease persistent lower resp tract infection - Bronchiectasis Pancreatic insufficiencyRaised sweat chloride concentrationAzoopsermia (90%) congenital bilat absence of vas deferens

Comparison of Bronchiectasis & COPD

VariableCOPDBronchiectasisCauseCig smokingInfection, genetic or immune defectRole of infectionSecondaryPrimaryPredominant organism in sputumStrep pneumoniae, H influenzaeH influenzae, Ps aeroginosa Airflow obstruction & hyperresponsivenessPresentPresentFinding on CXRHyperlucency, hyperinflation, airway dilatationAirway dilatation & thickening, mucus plugsQuality of sputumMucoid, clearPurulent, three layered

InvestigationsSputum examination (microscopy, culture & AFBs)Chest X RaySinus radiographsHRCT Scan chestSpirometryBronchography (Historical)Fiberoptic bronchoscopySpecialized testsMucociliary function - ciliary beat frequencyImunoglobulin levelsSweat chloride test (>80mmol/l)Semen analysisTests for aspergillusAntibody testing Antigen detection in serum & urine

Chest XrayFocal pneumonitisScattered irregular opacitiesPulmonary collapse & fibrosisLinear or plate like atelectasisDilated & thickened airwaysRing shadows Tram-linesGloved finger shadowPulmonary hypertensionSitus inversusInvestigations

Chest X Ray

HRCT ScanRing shadows partially filled with secretionsMarkedly dilated airways

HRCT ScanSevere cystic bronchiectasis, cicatrization atelectasisDilated thick airways(signet rings)

SpirometryAirflow limitation (reduced FEV1/FVC)Normal or slightly reduced FVCReduced FEV1

ComplicationsInfection within dilated bronchiInfection spread to lungs and pleuraDistant spread of infectionGeneral effects of chronic infection within bronchiAmyloid diseasePneumothoraxCor pulmonaleRespiratory failureSecondary infectionInvasive broncho pulmonary aspergillosis

Causes of death from bronchiectasisMassive hemoptysisCor pulmonaleRespiratory failureSecondary infection leading to pneumonia and septicemiaInvasive bronchopulmonary aspergillosis

TreatmentPostural drainageAntibioticsAmoxicillin, AugmentinAzithromycin, ClarithromycinAntipseudomonal agent (Pseudomonas aeroginosa)Mucolytic agents Recombinant human DNAse (dornase alfa) Corticosteroids for airway obstruction, ABPAPrednisolone 30-40mg/day GERDImmunoglobulins- for immunodeficiency syndromes SurgeryPalliative surgical resection - Lobectomy, pneumonectomyHeart lung transplant