Bronchiectasis

Thanun thanwiset

Outline
Introduc4on Epidemiology Type Clinical Pathophysiology Natural history Diagnosis management

Bronchiectasis dene by dilata4on or ectasia of the airways or bronchus Share clinical feature with COPD : inamed and easily collapsible airways, obstruc4ve out ow, recurrent or refractory infec4on , hemoptysis

Epidemiology
No systema4c data on the incidence or prevalence for bronchiectasis Decline rate of Brochiectasis aFer 20th century due to vaccine and ATB development Es4mate 110,000 individual in USA

 Prevalence increase by age USA 4.2/100,000 18-34 yr. 272/100,000 72 yr. More common in women USA : increase number of brochiectasis cases associtated with TB or NTM Auckland NZL 1/6,000 admit 16.4/100,000

Type of bronchiectasis
Local lobe or segment
Intrabronchial obstruc4on:
FB , slow growing tumor

Extrabronchial obstruc4on
Lymph node compression

Pos4nfec4on : pneumonia , pulmonary TB

 Diuse
Ciliated epithelium dysfunc4on : primary ciliary dyskinesia Immunodeciency : HIV , congenital and acquired hypogammaglobulinemia Congenital disorder: Cys4c brosis (CF), alpha-1 an4trypsin deciency, tracheobronchomegaly ( Mounier-Kuhn syndrome), car4lage deciency ( William-Cambell syndrome , young s syndrome, Marfan sydrome

 Rheuma4c disease : RA, inamatory bowel disease, SLE, Sjogren s disease Post infec4on : adenovirus, measle, inuenza

Clinical nding
Classic clinical : cough and the daily produc4on of mucopurulent and tenacious sputum las4ng month or year Complaint include: dyspnea, wheezing, pleuri4c pain, hemoptysis

Clinical nding
Physical nding
Crackle , wheezing, were common Digital clubbing only 2 percent

Urinary incon4nence in the pa4ents with bronchiectasis 47% (10% in general popula4on)

bronchiectasis e4ology Role of infec4on Predominant organism in sputum Airow obstruc4on and hyperresponsiveness Finding of Chest immage Quality of sputum Infec4on, genene4c ,immune defect primary H. inuenza, Pseudomonas aerugenosa prensent Airway dila4on ad thickening , mucoid plug Purulent,

COPD Cigarehed smoking Secondary Strep. Pneumoniae H. inuenza Present Hyperlucency,hyperina4on , airway dilata4on Mucoid, clear

Pathogenesis
Various mechanism to produce permanent, pathologic dilata4on and damage of the airways. Trac4on brochiectasis Pulsion bronchiectasis Weakness of the airwary

Pathogenesis
Trac4on brochiectasis Distending force of the nega4ve intrapleural pressure are transmihed to the airways. Fibro4c lung change such as sarcoidosis, inters44al lung disorder, infec4on such as TB, Local trac4le result xed dilata4on.

Pathogenesis
pulsion brochiectasis Allergic bronchopulmonary aspergillosis (ABPA) is prototype Reac4on to inhaled Aspergillus. Fungi , mucoid, iname mediator obstruct airway Pressure distend the central air way

 Weakness of the airways

Classic pos4nfec4on : chronic iname lead bronchial wall loss of structural integrety Primary weakness : Mounier-Kuhn syndrome (congenital tracheobronchiomegaly) William cambell syndrome ( loss of car4lage ring in brochi) , relapsing polychondri4s

Vicious cycle and microbiology

infec4on

Defect of host defence

Impaired drainage

Airway obstruc4on

 Impair secre4on drainge obstruc4on, impair mucociliary, Collec4on of sputum and bacteria increase infec4on rate Airways obstruc4on, dilate bronchus , thickening bronchial wall Increase mucus secre4on

Microbiology
Nonenteric gram-nega4ve bacteria commonly infect 1/3 pa4ent are chronically colonized with Pseudoaeruginosa Staph. Aureus raises the suspicious for Cys4c brosis

E4ology
cause of bronchiectasis may be dicult. Even with exhaus4ve clinical, laboratory, and pathologic tes4ng, up to 50 to 80% of cases of bronchiectasis may s4ll be idiopathic

Lung injury due to infec4on

In Thailand TB is the cause of 1/3 bronchiectasis childhood respiratory tract infec4ons may contribute to permanent airway damage The presence of Staphylococcus aureus is associated with cys4c brosis

 Lady Windermere syndrome: Primary Mycobacterium avium complex infec4on has been recognized in white women over 60 years of age. Chronic, unrelen4ng cough and middle-lobe involvement. AIDS : repeated respiratory tract infec4ons and impaired host response. Previous pyogenic, pneumocys4c, and mycobacterial infec4ons

Middle lobe bronchiectasis in primary MAC infection

 AIDS : repeated respiratory tract infec4ons and impaired host response. Previous pyogenic, pneumocys4c, and mycobacterial infec4ons

Primary ciliary dyskinesia

Poorly func4on of cilia lead to retainng secre4on and recurrent infec4on causing Bronchiectasis Incidence 1:15,000-40,000 Half of pa4ent have Kartagener s Syndrome: (bronchiectasis, sinusi4s, situs inversus or par4al laterlizing abnormality)

 Orther abnormality: o44s media, GEReux, Infer4le Inves4gate : chromosome iden4cal 5p15- p14,DNA H5

Airway do not taper periphery in Katagener.

Cy4c brosis
Abnormal gene:Cys4c Fibrosis Transmembrane conductane regulator(CFTR) decrease Cl- secre4on, increase Na reabsorp. Mucus plug airways obstruc4on. onset in adulthood of repeated respiratory tract infec4ons in the absence of exocrine pancrea4c insuciency, sinunsi4s is recognized as a presenta4on of cys4c brosis

 Upper-lobe inltra4on apparent on imaging of the chest and the growth of S. aureus or mucoid Pseudomonas aeruginosa on sputum

Incidence 1:2,000-3,000 west Thai report 1 case Elevated chloride concentra4ons(>55-60 mmol/L) on sweat chloride tes4ng are diagnos4c HRCT-bronchiectasis predominent upper lobe

Disorder of immunity
Deciencies of IgG, IgM, and IgA are risk for recurrent suppura4ve sinopulmonary infec4ons and bronchiectasis Acquire hypogammaglobulinemia is the most frequent syndrome recognized in this group of disease Hx childhood with repeated sinopulmonary infec4on

 Recurrent lung infec4on with encapsulated capsule S.pneumonia, H.inuenzae. S.aureus, P.seudoaerugenosa The diagnos4c : low level of gamma globulin, failure response to produce an4body following vaccine. Reple4on with gamma globulin can prevent further respiratory tract infec4on and lung damage

Alpha1-an4trypsin anomaly
A review of the clinical manifesta4ons and chest CT scans of pa4ents with alpha-1 an4trypsin deciency found that radiographic abnormali4es sugges4ve of bronchiectasis (95 percent) ATS/ERS recommend alpha-1 tes4ng in BXSIS pt. and no other e4ology HRCT-severe diuse cys4c bronchiectasis, emphysema

Bronchial car4lage
Mounier-Kuhn syndrome ( congenital tracheobronchomegaly Rare disorder associate :dilate of C ring of trachea bronchi segment Underlying defect is absence elas4c ber and smooth m. of large airway.

 Recurent lower respira4on infec4on in early year and 4 cascade Diagnosis : extraordinary dilata4on of the trachea and central bronchi on CT. Management: consider PEEP support and silicone or metallic stent Lung transplant is an op4on

Connec4ve 4ssue disease

Found bronchiectasis in RA, Sjogren syndrome, SLE Found bronchiectasis in 1-3 %in RA Arthropathy and sicca are usually advanced when bronchiectasis becomes apparent.

 HRCT may help diag up to 30% Five yr. Follow up : pt. With bronchiectasis and RA were die 5 4mes with RA alone

Allergic bronchopulmonary aspergillosis

Develop mucoid plugs in the medium sized bronchi. Hyperimmune reac4on to the aspegillus organism . Character by an exaggerated T helper cell response. Immunologic featured; blood eosinophilia, very high plasma IgE level ,precipitate by specifec an4body to Aspergillus

Allergic bronchopulmonary aspergillosis

Non specifec chest Symptom: produc4ve cough,mucopurulent, fever, Suspected in pa4ents with
long Hx of ashma ,resist to bronchodilator and steroid treatment Inamma4on and disten4on typically result in thin wall BXSIS of the central and mid-lung airway CT chest: central airway BXSIS which is unusual in other disorder.

 Cigarehe smoking
Causal Role of cigarehe smoking in bronchiectasis is not conclusive Smoking may worsen PFT, and progress of disease

Young syndrome
Syndrome exhibit. : Bronchi4s , sinusi4s ,pancrea4c insuciency like CF, obstruc4ve azoospermia. Normal sweat chloride levels.

Natural history
Clinical course is variable Frequent 1.5 exacerba4ons per yr. US., EU, Ireland Decline FEV1 50ml/yr Accelera4on decline Lung func4on associate with Pseudomonas aeruginosa

 Mortality highest with: chronic hypoxemia,hypercapnea, radiologic extent disease

Diagnosis
Inves4ga4on Aim Diagnose bronchiectasis Evaluate for poten4al underlying cause Rx treatable cause

Clinical clue for cause of BXSIS

cause Focal bronchiectasis Airway obstruc4on Diuse bronchiectasis ABPA Cys4c brosis Immunodeciency state Primary ciliary dyskenisia Youngs syndrome Alpha-1 an4trypsin deciency Steroid dependent asthma,Central bronchiectasis, migratory inltrate,eosinophilia Upper lobe,pancrea4c insu. Cirrhosis Recurrent sinopulmonary infec4on Kartageners syndrome Infer4le from azoospermia Severe diuse cys4c brochiectasis, emphysema Recurrent infect. One lobe,Hx FB asp. Primary M. Avium complex infec4on Old female, middlelobe bronchiectasis Clinical clue and inves5ga5on

Diagnosis
Chest X- ray
Abnormal in most pa4ent( 90%) but not specic picture Finding suggest BXSIS
Tram track Mul4ple ring shadow

 HRCT scan
There are suspicious clinical ndings but a rela4vely normal chest radiograph. The chest radiograph has abnormal ndings and bronchiectasis is strongly suspected.

 Management decisions must be made that depend upon the extent of bronchiectasis The presence (or absence) of confounding diseases needs to be dened, such as chronic obstruc4ve lung disease, inters44al lung disease, or malignancy.

 Finding Airway dilata4on :

Luminal airway diameter 1.0-1.5 4mes of adjacent vessel is normal( >1.5 cylindrical bronchietasis)

Lack of tapering (in combina4on with dilata4on is more specic)

 Bronchial wall thickening : associate with severity Cys4c change

alway aect peripheral region Heavily : grape like appearance, honey comb appearance

Honey comb appearance

 Spirometry
Func4onal assessment of pulmonary impairment Finding
Minimal reduce or normal FVC, low FEV1 are most nding Very low FVC can seen in advance disease

management
1. 2. 3. 4. 5. Airway hygiene and hyperosmo4c agents An4microial therapy An4-inammatory therapy Surgery Cause treatment

Airway hygiene and hyperosmo4c agent

Remove inamatory secre4on for bronchi and paranasal sinus Traheobronchial clearance
Postural drainage and chest physiotherapy Therapeu4c vest

 Mucus-mobilizing method
Inhaled beta-agonist/an4cholinergic bronchodilator Hypertonic saline or manitol inhala4on Dornase alfa : hydrolyze neutrophil DNA benet in CF and may be benet in other condi4on. N-acetyl cysteine

an4bio4c

 Empirical oral ATB: Amoxycillin, Co-amoxiclav, Ciproox, : adjust depend on individual ,sputum Aerosol an4bio4c prevent exacerba4on: Tobramycin in CF (with Pseudo aeruginosa) pa4ent ,non CF are no ecacy

An4-inammatory therapy
Goal is limit progression of decline lung func4on NSAID
Prostaglandin play role in augmen4ng airway secre4on Inhaled indomethacin signicant decreased the amount of sputum ( prospec4ve double blind ) Cochrane review.:High dose ibuprofen slow progressive of CF especially in children

 Inhaled cor4costeroid
Tsang ect. Inhale Flu4casone 1 yr: decrease sputum , no eect to other factor Review clinical trial in CF did not cause improvement

Intermihent macrolide therapy

Aim to prevent exacerba4on Azithromycin 500 mg x2/ wk. 6 months

 Surgery
uncontrolled hemoptysis Remove FB or obstruct tumor Focal bronchiectasis : frequent exacerbate

Lung transplant
Indicate in end stage lung Bilat lung transplant or heart lung transplant

Reference
1. Anne E. O Donnell; Bronchiectasis; Chest 2008;134;815-823 2. Alan F. Barker,M.D. ; Bronchiectasis: NEJM 2002 Vol.346,NO.18 ;1383-93 3. Michael D. Iseman, MD. :Bronchiectasis 2010 Murray&Nadel s Text book of Respiratory medicine 42: 1023-46 4. ; Bronchiectasis 2550 :456-63