bronchiectasis by dr bashir ahmed dar associate professor medicine sopore kashmir

BRONCHIECTASIS BY DR.BASHIR AHMED DAR ASSOCIATE PROFESSOR MEDICINE CHINKIPORA SOPORE KASHMIR EMAIL- [email protected]

BRONCHIECTASIS Bronchiectasis is

defined as the irreversible dilatation of the cartilage-containing airways bronchi or bronchioles.

BRONCHIECTASIS The airways are

dilated up to 4 times the normal size

Bronchi and bronchioles are so dilated they can be followed out to the pleural surfaces•

Respiratory tract The trachea is approximately 22

cm long, with a cross-sectional area of 2 cm.

At the tracheal carina it divides into two major bronchi.

Conductive structures of a size down to 2 mm are termed bronchi. Smaller ones are called bronchioles .

Histology Respiratory tract The epithelium lining the

trachea is ciliated pseudostratified columnar which contains numerous goblet cells. This epithelium has an unusually thick basement membrane, . This epithelium plus its underlying layer of loose connective tissue (the lamina propria) make up the tracheal mucosa.

Histology Respiratory tract The layer under the

mucosa is the submucosa wherein you'll find numerous seromucous glands. The mucosa is separated from the submucosa by a layer of longitudinal elastic fibers.

Histology Respiratory tract Outside the

connective tissue layers, observe the C-shaped rings of hyaline cartilage which help to keep the lumen of the trachea from collapsing.

Histology Respiratory tract Mucous

Membrane.—The mucous membrane is continuous above with that of the larynx, and below with that of the bronchi

Histology Respiratory tract Mucus membrane

consists of areolar and lymphoid tissue, and presents a well-marked basement membrane, supporting a stratified epithelium, the surface layer of which is columnar and ciliated, while the deeper layers are composed of oval or rounded cells.

Histology Respiratory tract Beneath the

basement membrane there is a distinct layer of longitudinal elastic fibers with a small amount of intervening areolar tissue.

Histology Respiratory tract The submucous layer

is composed of a loose mesh-work of connective tissue, containing large blood vessels, nerves, and mucous glands; the ducts of the latter pierce the overlying layers and open on the surface

A. Bronchi The bronchial tree

is aptly named for its resemblance to the branches of a tree, as larger tubes perpetually concede to smaller tube in an intricate framework of branches.

Bronchi The trachea bifurcates into two

primary bronchi, which enter the lung and then branch several times to give rise to smaller secondary and tertiary bronchi. Bronchi differ from the trachea in having plates rather than rings of cartilage, and in having a layer of smooth muscle between the lamina propria and submucosa.

Bronchi The Right Bronchus (bronchus

dexter), wider, shorter, and more vertical in direction than the left, is about 2.5 cm. long, and enters the right lung nearly opposite the fifth thoracic vertebra.

Bronchi Bronchioles are

smaller branches of the bronchi, and are distinguished from them by the absence of cartilage and glands

Bronchi In larger bronchioles,

the epithelium is still ciliated, but is now usually simple columnar, whereas in the smallest bronchioles, the epithelium will be simple cuboidal (mostly containing Clara cells) and lack cilia altogether.

Bronchi The smooth

muscle layer is generally quite prominent in these structures circularly arranged bundles of smooth muscle in the bronchiolar wall.

Bronchi As mentioned above,

the smallest conducting bronchioles consist of a simple cuboidal (or perhaps "low columnar") epithelium of mostly Clara cells, a few ciliated cells, and no goblet cells, and are called terminal bronchioles .

Bronchi In smaller branches, the amount of

cartilage decreases, whereas the amount of smooth muscle increases. Also, the number of glands and goblet cells decreases or absent.

B. Bronchioles

Bronchioles are smaller branches of the bronchi, and are distinguished from them by the absence of cartilage and glands. In larger bronchioles, the epithelium is still ciliated, but is now usually simple columnar, whereas in the smallest bronchioles, the epithelium will be simple cuboidal (mostly Clara cells) and lack cilia altogether.

Bronchioles Nearly 50 distinct types of cells have

been identified in the lungs, of which at least 12 can be found in the airways. Mucus is secreted onto the bronchial surfaces by submucous glands and by "goblet cells" that are present in abundance on the bronchial epithelial surface.

Bronchioles The coordinated

movement of the cilia aid in the transport of mucus covering the airways toward the mouth, where approximately 500 mL of airway fluid is swallowed each day.

Bronchioles It is this "mucociliary

elevator" which is largely responsible for removing foreign material, including organisms that land on the bronchial surfaces.

Bronchioles Injury to these ciliated cells is characteristic

in smokers, who are likely to develop chronic bronchitis, with colonization of the trachea and bronchi by oral organisms and production of excessive amounts of mucus. Patients with congenital abnormalities of ciliary function (Kartagener's syndrome) must raise mucus from the airways by coughing.They are prone to develop respiratory infections and dilation of the bronchi, referred to as bronchiectasis.

Bronchioles Excess bronchoconstriction of

small bronchi between 2 and 5 mm in diameter appears to be responsible for increased airway resistance in most patients with asthma.

Bronchioles The bronchioles contain very little

cartilage, and are controlled via thicker, smoother muscles. The muscle controls whether or not these smaller airways contract or expand. As it refers to air flow resistance, bronchioles provide the most resistance of the air flow passages, similar to the functions associates with the arterioles in the circulatory system.

Mechanisms of development of Bronchiectasis Bronchiectasis may

result from one of three main mechanisms:

A.Bronchial wall injury

Desquamation of the epithelium and necrotising ulceration•

Mechanisms of development of Bronchiectasis Chronic inflammation of bronchial wall: .lymphocytes predominate .Unusually squamous metaplasia More Advanced Stage •Granulation tissue in lamina propria •Cartilage fragmented or destroyed •Muscle erased or focal hyperplastic

changes

Mechanisms of development of Bronchiectasis •Mucous glands persist longer than

other structures. •Bronchial arteries: often ¦greatly

enlarged ¦tortuous •Adjacent lung parenchyma Shows

varying changes from none to advanced organizing pneumonia

Mechanisms of development of Bronchiectasis B.Bronchial lumen

obstruction Focal Congenital

bronchial atresia Foreign body Broncholithiasis Endobronchial

neoplasm Rt mid lobe synd

Mechanisms of development of Bronchiectasis

C.And traction from adjacent fibrosis

Mechanisms of development of Bronchiectasis Many conditions may lead to bronchial

wall injury.These include infections like Recurrent infections Impaired host defense leading to

infection Exaggerated immune response Congenital structural defects of the

bronchial wall And extrinsic insults damaging the airway

wall

Mechanisms of development of Bronchiectasis These conditions share the common

denominator of mucus plugging and superimposed bacterial colonization. The mucus plugging is either a result of abnormal mucus constituency or abnormal mucus clearance. The toxins released by the bacteria and the cytokines and enzymes released by the surrounding inflammatory cells create a vicious cycle of progressive wall damage, mucus plugging, and increased bacterial proliferation.Once bronchiectasis begins, therefore, it is sure to progress.

Mechanisms of development of Bronchiectasis Airway obstruction is most

commonly caused by an intraluminal lesion such as

Carcinoid tumor, Inflammatory myofibroblastic tumor, Or a fibrous stricture usually from

prior granulomatous infection such as histoplasmosis or tuberculosis.

Mechanisms of development of Bronchiectasis When bronchiectasis is from

bronchial wall damage or bronchial obstruction, the bronchial wall becomes thickened because of infiltration by mononuclear cells and fibrosis.

Mechanisms of development of Bronchiectasis Traction bronchiectasis, as its name

implies, is caused by retraction of mature fibrosis of the parenchyma around the bronchi. Such bronchiectasis follows the distribution of the underlying fibrosis. The traction bronchiectasis has an upper lobe distribution in cases of radiation fibrosis, sarcoidosis, and sequela of tuberculosis .

Mechanisms of development of Bronchiectasis In cases of usual interstitial

pneumonitis (UIP) (idiopathic pulmonary fibrosis) and fibrosing nonspecific interstitial pneumonitis (NSIP), the traction bronchiectasis tends to be mostly in the periphery and the lung bases.

Mechanisms of development of Bronchiectasis Congenital defects of the cartilage,

collagen, or other components of the bronchial wall lead to abnormal physiologic clearing of mucoid excretions, predisposing the bronchial epithelium to repeated infections and a vicious cycle of progressive bronchial dilatation.

Mechanisms of development of Bronchiectasis Structural wall defect is the

common feature of Mounier-Kuhn disease or tracheobronchomegaly, William-Campbell syndrome, and congenital bronchial atresia.

Mechanisms of development of Bronchiectasis Tracheobronchomegaly (Mounier-Kuhn

disease) Tracheobronchomegaly is an uncommon

disease that presents mostly in men, in the fourth and fifth decades. Although believed to be congenital, it may be associated with Ehlers-Danlos syndrome, Marfan syndrome, and generalized elastosis (cutis laxa).

Tracheobronchomegaly (Mounier-Kuhn disease) Pathological thinning of the muscle,

cartilage, and elastic tissue of the airway walls is seen.

This results in uniform dilatation of the tracheal and bronchial lumina and increased distensibility of the tracheal and bronchial walls. This tracheobronchomalacia leads to recurrent infections in the dependent lungs.

Mechanisms of development of Bronchiectasis Congenital bronchial atresia or mucocele This condition is characterized by

congenital focal obliteration of the lumen of a segmental bronchus, resulting in focal bronchiectasis and air trapping more distally. The dilated airway is commonly filled by inspissated mucus, which may occasionally calcify and looks like nodule .

Mechanisms of development of Bronchiectasis Conversely, acquired mucocele is caused

by focal scarring of a segmental bronchus because of prior granulomatous infection or from an endobronchial lesion. It should be differentiated from congenital bronchial atresia by the absence of air trapping of the distal lung parenchyma.The presence of an acquired mucocele should prompt further interrogation to exclude the possibility of an endobronchial neoplasm.

Mucociliary Clearance Abnormalities

Abnormalities of the structure and function of the cilia of the airway epithelium, as seen in primary ciliary dyskinesia or immotile ciliary syndrome, leads to ineffective mucus clearance and secondary colonization of the airway lumina by bacteria. This chronic infection and repeated bouts of pneumonia lead to bronchiectasis.

Mucociliary Clearance Abnormalities Kartagener's syndrome or triad is

present in half of the PCD patients. This triad consists of situs inversus, bronchiectasis, and sinusitis.

Cystic fibrosis as cause of Bronchiectasis Cystic fibrosis--- CF is an autosomal recessive

trait and occurs in approximately 1 in 3000 live births in the United States and Europe.

Failed secretion of chloride leading to dehydration of the endobronchial secretions.

This thickened mucus cannot be efficiently cleared by the mucociliary system, leading to obstructed airways and bacterial infection.Colonization and recurrent infection

Hyper-Immune Response as a cause for Bronchiectasis Inflammatory bowel disease, rheumatoid

arthritis, Sjogren disease, antineutrophilic cytoplasmic antibody (c-ANCA)–positive vasculitis (Wegener disease), and allergic bronchopulmonary aspergillosis all can lead to bronchiectasis, possibly because of inflammation of the airway wall in the setting of a hyperimmune response to internal or external antigens. The chronic inflammation damages the bronchial walls, leading to bronchiectasis.

Hyper-Immune Response as a cause for Bronchiectasis Granulomata in the bronchi and

bronchioles, associated with mucus impaction.

It is most commonly seen in patients with atopic rhinitis, asthma, or CF

Mechanisms of development of Bronchiectasis It should be noted that bronchioles

are also line by surfactant and that displacement of surfactant by inflammatory exudates leads to the bronchiolar instability and thus impairs their function.

Mechanisms of development of Bronchiectasis Vaccines and antibiotics have

dramatically reduced the frequency and the importance of bronchiectasis.

At present, the most common cause of bronchiectasis is adenovirus infection.

Post-infectious conditions ¦e.g. necrotising bacterial, viral or

fungal pneumonia

Mechanisms of development of Bronchiectasis Rubella,whooping cough

(B.pertusis),measles in childhood

Post infective Bronchiectasis Measles and Pertusis Adeno & Influenza virus Bacterial infection with

virulent organisms: S.aureus, Klebsiella Anaerobes

Atypical mycobacteria Mycoplasma HIV TB Fungi

Types of Bronchiectasis

1. Cylindrical or tubular bronchiectasis

2. Varicose bronchiectasis 3. Saccular or cystic bronchiectasis

1.Cylindrical or tubular or fusiform bronchiectasis1.Cylindrical/tubular

bronchiectasis

The luminal dilatation is uniform and the wall thickening is smooth and there is failure of normal tapering of the bronchi.

2.Varicose bronchiectasis

The bronchi resemble like varicose veins and also like serpentine.The luminal dilatation is characterized by alternating areas of luminal dilatation and constriction, creating a beaded appearance, and the wall thickening is irregular.This varicose bronchiectasis serves as an intermediate step before the development of grossly dilated, cystic airways.

3.Saccular or cystic bronchiectasis Most severe form of

bronchiectasis.The bronchi are severely dilated and the bronchi end blindly in a dilated thick-walled cyst

Clinical Manifestation of Bronchiectasis Chronic Cough (90 %) Dyspnea (72%) Hemoptysis (56%) Chest pain Malnutrition/wasting Anemia

Clinical Manifestation of Bronchiectasis Use of accessory muscles Pursed lip breathing Cyanosis Signs of Cor pulmonale Increased respiratory rate Increased heart rate

Clinical Manifestation of Bronchiectasis

Copious postural foul smelling mucopurlant sputum

Sputum production Mild <15 cc/d Moderate 15-150 cc/d Severe >150 cc/d

Clinical Manifestation of Bronchiectasis Clubbing of fingers Wheez Rhonchi

Symptoms of acute exacerbation of Bronchiectasis Change in sputum production Increased dyspnea Increased cough Fever Increased wheezing Malaise,fatigue,lethargy or decreased

tolerance Reduced pulmonary function Changes in chest sounds New radiographic changes

Bronchiectasis Complications Pneumonia Lung abscess Empyema Septicaemia Cor pulmonale Metastatic cerebral abscesses Secondary Amyloidosis with nephrotic

syndrome Recurrent pleurisy

Bronchiectasis Complications Broncho pleural fistula Purulent pericarditis Respiratory failure Metastic abcesses in bones etc

Investigations Bronchiectasis CRP Increased Sed’ rate Anaemia

Radiological features of Bronchiectasis Bronchi,

bronchioles are dilated the bronchial walls are thickened and shown as ring shadows on plain x ray.

Radiological features of Bronchiectasis 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)

Radiological features of Bronchiectasis

In mild cases x ray may be normal.

RADIOLOGY - CXRBronchiectasis

- vessel ‘crowding’- loss of vessel markings- tramline/ring shadows- cystic lesions/ air-fluid

levels- evidence of TBPoor: diagnostic sensitivity monitoring of progression

Radiological features of Bronchiectasis The definitive

diagnosis is made bronchographically (by instillation of radiographic contrast medium into the lung).

CT Scan of Bronchiectasis

Shows peribronchial thickening, dilated bronchioles.


Shows peribronchial thickening, dilated bronchioles

CT Scan of Bronchiectasis CT scan also may show

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


Marked airway dilatation both upper lobes.

CT Scan of Bronchiectasis- Bronchial dilatation

- Bronchial wall

thickening

Further investigations of Bronchiectasis IgE , IgM, IgG levels. Aspergillus precipitin . Sputum culture: fungi /

Pseudomonas aeuruginosa, H.influenzae.etc

EKG. Film PNS

Further investigations of Bronchiectasis Diffuse bronchiectasis Yellow nail

syndrome Pleural fluid TG/Chol lymphedema.

Diffuse panbronchiolitis HRCT(bizzare nodules) transbronchial biopsy.

Further investigations of Bronchiectasis Lung function: Airflow obstruction – FEV1

decreased. Air trapping - RV increased

Further investigations of Bronchiectasis Sweat test - Increased sodium

and chloride in cystic fibrosis Bronchoscopy: Obstruction –

foreign body, tumor. Immunoglobulin levels Cilia function and structure –

Kartagener's syndrome Alpha-1 antrip level.

Treatment of Bronchiectasis

Treatment Goals:

1.Eliminate cause 2.Improve tracheo bronchial

clearance 3.Control infection 4.Reverse airflow obstruction


Proposed courses of treatment

Short course Prolonged course Intermittent regular courses Inhalation

Treatment of Bronchiectasis Antibiotics and anti inflammatory

agents.

Are used in these proposed courses To treat acute exacerbation,To

prevent infection or to reduce bacterial burden

Treatment of Bronchiectasis Drainage Procedures Postural drainage consists of

adopting a position in which the lobe to be drained is uppermost.postural drainage should be performed for a minimum of 5-10 minutes twice a day.

Treatment of Bronchiectasis Gentle percussion of the chest wall with

cupped hands aids dislodgement of sputum.

Baroscopic removal of inspissated secretions is rarely required.

Role of mucolytic agents is controversial.

Treatment of Bronchiectasis Antibiotic theraphy.

Choice of antibiotic sould primarily be based on the results of culture and sensitivity.

When no specific pathogen is identified and the patient is not seriously ill,an oral agent like


AmoxycillinAmpicillinTetracyclineCotrimoxazoleor a fixed combination of amoxycillin

and clavulanic acid should be used.more seriously ill patients with pneumonitis should be given parenteral antibiotics.

Treatment of Bronchiectasis Duration of theraphy is variable. A 5-10 days course is usually

sufficient.in some patients it may need to be prolonged for several weeks.

Treatment of Bronchiectasis Brochiodilators Brochiodilators to improve

obstruction and aid clearance of secretions are particularly usefull when some element of reversible airway obstruction is present.

Treatment of Bronchiectasis Other Measures Cigarette smoking should be stopped. A programme of graded exercise ,routine

deep breathing and maintance of nutrition form essential part of general management.

Episodes of sinusitis should be treated. Complicating allergic bronchopulmonary

aspergillosis should be treated with prednisolone.

.

Treatment of Bronchiectasis Primary Hypogammaglubolinemia

should be treated with human immune serum albumin.

Complicated cases may require nasal oxygen on a chronic basis, to maintain adequate oxygenation


3 Strategies 1. High oral dose for prolonged

period (at least 4 weeks) (macrolide and FQ) Azithromycin 500mg plus Levofloxacin 250mg 2/wk x 6Mn (reduced exacerbation)


2. Aerosolized antibiotic (e.g during alternate month) (Gentamicin 40mg bid x 3 days.

Tobramycin 300mg bid x 4wk, (Gatifloxacin) Fortum 1g +

Tobramycin 100mg bid x 12mn.

Treatment of Bronchiectasis 3. Regular pulsed course of iv

antibiotic (e.g.2-3 wk courses with 1 or 2 months in between).

Treatment of Bronchiectasis These episodic or suppressive

antibiotics

Decrease inflammation Slow progression Eradication

Treatment of Bronchiectasis Anti-inflammatory agents :

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.

Treatment of Bronchiectasis Anti-inflammatory

agents :Systemic corticosteroid May better at penetrating the bronchial wall and therefore be more effective but available data only for CF-Bronchiectasis.inhibit neutrophil mediated inflammation.

Treatment of Bronchiectasis 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.

Treatment of Bronchiectasis Anti-inflammatory agents

Macrolide (Azithromycin) suppress inflammation.

Azithromycin 500mg twice a week x 6mn Decrease daily sputum production and exacerbation.

Treatment of Bronchiectasis 3 Months of Clarithromycin

decreased total No. of leucocyte, proportion of PMN and concentration of IL-8.

Treatment of Bronchiectasis Brochiodilators

Anticholinergic or oral Methylxanthine in patient with non-CF bronchiectasis.

Bronchopulmonary hygiene physical therapy 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.

Bronchopulmonary hygiene Physical therapy Rhythmic Chest clapping or

cupping and of back, while the patient assumes a number of positions (head down, primarily), may help the lungs to drain more effectively. This is called chest physical therapy, or percussion and postural drainage.

Bronchopulmonary hygiene Physical therapy Inflatable vests or mechanical

vibrators.Oral devices that apply positive end-expiratory pressure maintain the potency of the airway during exhalation.

Inspiratory muscle training X 8WKS.

Mucolytics Medications are available to help

thin the sputum, so that it can be more effectively coughed up.

Mucolytics Maintain adequate systemic hydration

by normal saline,enhanced by nebulization with saline.

Acetylcysteine delivered by nebulizer thins secretions.Aerosolized recombinant human DNase (rhDNase) in patients with cystic fibrosis.


Alpha-1 antrip def Alpha-1 antritrip replacement.

Oxygen and diuretics

Treatment of Bronchiectasis Anti-inflammatory agents

Macrolide (Azithromycin) suppress inflammation.

Azithromycin 500mg twice a week x 6mn Decrease daily sputum production and exacerbation.

Surgical treatment of Bronchiectasis Surgery : If the area of bronchiectasis is

localized and symptoms are debilitating or life threatening.

Surgical treatment of Bronchiectasis When bleeding occurs from

irritated bronchial tubes and overgrown bronchial blood vessels, surgery may be required either to remove an area of the bronchial tube, or to inject the bleeding blood vessel with a material to stop the bleeding.

Surgical treatment of Bronchiectasis In some patients, bronchiectasis

eventually leads to a constantly low level of blood oxygen, despite other treatments. These patients usually have an associated increase in the size of the right side of their hearts, along with a decrease in the heart's ability to pump blood through the lungs. Some patients with extremely severe symptoms and disability have been treated with lung transplantation.

Surgical treatment of Bronchiectasis Surgery Localized bronchiectasis Proximal obstructive

lesion,Obstruction may require the removal of a foreign object or tumor or aspirated material

Massive hemoptysis Recurrent infections Lung transplant

Treatment of Bronchiectasis Prevention of Bronchiectasis

Adequate treatment and prophylaxis of childhood whooping cough, measles, and primary tuberculosis.

Genetic counseling in cystic fibrosis.

PSEUDOBRONCHIECTASIS A true bronchiectasis is not

reversible.whereas the pseudobronchiectasis is reversible.

PSEUDOBRONCHIECTASIS The bronchographic abnormalities

displayed by atelectasis and tracheo bronchitis

with ulceration of bronchial mucosa simulate cylindrical bronchiectasis.but re expansion of collapsed lung in atelectasis and regeneration of mucosa in trachiobroncitis result in reversibility of bronchographic appearance.this is known as pseudobronchiectasis.

Bronchiectasis sicca ( dry bronchiectasis)

This is a condition where bronchiectasis presents with repeated episodes of hemoptysis without sputum production.this usually occurs in upper lobe bronchiectasis of post tuberculous variety.

Middle lobe syndrome Or Brocks syndrome post

obstructive. bronchiectasis

THANK YOU SO MUCH

Trust the Physician and the teacher , and drink his remedy in silence and tranquility: For his hand, though heavy and hard, is guided by the tender hand of the unseen,And the cup he brings, though it burn your lips, has been fashioned of the clay which the Potter have moistened with His tears and sacred feelings.

bronchiectasis by dr bashir ahmed dar associate professor medicine sopore kashmir

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