Bronchiectasis

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Bronchiectasis : 

Bronchiectasis October 12th, 2009 Sarayuth Eiamsa-ard.,M.D. Chest Fellow, Division of Pulmonology and pulmonary critical care. Prince of Songkhla University.

Bronchiectasis : 

Bronchiectasis Bronkos + Ectasia = Bronchi + Dilatation

Bronchiectasis : 

Bronchiectasis Permanent dilatation of bronchi with destruction of elastic and muscular component of their, usually due to acute or chronic infection. (AACP evidence base clinical practice guideline 2006) The permanent dilatation of bronchi ( Lyne Reid since 1950)

Pathophysiology : 

Pathophysiology Bronchi and bronchioles involving a vicious cycle of transmural infection and inflammation with mediator release. (NEJM 2002 ) 2 main insults - Infectious insult - Impairment of drainage, airway obstruction, and/or a defect in host defense.

Pathophysiology : 

Pathophysiology infection Retained secretion and inflammation Airway destruction and remodeling

Bronchiectasis : 

Bronchiectasis Focal presentation Diffuse presentation

Focal bronchiectasis : 

Focal bronchiectasis Local or focal obstructive process of a lobe or segment of a lung 3 types of focal airway obstruction 1) luminal blockage by a FB, broncholith or slowly growing tumor (usually benign) 2) extrinsic narrowing : Enlarged LN  Middle lobe syndrome 3) Twisting of displacement of the airway after a lobar recretion

Diffuse presentation : 

Diffuse presentation Involving much of both lungs Often accompanied by other sinopulmonary disease e.g. sinusitis and asthma

Slide 9: 

Radio Clin N Am 47 2009 289-306

3 (imaged) forms of bronchiectasis : 

3 (imaged) forms of bronchiectasis Cylindrical/tubular bronchiectasis 2. Varicose bronchiectasis 3. Cystic/saccular bronchiectasis

Cylindrical/tubular bronchiectasis : 

Cylindrical/tubular bronchiectasis Uniform luminal dilatation The wall thickening is smooth

Cylindrical/tubular bronchiectasis : 

Cylindrical/tubular bronchiectasis characterized by dilated airways alone and is sometimes seen as a residual effect of pneumonia

Cylindrical/tubular bronchiectasis : 

Cylindrical/tubular bronchiectasis

Cylindrical/tubular bronchiectasis : 

Cylindrical/tubular bronchiectasis

Varicose bronchiectasis : 

Varicose bronchiectasis

Cystic/saccular bronchiectasis : 

Cystic/saccular bronchiectasis Most severe form of bronchiectasis Cyst like Dilated, thick-walled bronchus terminates in a thick-walled cyst

Cystic/saccular bronchiectasis : 

Cystic/saccular bronchiectasis

Cystic/saccular bronchiectasis : 

Cystic/saccular bronchiectasis

Cystic/saccular bronchiectasis : 

Cystic/saccular bronchiectasis

Clinical manifestation : 

Clinical manifestation Cough (90 %) Daily sputum production (76%) Dyspnea (72%), Wheezing Hemoptysis (56%) Recurrent pleurisy Dry Bronchiectasis Acute exacerbation Late : hypoxemia and hypercapnia

Clinical manifestation : 

Clinical manifestation Abnormal lung sound (Crackles, wheezes, rhonchi) Clubbing of finger 3-24% Chest X ray : abnormal (>90%) Non specific : focal pneumonitis, scattered irregular opacities, linear or plate-like atelectasis Specific : dilated and thickened airways that appear as ring-like shadows (of airways that are seen on end) or tram lines (in the case of airways that are perpendicular to the x-ray beam)

CT finding : 

CT finding Internal bronchial diameter greater than that of the adjacent pulmonary artery Lack of bronchial tapering Presence of bronchi within 1 cm of the costal pleura Presence of bronchi abutting the mediastinal pleura Bronchial wall thickening

Bronchiectasis : 

Bronchiectasis UK THAI 53% idiopathic 36% idiopathic 29% Postinfectious 32% TB 8% Immune defect 10% Severe pneumonia 7% ABPA 4% Diffuse panbronchiolitis 4% Aspiration 4% HIV 3% Young’s syndrome 4% RA 3% Cystic fibrosis 4% Severe persistent asthma 3% RA 2% Lung cyst 1.5% Ciliary dysfunction 2% Ciliary dyskinesia < miscellaneous causes Respirology 2002 7, 63–66 Am J Respir Crit Care Med 2000 162, 1277-84

Acute exacerbation of bronchectasis : 

Acute exacerbation of bronchectasis

Diagnosis : 

Diagnosis Clinical history and physical examination Radiologic imaging : Dx and DDX Further investigation :

Slide 29: 

Radio Clin N Am 47 2009 289-306

Clinical clues : 

Clinical clues Focal bronchiectasis Airway obstruction Recurrent infection in one lobe Hx of FB aspiration MAC Old female, middle lobe atelectasis

Clinical clues : 

Clinical clues Diffuse bronchiectasis ABPA Steroid-dependent asthma Central bronchiectasis Migratory pulm. Infiltration Eosinophilia PCD Kartagener’s syndrome ( Sinusitis, Bronchiectasis, Situs inversus )

Clinical clues : 

Clinical clues Diffuse bronchiectasis Cystic fibrosis Upper lobe, cirrhosis Pancreatic insufficiency Immunodeficiency Recurrent sinopulmonary infection, no site specific Young’s syndrome Infertility from azoospermia Alpha-1 antitripsin Severe diffuse cystic deficinency bronchiectasis, emphysema

Clinical clues : 

Clinical clues Diffuse bronchiectasis Yellow nail syndrome Yellow nail, chylothorax lymphedema Diffuse panbronchiolitis Post nasal drip, small nodule and hypoxemia

Investigation : 

Investigation Focal bronchiectasis Airway obstruction CXR, Bronchoscopy MAC Sputum AFB, Mycobacterial culture

Investigation : 

Investigation Diffuse bronchiectasis ABPA Ig E level, Aspergillus preciptin Sputum fung stain/culture PCD CXR, EKG, Film PNS

Investigation : 

Investigation Diffuse bronchiectasis Cystic fibrosis Sweat Cl- test, CFTR gene mutation Immunodeficiency Total Ig G, M, A Young’s syndrome Sperm count Alpha-1 antitripsin Alph-1 antitripsin level deficinency

Investigation : 

Investigation Diffuse bronchiectasis Yellow nail syndrome Pleural fluid TG/Chol lymphedema Diffuse panbronchiolitis HRCT(bizzare nodules) transbronchial biopsy

Pathophysiology : 

Pathophysiology infection Retained secretion and inflammation Airway destruction and remodeling Antibiotic Bronchial hygiene Rx Pulmonary rehabilitation

Management : 

Management 1. Acute exacerbation 2. Specific disease 3. Stable disease 4. Life threatening condition e.g. massive hemoptysis

Management of specific disease : 

Management of specific disease Focal bronchiectasis Airway obstruction Sx,laser,bronchoscopic removal MAC Clarithro+RIF+ETB

Management of specific disease : 

Management of specific disease Diffuse bronchiectasis ABPA oral CS CF Dnase, Tobra, gene RX Immunodef Immunoglobulin Alpha-1antrip def Alpha-1 antritrip replacement Diffuse panbronchiolitis Macrolide

Management : 

Management Nonpharmacological treatment Pharmacological treatment - Antibiotic - Mucolytic - Anti-inflammatory/immunomodulator - Bronchodilator

Slide 44: 

BMJ 2007 335 1089-93

Antibiotic : 

Antibiotic Used to treat acute exacerbation, to prevent infection or to reduce bacterial burden 3 strategies 1) high oral dose for prolonged period (at least 4 weeks)(macrolide and FQ) Zithromax500mg 2/wk x 6Mn (reduced exacerbation) 2) Aerosolized antibiotic (e.g during alternate month) (Gentamicin40mgbidx3days,Tobramycin 300mgbidx4wk,Fortum1g+Tobramycin100mgbidx12mn) 3) Regular pulsed course of iv antibiotic (e.g.2-3 wk courses with 1 or 2 months in between)

Slide 46: 

Fishman’s Pulmonary diseases and disorders,4th edition

Pneumococcal vaccination : 

Pneumococcal vaccination

Influenza vaccine : 

Influenza vaccine Lacking of RCT in patient with bronchiectasis (1-, C)

Mucolytic : 

Mucolytic To improve tracheobronchial clearance Oral Bromhexine (30mg tid) (1+,B) Improved expectoration,quantitiy/quality of sputum and auscultation finding 2) Nebulized rh DNase I (2.5mgod/bid x 2wk) More frequent exacerbation and decline in FEV1 in non-CF bronchiectasis (1+, A* for CF) 3) Inhaled Manitol (300-400mg od) improved tenacity and hydration of sputum,but large RCT are needed. (2++,B)

Slide 50: 

BMJ 2007 335 1089-93

Anti-inflammatory agents : 

Anti-inflammatory agents ICS Inhaled Fluticasone 500ug bid decreased density of leucocyte, concentration of -inflammatory mediator 1B, IL-8 and LT-B4 in sputum -improved sputum volume but no change frequent of exacerbation, lung function and purulence sputum. (1+, B)

Anti-inflammatory agents : 

Anti-inflammatory agents Systemic corticosteroid May better at penetrating the bronchial wall and therefore be more effective but available data only for CF-Bronchiectasis (2+, D) LTA inhibit neutrophil mediated inflammation But no RCT ( 4, D) NSAIDS inhibit neutrophil function and release of elastase (Indocid25mgtidx4wk but opened label study, lung function, sputum vol/quality did not change but high dose Ibuprofen x 4 yr found significantly slowed progression of lung disease in CF-Bronchiectasis. (1+ ,B)

Anti-inflammatory agents : 

Anti-inflammatory agents Macrolides suppress inflammation, improved clinial status and lung function in a few small studies. RCT in children showed that 3 months of Clarithromycin decreased total No. of leucocyte, proportion of PMN and concentration of IL-8 in BAL Azithromycin 500mg twice a week x 6mn Decrease daily sputum production and exacerbation (1+, B)

Bronchodilator : 

Bronchodilator Cochrane reviews found no RCT on SABA, LABA, Anticholinergic or oral Methylxanthine in patient with non-CF bronchiectasis ( 4, D)

Bronchopulmonary hygiene physcial therapy : 

Bronchopulmonary hygiene physcial therapy Chest physical Rx that aims to remove lung secretions in pt with acute and chronic airway disease. Many active and passive technique are available Evidence is variable and the literature is conflicting. Two systemic reviews found insufficient evidence to support or refute this form of Rx ( 3, D)

Exercise training : 

Exercise training Only one RCT 8 wk duration of…….. Pulmonary rehabilitation alone vs Pulmonary rehab+inspiratory muscle training vs placebo Conclusion : Pulmonary rehabilitation improve exercise tolerance but simultaneous inspiratory muscle training offered no additional benefit. (1+, B)

Surgery : 

Surgery Cochrane reviews found no RCT, just case series and case-controlled study. Overall MR 3.5%, 71% had no symptoms during follow-up. Surgery is a possibility if the area of bronchiectasis is localized and symptoms are debilitating or life threatening.

Summary points : 

Summary points Bronchiectasis refers to abnormal bronchial dilatation caused by vicious cycle of transmural infection and inflammation Symptom include chronic productive cough, wheeze, and dyspnea; repeat respiratory infection may dominate the clinical picture Dx is based on daily production of mucopurulent phlegm and thickned airway on CT.

Summary point : 

Summary point Dx should lead to investigation and treatment of possible causes and associated condition Acute exacerbation should be treated promptly with short course antibiotic Frequent exacerbation may be treated with prolonged and aerosolized antibiotic The role of mucolytic, anti-inflammatory agent and bronchidilator is not clear Surgery is a possibility if localized bronchiectasis and symptoms are debilitating or life threatening.

Slide 60: 

Thank you………..