Bronchiectasis PDF

Bronchiectasis Dr Ramez M Othman Pulmonary Consultant PMAH Bronchiectasis Syndrome Irreversible dilatation and destruction of bronchi & Inadequate clearance and pooling of mucus in the airways o Characterized by Persistent microbial infection and inflammatory re...

Bronchiectasis Dr Ramez M Othman Pulmonary Consultant PMAH Bronchiectasis Syndrome Irreversible dilatation and destruction of bronchi & Inadequate clearance and pooling of mucus in the airways o Characterized by Persistent microbial infection and inflammatory response Divided into Cystic Fibrosis (CF) and Non-CF forms CYSTIC FIBROSIS Most common genetic dz in the US (1 in 3,000 births in white population) Autosomal recessive with variable penetrance CF gene on the long arm of chromosome 7 Encodes the CF transmembrane regulator protein (CFTR) CFTR Mutations In lung, changes properties of the mucus layer lining the airways Impaired mucociliary clearance Persistent bacterial infection Increased inflammation (accumulation of cellular debris, including DNA and elastase) Airway obstruction Progressive lung dysfunction Fahy JV, Dickey BF. NEJM 2010; 363:2233--47 Management Clearanfor clearance of sputum, maintenance of lung function, and improved quality of life Chest physiotherapy Forced expiratory techniques Mechanical vests Flutter valves None proven superior to others Aerobic exercise recommended as an adjunct ce of airway secretions recommended for all patients with cystic fibrosis Pulmonary Complications Hemoptysis Pneumothroax Non-tuberculous mycobacterial infection ABPA Respiratory failure; cor pulmonale  H.Influenzae Chronic infec)on P.aeruginosa with respiratory S.Pneumoniae pathogens *S.aureus atypical Progression of airway damage Ly)c enzymes Inflamma)on released by with intense bacteria or PMN infiltra)on PMNs Diagnostic Testing In most patients CBC, IgG, IgA, IgM, spirometry, HRCT As directed by history RF, IgE, aspergillus precipitans, alpha-1 antitrypsin, sputum cultures for mycobacteria and fungi, sweat chloride testing Ciliary testing if hx of frequent upper respiratory infections or otitis, infertility Diagnostic Testing Bronchoscopy focal obstruction EM for cilia BAL for NTM Biopsy for diffuse bronchiolitis  Radiographic Findings Routine CXR Tram tracks Hyperinflation Ring shadows HRCT (gold standard) Internal bronchus diameter wider than adjacent artery Failure of bronchi to taper Excessive wall thickening Impacted mucus CXR HRCT  Therapy Treat underlying disease where possible Bronchodilators for AHR Airway clearance techniques Mucolytic agents Dornase alpha NOT recommended in non-CF patients Hypertonic saline and mannitol may be of benefit; used commonly, but not recommended routinely Exercise training Pneumococcal and influenza vaccination Therapy for Bronchiectasis Antibiotics Acute exacerbations, prevent exacerbations, reduce bacterial burden Pattern of colonization relatively stable over time Similar to patients with CF, chronic pseudomonas infection has been independently associated with mortality Small trials/BTS Guidelines support regular inhaled aminoglycosides for pseudomonas may be associated with increased bronchospasm No good evidence for use with other pathogenic organisms Macrolides in Non-CF Bronchiectasis 3 recently published trials suggest benefit (erythromycin, azithromycin) Reduced exacerbations Improved lung function in two of the trails Trend towards improved QOL THANK YOU