Bronchiectasis

Dr. Shahid PervaizDr. Shahid Pervaiz

Postgraduate Trainee Postgraduate Trainee

Pulmonology Department Pulmonology Department

Nishtar Hospital MultanNishtar Hospital Multan

BRONCHIECTASIS

• DefinitionDefinition

• EtiologyEtiology

• PathologyPathology

• Clinical presentationClinical presentation

• Diagnosis & differential diagnosisDiagnosis & differential diagnosis

• Treatment Treatment

• Bronchiectasis is an abnormal Bronchiectasis is an abnormal and permanent dilatation of and permanent dilatation of bronchibronchi

• It may be either focal or diffuseIt may be either focal or diffuse

• It is a disorder that typically It is a disorder that typically affects older individualsaffects older individuals

• Approximately 2/3 of patients Approximately 2/3 of patients are womenare women

Definition

Etiology

Conditions associated with the Conditions associated with the development of bronchiectasisdevelopment of bronchiectasis

1. Postinfection1. Postinfection

• Bacterial pneumoniaBacterial pneumonia

• TuberculosisTuberculosis

• PertussisPertussis

• MeaslesMeasles

• InfluenzaInfluenza

Etiology

2. Proximal airway obstruction2. Proximal airway obstruction

• Foreign body aspirationForeign body aspiration

• Benign airway tumorsBenign airway tumors

Etiology

2. Proximal airway obstruction2. Proximal airway obstruction

• Middle lobe syndromeMiddle lobe syndrome Extrinsic compression by enlarged Extrinsic compression by enlarged

lymph nodes of the right middle lymph nodes of the right middle lobe of the lung that obstructed lobe of the lung that obstructed bronchi and lead to right middle bronchi and lead to right middle lobe atelectasis and recurrent lobe atelectasis and recurrent infection.infection.

Etiology

3.Abnormal host defense3.Abnormal host defense

• Ciliary dyskinesia ( Kartagener’s Ciliary dyskinesia ( Kartagener’s syndrome)syndrome)

• Humoral immunodeficiencyHumoral immunodeficiency

4.Genetic disorders4.Genetic disorders

• Cystic fibrosisCystic fibrosis

• α1- Antitrypsin deficiencyα1- Antitrypsin deficiency

5.Others5.Others

Bronchiectasis Pathogenesis

Airway Injury + Secretion Stimuli

Secretion Stasis Infection

Airway Destruction + Airway Dilation

Pathology

• Dilation and distortion of the Dilation and distortion of the bronchibronchi

• Damage of airway epitheliumDamage of airway epithelium

• Dilation and hyperplasia of Dilation and hyperplasia of blood capillaryblood capillary

•Bronchial dilatation is associated Bronchial dilatation is associated with with destructivedestructive and and inflammatoryinflammatory changes in the walls changes in the walls of of medium-sized airwaysmedium-sized airways, often at , often at the level of segmental or sub-the level of segmental or sub-segmental bronchisegmental bronchi

•Airway inflammation is Airway inflammation is primarily primarily mediated by neutrophils mediated by neutrophils and and results in up-regulation of enzymes results in up-regulation of enzymes such as elastase and matrix such as elastase and matrix metalloproteinasesmetalloproteinases

Pathology

As a result of inflammation, vascularity of the bronchial wall increases, with associated enlargement of the bronchial arteries.

Pathogenesis

Three different patterns of Three different patterns of bronchiectasis have been bronchiectasis have been describeddescribed

cylindrical bronchiectasiscylindrical bronchiectasis: the : the involved bronchi appear uniformly involved bronchi appear uniformly dilated dilated

varicose bronchiectasisvaricose bronchiectasis: the : the affected bronchi have an irregular affected bronchi have an irregular or beaded pattern of dilatation or beaded pattern of dilatation resembling varicose veinsresembling varicose veins

Patterns Of Bronchiectasis

• Saccular (cystic) Saccular (cystic) bronchiectasisbronchiectasis: The : The bronchi have a ballooned bronchi have a ballooned appearance at the appearance at the periphery, ending in blind periphery, ending in blind sacs without recognizable sacs without recognizable bronchial structures bronchial structures distal to the sacsdistal to the sacs

Morphological types

• Cylindrical or tubular bronchiectasisCylindrical or tubular bronchiectasis

• VaricoseVaricose

• saccular or cystic bronchiectasissaccular or cystic bronchiectasis

AdenovirusAdenovirus and and Influenza Influenza virus virus are the main viruses that are the main viruses that cause bronchiectasis in cause bronchiectasis in association with lower association with lower respiratory tract involvementrespiratory tract involvement

Virulent bacterial infections, Virulent bacterial infections, especially with potentially especially with potentially necrotizing organisms such as necrotizing organisms such as Staphylococcus aureusStaphylococcus aureus, , KlebsiellaKlebsiella, and , and AnaerobesAnaerobes

Infectious Causes

Infection with Infection with Bordetella Bordetella pertussispertussis, particularly in childhood, particularly in childhood

Bronchiectasis has been reported in Bronchiectasis has been reported in patients with patients with HIV infectionHIV infection

TuberculosisTuberculosisNontuberculous mycobacteria Nontuberculous mycobacteria

can serve as primary pathogens & can serve as primary pathogens & secondary infections or colonizing secondary infections or colonizing organismsorganisms

Infectious Causes

Impaired host defense Impaired host defense mechanisms are often mechanisms are often involved in the predisposition involved in the predisposition to recurrent infectionsto recurrent infections

The major cause of The major cause of localized localized impairment impairment of host defenses is of host defenses is endobronchial obstructionendobronchial obstruction

Slowly growing endobronchial Slowly growing endobronchial neoplasms such as carcinoid neoplasms such as carcinoid tumors may be associated with tumors may be associated with bronchiectasisbronchiectasis

Foreign-body aspiration is Foreign-body aspiration is another important cause of another important cause of endo-bronchial obstruction, endo-bronchial obstruction, particularly in childrenparticularly in children

Airway obstruction can also Airway obstruction can also result from result from bronchostenosis, from bronchostenosis, from impacted secretions or impacted secretions or extrinsic compression by extrinsic compression by enlarged lymph nodesenlarged lymph nodes

Generalized impairment Generalized impairment of of pulmonary defense mechanisms pulmonary defense mechanisms occurs with: occurs with:

immunoglobulin deficiencyimmunoglobulin deficiency primary ciliary disordersprimary ciliary disorderscystic fibrosis (CF)cystic fibrosis (CF)With panhypogammaglobulinemia With panhypogammaglobulinemia patients often also have a history of patients often also have a history of sinus or skin infectionssinus or skin infections

Selective deficiency of an IgG Selective deficiency of an IgG subclass, especially IgG2, has also subclass, especially IgG2, has also been described in a small number of been described in a small number of patients with bronchiectasis.patients with bronchiectasis.

•Are responsible for 5–10% of cases of Are responsible for 5–10% of cases of bronchiectasisbronchiectasis

• Is inherited in an Is inherited in an autosomal recessive autosomal recessive fashionfashion

•The cilia become dyskinetic; their The cilia become dyskinetic; their coordinated, propulsive action is coordinated, propulsive action is diminished, and bacterial clearance is diminished, and bacterial clearance is impairedimpaired

•Clinical effects include recurrent upper Clinical effects include recurrent upper and lower respiratory tract infections, and lower respiratory tract infections, such as sinusitis, otitis media, and such as sinusitis, otitis media, and bronchiectasisbronchiectasis

Primary Ciliary Dyskinesia

males are generally males are generally infertileinfertile

approximately half of approximately half of patients fall into the patients fall into the subgroup of subgroup of Kartagener's syndromeKartagener's syndrome

1)1)situs inversus situs inversus

2)2)bronchiectasis bronchiectasis

3)3)sinusitisinusitiss

Some cases of bronchiectasis are Some cases of bronchiectasis are associated with exposure to a toxic associated with exposure to a toxic substance substance

Inhalation of a toxic gas such as Inhalation of a toxic gas such as ammoniaammonia or or aspiration of acidic gastric aspiration of acidic gastric contentscontents

An immune response in the airway may An immune response in the airway may also trigger inflammation, destructive also trigger inflammation, destructive changes, and bronchial dilatation changes, and bronchial dilatation ABPAABPA

Noninfectious Causes

ΑΑ11-antitrypsin deficiency -antitrypsin deficiency »» panacinar emphysema & panacinar emphysema & bronchiectasisbronchiectasis

Yellow nail syndromeYellow nail syndrome which is due to which is due to hypoplastic lymphatics, is accompanied hypoplastic lymphatics, is accompanied by bronchiectasis in approximately by bronchiectasis in approximately 40% of patients:40% of patients:

a)a)LymphedemaLymphedema

b)b)Pleural effusionPleural effusion

c)c)Yellow discoloration of the nailsYellow discoloration of the nails

Noninfectious Causes

Clinical presentation1.1. The production of large quantities of The production of large quantities of

purulent and often foul-smelling purulent and often foul-smelling sputum.sputum.

The volume of sputum can be used for The volume of sputum can be used for estimating the severity of the diseaseestimating the severity of the disease

• Mild < 10 mLMild < 10 mL• Moderate 10~150 mLModerate 10~150 mL• Severe >150 mLSevere >150 mL

※ ※ Dry bronchiectasisDry bronchiectasis usually involve the upper lobes usually involve the upper lobes

Clinical presentation2. Chronic cough2. Chronic cough3. Hemoptysis:3. Hemoptysis:• FrequentFrequent• More commonly in dry varietyMore commonly in dry variety• Usually mild (blood streaking of purulent Usually mild (blood streaking of purulent

sputum)sputum)• Massive hemoptysis is usually from Massive hemoptysis is usually from

dilated bronchial arteries or bronchial-dilated bronchial arteries or bronchial-pulmonary anastomoses under systemic pulmonary anastomoses under systemic pressurepressure

Clinical presentation

4. Recurrent pneumonia:4. Recurrent pneumonia: same segmentsame segment

5. Systemic manifestations:5. Systemic manifestations: fever, weight lossfever, weight loss

Clinical presentationPhysical findingPhysical finding

• Early phases or dry variety: Early phases or dry variety: normalnormal

• Severe or secondary infection: Severe or secondary infection: persisting crackling rales in the persisting crackling rales in the same part of lungsame part of lung

• Later stage: digital clubbing, Later stage: digital clubbing, emphysema, and cor pulmonale.emphysema, and cor pulmonale.

Patients typically present with Patients typically present with persistent or recurrent cough and persistent or recurrent cough and purulent sputum productionpurulent sputum production

Hemoptysis occurs in 50–70% of Hemoptysis occurs in 50–70% of cases cases

More significant, even massive More significant, even massive bleeding is often a consequence of bleeding is often a consequence of bleeding from bleeding from hypertrophied hypertrophied bronchial arteriesbronchial arteries

Systemic symptoms such as fatigue, Systemic symptoms such as fatigue, weight loss, and myalgias can also weight loss, and myalgias can also occuroccur

Clinical Manifestations

some cases are either asymptomatic some cases are either asymptomatic or have a nonproductive cough or have a nonproductive cough »»»»»»»»»»»»»»»»»»»»»» dry bronchiectasis in an dry bronchiectasis in an upper lobeupper lobe

Dyspnea or wheezing generally Dyspnea or wheezing generally reflects either reflects either widespread widespread bronchiectasis bronchiectasis or or underlying COPDunderlying COPD

With exacerbations of infection, the With exacerbations of infection, the amount of sputum increasesamount of sputum increases, , becomes becomes more purulent more purulent and often and often more bloodymore bloody; systemic symptoms, ; systemic symptoms, including including feverfever, may also be , may also be prominentprominent

Physical examinationPhysical examination : :Any combination of Any combination of crackles, rhonchi, and crackles, rhonchi, and wheezes may be heardwheezes may be heard

Clubbing may be presentClubbing may be presentCor pulmonale and right Cor pulmonale and right ventricular failureventricular failure

•Chest xrayChest xray•The findings of chest radiograph are The findings of chest radiograph are

often nonspecificoften nonspecific•Radiograph may be normal with mild Radiograph may be normal with mild

diseasedisease•Cyctic dilated areas may be visible in Cyctic dilated areas may be visible in

advanced casesadvanced cases•Ring shadows or honeycoming Ring shadows or honeycoming •There will be associated features of There will be associated features of

volume lossvolume loss

In one or more segments/lobesIn one or more segments/lobes

Radiographic Findings

MILD BRONCHIECTASISNormal chest radiographpresents with hemoptysis

MODERATE BRONCHIECTASIS- Coarse white linesextending out from hila

TOO MANY WHITE LINES

SEVERE BRONCHIECTASIS

Circle filledwith air

SEVERE BRONCHIECTASIS

RINGS (CYSTS) CONTAINING AIR-FLUID LEVELS

Evaluation

• CT or HRCT:CT or HRCT: high sensitivity high sensitivity and specificityand specificity

Train track sign:Train track sign: the bronchial the bronchial wall is thicken and visible; the wall is thicken and visible; the bronchi lose the trend of bronchi lose the trend of narrowing from proximal end to narrowing from proximal end to distal end.distal end.

•HRCT HRCT is now the standard is now the standard technique for testing or technique for testing or confirming the diagnosis of confirming the diagnosis of bronchiectasis saccular bronchiectasis saccular bronchiectasis bronchiectasis may have may have prominent cystic spaces,with or prominent cystic spaces,with or without air-liquid levels, without air-liquid levels, corresponding to the dilated corresponding to the dilated airwaysairways

When seen longitudinally, the When seen longitudinally, the airways appear as airways appear as "tram tracks“"tram tracks“

when seen in cross-section, they when seen in cross-section, they produce produce "ring shadows" "ring shadows"

•Because the dilated Because the dilated airways may be filled with airways may be filled with secretions, the lumen may secretions, the lumen may appear dense rather than appear dense rather than radiolucent, producing an radiolucent, producing an opaque tubular or opaque tubular or branched tubular structure.branched tubular structure.

•HRCT images 1.0–1.5 mm HRCT images 1.0–1.5 mm thick, provides an excellent thick, provides an excellent view of dilated airways view of dilated airways

Radiology of Bronchiectasis

HRCT of Bronchiectasis

Dilated bronchus

BRONCHIECTASIS

String of beads

BRONCHIECTASIS

Bronchi visiblewithin 1 cm of the pleura

Destroyedlung(Scarring)

BRONCHIECTASIS

Cystic Bronchiectasis

Cystis / saccular bronchiectasis

4. Diagnosis – Chest CT

Varicose bronchiectasis

4. Diagnosis – Chest CT

Cystis / saccular bronchiectasis

4. Diagnosis – Chest CT

Examination of sputumExamination of sputumWhen bronchiectasis is focal, FOB may When bronchiectasis is focal, FOB may reveal an underlying endobronchial reveal an underlying endobronchial obstructionobstruction

Upper lobe involvement may be suggestive Upper lobe involvement may be suggestive of either of either TBTB or or ABPAABPA

Measurement of Measurement of sweat chloride levels sweat chloride levels for CFfor CF

Structural or functional assessment of Structural or functional assessment of nasal or bronchial cilianasal or bronchial cilia or or spermsperm for for primary ciliary dyskinesiaprimary ciliary dyskinesia

Quantitative assessment of Quantitative assessment of immunoglobulins immunoglobulins may explain recurrent may explain recurrent airway infectionairway infection

Laboratory Findings

•PFTPFT tests may demonstrate tests may demonstrate airflow obstruction airflow obstruction

•Bronchial hyperreactivity Bronchial hyperreactivity to methacholine challenge, to methacholine challenge, and some reversibility of the and some reversibility of the airflow obstruction with airflow obstruction with inhaled bronchodilators are inhaled bronchodilators are relatively commonrelatively common

Evaluation

2.Bronchoscopy2.Bronchoscopy

• Evaluating the proximal airways for lesions.Evaluating the proximal airways for lesions.

• Assessing the cause of hemoptysisAssessing the cause of hemoptysis

• Localizing the source of hemoptysisLocalizing the source of hemoptysis

Diagnosis

• SymptomsSymptoms

• SignSign

• Radiological findingRadiological finding

Differential diagnosis

Differentiate from:Differentiate from:

• Chronic bronchitisChronic bronchitis

No recurrent hemoptysis; CT No recurrent hemoptysis; CT scan scan

• Lung abscessLung abscess

X-ray/CT: local infiltrated X-ray/CT: local infiltrated shadow or cavitations with air-shadow or cavitations with air-fluid level inside.fluid level inside.

Differential diagnosis

• TuberculosisTuberculosis

radiographic finding; sputum radiographic finding; sputum anti-fast smearanti-fast smear

• Congenital pulmonary cystCongenital pulmonary cyst

multi thin wall cavities without multi thin wall cavities without infiltration around.infiltration around.

Treatment

• Medical managementMedical management

1. Improving the drainage of airway1. Improving the drainage of airway

1) expectorant1) expectorant

2) bronchodilators2) bronchodilators

3) postural drainage3) postural drainage

•Therapy has several major Therapy has several major goals: goals:

(1)(1) Treatment of infection, Treatment of infection, particularly during acute particularly during acute exacerbations exacerbations

(2)(2) Improved clearance of Improved clearance of tracheobronchial secretionstracheobronchial secretions

(3)(3) Reduction of inflammation Reduction of inflammation

(4)(4) Treatment of an identifiable Treatment of an identifiable underlying problemunderlying problem

Treatment

Treatment• Medical managementMedical management

2. Antibiotic2. Antibiotic

• The choice of antibiotics should The choice of antibiotics should be accurately by the results of be accurately by the results of sputum culture and drug sputum culture and drug sensitivity test.sensitivity test.

• Empirical therapy ---Empirical therapy ---antipseudomonal antibiotics. antipseudomonal antibiotics.

Antibiotics are the Antibiotics are the cornerstone of cornerstone of bronchiectasis managementbronchiectasis management

•Antibiotics are used only during Antibiotics are used only during acute episodesacute episodes

•Choice of an antibiotic should Choice of an antibiotic should be guided by gram's stain and be guided by gram's stain and culture of sputumculture of sputum

•Empiric coverage (amoxicillin, Empiric coverage (amoxicillin, co-trimoxazole,levofloxacin) is co-trimoxazole,levofloxacin) is often given initially often given initially

•Infection with Infection with P. AeruginosaP. Aeruginosa is of is of particular concern, as it appears to particular concern, as it appears to be associated with greater rate of be associated with greater rate of deterioration of lung function and deterioration of lung function and worse quality of life.Fluroquinolons worse quality of life.Fluroquinolons are only oral antibiotic against are only oral antibiotic against p.Aeruoginosa.p.Aeruoginosa.

•There are no firm guidelines for There are no firm guidelines for length of therapy, but a length of therapy, but a 10–14 day 10–14 day course course or longer is typically or longer is typically administeredadministered

•2g tetracylin (twice/weak) for 12 2g tetracylin (twice/weak) for 12 months reduce no. of exacerbationmonths reduce no. of exacerbation

Azithromycin 500mg/twice weak Azithromycin 500mg/twice weak for 8 wks reduce the volume of for 8 wks reduce the volume of sputum and improve pulmonary sputum and improve pulmonary functionfunction

•Tobramycin 300mg by aerosal Tobramycin 300mg by aerosal twice/daily for 4 wks will reduce twice/daily for 4 wks will reduce pseudomonas sputum density pseudomonas sputum density by 10000 colony forming by 10000 colony forming units/per gram of sputum units/per gram of sputum

•Facilitate drainage : Facilitate drainage : mechanical methods mechanical methods & & appropriate appropriate positioningpositioning

• Mucolytic agents to thin Mucolytic agents to thin secretions and allow better secretions and allow better clearance are controversialclearance are controversial

•Aerosolized recombinant DNase, Aerosolized recombinant DNase, which decreases viscosity of which decreases viscosity of sputum by breaking down DNA sputum by breaking down DNA released from neutrophils, has released from neutrophils, has been shown to improve been shown to improve pulmonary function in CF but pulmonary function in CF but may be deleterious and should may be deleterious and should be avoided in bronchiectasis not be avoided in bronchiectasis not associated with CFassociated with CF

•Bronchodilators to improve Bronchodilators to improve obstruction and aid clearance of obstruction and aid clearance of secretions are useful in patients secretions are useful in patients with with airway hyperreactivity airway hyperreactivity and and reversible airflow reversible airflow obstructionobstruction

Surgical therapy Surgical therapy when bronchiectasis is when bronchiectasis is localized and the morbidity localized and the morbidity is substantial despite is substantial despite adequate medical therapy.adequate medical therapy.

Treatment

• Surgical therapySurgical therapy

1.1. Recurrent and refractory Recurrent and refractory clinical symptoms are due to a clinical symptoms are due to a focal area of disease focal area of disease involvement.involvement.

2.2. Massive hemoptysisMassive hemoptysis

• Management of hemoptysisManagement of hemoptysis

In case of massive hemoptysisIn case of massive hemoptysis•Conservative therapy, including Conservative therapy, including

rest and antibioticsrest and antibiotics•Surgical resection Surgical resection •Bronchial arterial Bronchial arterial embolization embolization •Although resection may be Although resection may be successful if disease is successful if disease is localized, embolization is localized, embolization is preferable with widespread preferable with widespread diseasedisease

locallocal• Recurrent pneumoniaRecurrent pneumonia

• Lung abcessLung abcess

• EmpyemaEmpyema

• HemoptysisHemoptysis

• Pulmonary hypertensionPulmonary hypertension

Complication

SystemicSystemic• Hypoproteinemia &amyloidosisHypoproteinemia &amyloidosis

• Generalized edema (100gm Generalized edema (100gm sputum/4_5 g protein)protein sputum/4_5 g protein)protein loosing pneumopathyloosing pneumopathy

Complication