bronchiectasis

Bronchiectasis,Definition an acquired disorder of the major bronchi and bronchioles characterized by permanent abnormal dilation and destruction of bronchial walls the most common symptoms are chronic cough with large amounts of purulent sputum and/or recurrent hemoptysis,it involves chronic respiratory tract infection and an up-regulation of the host inflammatory response Enhanced cellular and mediator responses bronchial mucosal biopsies reveal infiltration by neutrophils and T lymphocytes; expectorated sputum has increased concentrations of elastase and the chemoattractants IL-8, TNFa, and prostanoids,Aetiology and pathogenesis of Bronchiectasis,Bronchiectasis is usually caused by chronic inflammation and infection in airways. TB is the most common cause of Bronchiectasis world-wide. Localised bronchiectasis may be due to bronchial distension resulting from the accumulation of pus beyond an obstructing bronchial lesion, such as enlarged tuberculous hilar lymph nodes, a bronchial tumour or an inhaled foreign body (e.g. an aspirated peanut).,CAUSES OF BRONCHIECTASIS,Congenital - Cystic fibrosis . - Ciliary dysfunction syndromes : Primary ciliary dyskinesia (immotile cilia syndrome) Kartageners syndrome (sinusitis and transposition of the viscera) - Primary hypogammaglobulinaemia Acquired-children - Pneumonia (complicating whooping cough or measles) - Primary TB . - Inhaled foreign body . Acquired-adults -Suppurative pneumonia. -Pulmonary TB . -Bronchial tumours - Allergic bronchopulmonary aspergillosis complicating asthma,Normal Lung and the Lung of a Patient with Bronchiectasis. Bronchiectasis is primarily in the lower lobe, which is the most common distribution. The saccular dilatations and grapelike clusters with pools of mucus are signs of severe bronchiectasis.,Normal lung,Bronchiectasis,Morphological types Cylindrical or tubular bronchiectasis: dilated airways alone, sometimes seen as a residual effect of pneumonia Varicose bronchiectasis: focal constrictive areas along the dilated airways that result from defects in the bronchial wall Saccular or cystic bronchiectasis: progressive dilatation of the airways, which end in large cysts, saccules, or grape-like clusters (the most severe form ),Bronchiectasis. A, Varicose bronchiectasis. B, Cylindrical bronchiectasis. C, Saccular bronchiectasis. Also illustrated are excessive bronchial secretions (D) and atelectasis (E), which are both common anatomic alterations of the lungs in this disease.,D,E,A,B,C,Cylindrical bronchiectasis. Left posterior oblique projection of a left bronchogram showing cylindrical bronchiectasis affecting the whole of the lower lobe except for the superior segment. Few side branches fill. Basal airways are crowded together, indicating volume loss of the lower lobe, a common finding in bronchiectasis. (From Armstrong P et al: Imaging of diseases of the chest, ed 2, St. Louis, 1995, Mosby.),Saccular bronchiectasis. Right lateral bronchogram showing saccular bronchiectasis affecting mainly the lower lobe and posterior segment of the upper lobe. (From Armstrong P et al: Imaging of diseases of the chest, ed 2, St. Louis, 1995, Mosby.),Varicose bronchiectasis. Left posterior oblique projection of left bronchogram in a patient with the ciliary dyskinesia syndrome. All basal bronchi are affected by varicose bronchiectasis. (From Armstrong P et al: Imaging of diseases of the chest, ed 2, St. Louis, 1995, Mosby.),Bronchiectasis,The lumen of the bronchus is dilated and there is prominent chronic inflammation in the bronchial wall,Bronchiectasis can present in either of two forms a local or focal obstructive process of a lobe or segment of a lung or a diffuse process involving much of both lungs and often accompanied by other sinopulmonary diseases, such as sinusitis and asthma,Pathophysiology and Etiology Three types of focal airway obstruction(1) luminal blockage by a foreign body, broncholith, or slowly growing benign tumor(2) extrinsic narrowing due to enlarged lymph nodes (middle lobe syndrome)(3) twisting or displacement of the airways after a lobar resection or chronic infection,Pathophysiology and EtiologyInfections Childhood respiratory tract infections (pertussis or whooping cough) Chronic infections( chronic lung abscess, TB)Primary Ciliary Dyskinesia an autosomal recessive syndrome, estimated frequency at birth is 1 in 15,000 to 1 in 40,000 half have Kartageners syndrome (bronchiectasis, sinusitis, and situs inversus viscerum),Pathophysiology and EtiologyImmunodeficiency humoral immunodeficiency syndromes (deficiencies of IgG, IgM, and IgA) at risk for recurrent suppurative sinopulmonary infections and bronchiectasis immune globulin replacement reduces the frequency of infectious episodes and prevents further destruction of the airways,Clinical findingsSymptoms: daily cough productive of mucopurulent phlegm intermittent hemoptysis recurrent lung infectionsshortness of breathPhysical findings: crackles, cyanosis,Chest radiography,High-resolution computed tomographic scanning (HRCT) the defining modality for diagnosis of bronchiectasis Specific abnormalities found on HRCT include (1) dilatation of an airway lumen 1.5 times as wide as a nearby vessel (2) lack of tapering of an airway toward the periphery (3) varicose constrictions along airways (4) ballooned cysts at the end of a bronchus,Bronchoscopy an important diagnostic tool to examine for obstruction ( foreign body, tumor, structural deformity, or extrinsic compression from lymph nodes) and to help localize the bleeding，to identify pathogen,Pulmonary function tests,obstructive impairment ( low FEV1 and/or low FEV1/FVC) airway hyperresponsiveness ( 40% with a positive reversibility test result, and 30% to 69% with a positive bronchial provocative test result),Assessment of ciliary function,A screening test can be performed in patients suspected of having a ciliary dysfunction syndrome by assessing the time taken for a small pellet of saccharin placed in the anterior chamber of the nose to reach the pharynx, when the patient can taste it. This time should not exceed 20 minutes and is greatly prolonged in patients with ciliary dysfunction. Ciliary beat frequency may also be assessed using biopsies taken from the nose. Structural abnormalities of cilia can be detected by electron microscopy.,When to suspect bronchiectasis?,Chronic cough, sputumhemoptysisCoarse ralesPersistent respiratory symptoms Recurrent pneumoniaProgressive obstructive lung disease clubbing,Clinical Characteristics,Sputum productionMild 150 cc/d,Bronchiectasis,If wide spread DyspneaClubbing of the fingers h pulmonary blood pressure Cor pulmonale,Diagnostic evaluation to confirm the diagnosis of bronchiectasis to identify potentially treatable underlying causes to provide functional assessment Imaging of the chest is always necessary to confirm the diagnosis,Differential diagnosis Chronic bronchitis Lung abscess Pulmonary tuberculosis Congenital bronchocele Diffuse panbronchiolitis,Treatment,Treatment of the specific underlying causeEducationAirway clearancePhysiotherapy and exerciseMucolytic and hyperosmolar therapiesAirway drug therapyBronchodilationAnti-inflammatoryAntibiotic therapy Surgical management Management of complications,Smoking Cessation,Associated airways obstructionSuper-added COPD?Increased cancer risk.,Airway clearance,All patients with a productive cough should be taught airway clearance by a physiotherapistActive cycle of breathing techniquesPostural drainagePositive expiratory pressure (PEP)AcapellaDuration?Frequency?,Bronchopulmonary HygienePostural drainage, chest physiotherapy, thinning and loosening of secretions, the administration of a bronchodilator and of inhaled corticosteroids part of maintenance therapy and treatment for acute exacerbations,Physiotherapy,Airway clearance techniques,Vicious cycle,Vicious cycle,Airway clearance strategies,Airway clearance,Stable, productive bronchiectasis:Physiotherapy and adjuncts self managementReduces bacterial loadReduces exacerbation rate/need for antibioticsImproves daily symptomsAcute exacerbations:Inpatient strategies IPPB (Bird), IPV, Chest Physical Therapy (CPT), Postural drainage?Role for nebulised mucolytics mannitol, hypertonic saline, DNase, bromhexine,Physiotherapy Methods,Voluntary coughVigorous coughing equivalent to autogenic drainage or percussionHuffingVarious levels of bronchi depending on the volume of “huff”Forced Expiration Technique“Huff” - Relax - “huff”,Active Cycle of Breathing Technique (ACBT),BREATHING CONTROL,3 or 4 THORACIC EXPANSION EXERCISES,BREATHING CONTROL,3 or 4 THORACIC EXPANSION EXERCISES,BREATHING CONTROL,FORCED EXPIRATION TECHNIQUE (1 -2 huffs with breathing control),3 second hold,Chest clapping,Chest shaking,Chest shaking,Chest clapping,3 second hold,Active Cycle of Breathing,Gold Standard PhysiotherapyMoves air distal to mucous plugs into the peripheriesIncreases tidal volumeCan be done with or without an assistantRequires training/teaching,Postural Drainage,postural positionsPositions each bronchus over the tracheaGood for very thick sputumTakes 40 minutesNeeds a specialised bed of wedgeCan lead to cross contamination of segments,PEP mask,One way valve + expiratory resistor10 20 cm H2O Good in moderate sputum producersCombine with nebulised drug/ACBT30 - 60,Flutter valve,5 20 cm H2O PEEP12 Hz oscillatory vibrationSame as cilia beat frequencyAs effective as ACBTLess effective in large producers30 40Used with ACBT, not with postural drainage,Acapella device,Similar to FlutterCan be used in any postural positionAs effective as ACBT40 - 50,Inhaled Therapy,Inhaled drug therapy,MucolyticsPulmozyme (not effective)MannitolBronchodilators?Inhaled corticosteroids?,Inhaled Therapy,No evidenceICS cannot eradicate particular organismsDo improve symptomsDont reduce exacerbationsShould not be used in isolation,Inhaled Corticosteroids,Cochrane Review in 1999Only randomised double blind studies includedNon-CF bronchiectasis2 papers- 4-6 weeks duration (54 patients)Too short, too smallLarger, longer studies neededNo consensus,Long term antibiotics,Nebulised tobramycinOral azithromycin,Macrolides,Azithromycin 250 mg Thrice WeeklyClarithromycin 250 mg ODErythromycin 500 mg BD,Antibiotics,Postural drainageBased on sputum culture & sensitivityNon-Pseudomonas organism:Amoxicillin: 1gm TID 14 daysAugmentin 625 mg TID 14 daysDoxycyline 100mg BD 14 daysCiprofloxacin 750 mg BD 14 days (Pseudomonas)Ceftazidime 2gm TID 7-14 days (Pseudomonas)Nebulised Tobramycin (Pseudomonas),Hemoptysislife-threatening hemoptysis: 600 ml/ daybronchoscopy or CT of the chest is warranted to help determine which lobe or side is bleedinginterventional radiology: selective bronchial arteries embolization surgery may be required to resect an area suspected of bleeding,Surgery Removal of an obstructing tumor or residue of a foreign body Elimination of the segments or lobes the most damaged and suspected of contributing to acute exacerbations, overwhelming viscous secretions, mucous impaction, and plugs Elimination of areas that are subject to uncontrolled hemorrhage,Surgical treatment,Localised bronchiectasisProximal obstructive lesionMassive hemoptysisRecurrent infections,Surgical treatment Surgery is only indicated in a small proportion of cases. These are usually young patients in whom the bronchiectasis is unilateral and confined to a single lobe or segment as demonstrated by CT. Unfortunately, many of the patients in whom medical treatment proves unsuccessful are also unsuitable for pulmonary resection because of either extensive bronchiectasis or coexisting chronic lung disease. Resection of areas of bronchiectasis lung has no role in the management of the progressive form of bronchiectasis - for example those associated with ciliary dysfunction & cystic fibrosis .,Removal of damaged lung suspected of harboring problematic organisms such as multidrug-resistant M. tuberculosis or M. avium Double-lung transplantation is now considered for patients with cystic fibrosis and respiratory failure (one year survival rate 75% and 48% at five years),Acute ExacerbationsAn exacerbation: including four of th