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13 bronchiectasis-dr khinchi
13 bronchiectasis-dr khinchi
13 bronchiectasis-dr khinchi
13 bronchiectasis-dr khinchi
13 bronchiectasis-dr khinchi
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13 bronchiectasis-dr khinchi

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  1. Bronchiectasis Dr Yog Raj Khinchi
  2. Chronic BronchitisDefinition ADULTS: Productive cough daily for 3 months/year for 2 consecutive years. CHILDREN: Productive cough of >2 months duration or recurrent episodes of productive cough >4 times/year.
  3. Bronchitis is a state ofincreased mucusproduction, with or withoutadequate mucociliarytransport, that requires coughfor clearance of airwaysecretions.
  4. Airway Stimulus/Injury Increasing Secretion ProductionIncreased Effective SecretionMucociliary Cough Inspissation AirwayClearance Obstruction Recurrent/Persistent
  5. Chronic Bronchitis: UnderlyingConditions Chronic Airway • Asthma Infection/Inflammation: • Cystic Fibrosis • Aspiration syndromes • Chronic foreign body • Tuberculosis Primary Host Defense • Ciliary dyskinesias Abnormalities: • Immunodeficiencies • Congenital • Acquired (HIV) Inefficient Cough • Airway compression Syndromes: • Trachobronchomalacia • Neuromuscular weakness • Tracheostomies • Bronchiolitis obliterans
  6. BronchiectasisPathogenesis Airway Injury + Secretion Stimuli Secretion Stasis Infection Airway Destruction + Airway Dilation
  7. FIGURE 2
  8. CF vs. Idiopathic Bronchiectasis CYSTIC IDIOPATHIC FIBROSIS BRONCHIECTATISMulti-organ Involvement Sinopulmonary Involvement Diffuse Multifocal Pseudomones Mouth Flora Tenacious Secretions Mucoid/Coughable 50% Bronchodilator 50% Bronchodilator Response Response Progressive Outcome Variable Outcome
  9. Treatments for Idiopathic (Post-Infectious)Bronchiectasis Reduce Secretion • Reduce Irritant Exposure Production: • Reduce Aspiration Events • Reduce GER Reduce Infection Exposure: • Dental Hygiene Antibiotics for: Upper Airway Lower Airway Exacerbations Promote Secretion • Chest Physiotherapy Clearance: Reduce Airway Obstruction: • ?Bronchodilators • Inhaled Steroids Prevent Infection: • Immunizations • ?Palivizumab, Flu shots • Pneumococcal vaccine (7 + 23 valent)
  10. Bronchiectasis• Irreversible, abnormal dilatation of one or more bronchi, with chronic airway inflammation. Associated chronic cough, sputum production, recurrent chest infections, airflow obstruction, and malaise• Prevalence unknown (not common)• Pathological endpoint with many underlying causes
  11. Pathogens associated with exacerbations and disease progression in bronchiectasis • Non-tuberculosisHaemophilus influenzae mycobacteriaHaemophilus parainfluenzae – M. avium complex (MAC)Pseudomonas aeruginosa – M. kansasii – M. chelonae – M. fortuitumStreptococcus pneumoniae – M. malmoenseMoraxella catarrhalis – M. xenopiStaphylocccus aureus • Aspergillus-relatedStenotrophomonas maltophiliaGram-negative enterobacter disease
  12. Causes / associations of bronchiectasis• Idiopathic• Postinfectious• Humoral immunodeficiency• Allergic bronchopulmonary aspergillosis (ABPA)• Aspiration/GI reflux• Rheumatoid arthritis• Youngs Syndrome• Cystic Fibrosis• Ciliary dysfunction• Ulcerative colitis• Panbronchiolitis• Congenital• Yellow nail syndrome
  13. Evidence for dysregulated immunity in bronchiectasis• Increased susceptibility to infection - bacterial, non- tuberculous mycobacterial (NTM), and aspergillus-related lung disease• Associated with autoimmune disease such as the inflammatory bowel disease, ulcerative colitis• Neutrophils are markedly raised, as predicted from high local levels IL-8• Associated with immune deficiency syndromes such as TAP deficiency syndrome
  14. Evidence for dysregulated immunity in bronchiectasis• Increased susceptibility to infection - bacterial, non- tuberculous mycobacterial (NTM), and aspergillus-related lung disease• Associated with autoimmune disease such as the inflammatory bowel disease, ulcerative colitis• Neutrophils are markedly raised, as predicted from high local levels IL-8• Associated with immune deficiency syndromes such as TAP deficiency syndrome
  15. Bronchiectasis associated with increased susceptibility to specific pathogens • Non-tuberculosisHaemophilus influenzae mycobacteriaHaemophilus parainfluenzae – M. avium complex (MAC)Pseudomonas aeruginosa – M. kansasii – M. chelonae – M. fortuitumStreptococcus pneumoniae – M. malmoenseMoraxella catarrhalis – M. xenopiStaphylocccus aureus • Aspergillus-relatedStenotrophomonas maltophiliaGram-negative enterobacter disease
  16. Evidence for dysregulated immunity in bronchiectasis• Increased susceptibility to infection - bacterial, non- tuberculous mycobacterial (NTM), and aspergillus-related lung disease• Associated with autoimmune disease such as the inflammatory bowel disease, ulcerative colitis• Neutrophils are markedly raised, as predicted from high local levels IL-8• Associated with immune deficiency syndromes such as TAP deficiency syndrome
  17. Bronchiectasis associated with autoimmune disease • Rheumatoid arthritis • Systemic lupus erythematosus • Relapsing polychondritis • Inflammatory bowel disease - Ulcerative colitis and Crohn’s disease
  18. Evidence for dysregulated immunity in bronchiectasis• Increased susceptibility to infection - bacterial, non- tuberculous mycobacterial (NTM), and aspergillus-related lung disease• Associated with autoimmune disease such as the inflammatory bowel disease, ulcerative colitis• Neutrophils are markedly raised, as predicted from high local levels IL-8• Associated with immune deficiency syndromes such as TAP deficiency syndrome
  19. Suppurative Lung Disease• Bronchiectasis• Lung abcess• Empyema
  20. Brochiectasis• Def:destructive lung disease :chronic permanent and abnormal dilatation of bronchial wall with variable inflammatory process in the lung. Chronic bronchial sepsis• Pathology: non-inflammatory:congenital acquired: Bronchial wall dilatation. excess mucus. loss of connective tissue support inflammation with metaplasia of ciliated epithelium to squamous or columnar + micro-abcess formation. Collapsibility of the wall leads to obstructive defect on spirometry.
  21. Notes• Bronchiectasis is of two types:1- Dry: there is dilatation, destruction, but no purulent secretion.2- Suppurative (refers to chronic bronchial sepsis): this is bronchiectasis that have suppuration ( pus formation).
  22. Pathogenesis• Vicious cycle: infectious insult & impaired drainage &/or defect in host defence• Role of positive : genetic susceptibilty family history
  23. Notes• Normally, in healthy individuals the immune system and mechanical system (cilia function) are intact, resulting in good clearance of microorganisms leading to recovery.• Therefore, with impaired cilia function and/or host defense a vicious cycle (ongoing inflammation) will occur leading to bronchiectasis.• People with family history and genetic susceptibility (e.g a- 1 antitrypsin deficiency), when having a microbial insult, they can not clear it probably because of defective immune and/or mechanical drainage system.• The most important organisms are: H.influenza, encapsulated streptococcus pneumoniae, and pseudomonus.
  24. ClassificationSpectrum:• 1.follicular cylindrical:transmural inflammation, loss of bronchial elastic tissue, mucosal edema; post infectious• 2.Saccular:ulceration ,craters; general dilatation specially terminal (saccules). (varicose:distortion by scarring and obstructions)• 3. Atelactatic: localised, could be secondary to 1, or 2.importance to site and distribution: lobar or segmental.
  25. Notes• Another type of classification is:1- follicular cylindrical it is called follicular because of presence of lymphoid follicles in the bronchus. But it is generally called cylindrical because it is transmural and localized to one bronchus.2- saccular: so called because there are succule like formation at the terminal part of the bronchus.- varicose: this is when you have two types together cylindrical and saccular.
  26. Causes of bronchiectasisCongenital:elastic bronchial wall def. other congenital abnormalities,lung sequestrationMechanical bronchial obstruction:Intrinsic and extrinsic:Lymph node or scaring :post-TBForeign body: selective lobesChild vs adult
  27. Notes• Causes:1- congenital:in fetus, the lung is not mature enough because its blood supply is deprived, therefore making it liable to bronchiectasis.2- bronchial obstruction: the process of obstruction will cause stagnation of secretion which will then start the process of microbial infection leading to abcess formation and then tissue destruction, hence bronchiectasis.- Bronchial obstruction can be intrinsic (in wall or lumen) or extrinsic (external compression).
  28. Cont’d Notes- TB produces both intrinsic (by scarring) and extrensic (compression by lymph node).• The common site of obstruction is the right posterior upper lobe.• Obstruction of airways in children can be caused by inhaling a foreign body, and the inhalation of a foreign body also leads to lung abcess.• Obstruction in adults can occur due to inhaling chemicals or impaired gastroesophageal reflex and aspiration.
  29. *Cont’d Causes*Inflammatory pneumonitis-Aspiration:chemical ,gastric-Inhalation: fumes*Granulomata and fibrosis: sarcoidosis ,TB,IPF*Immunological over-response: ABPA (allergic bronchial pulmonary aspergellosis), post lung transplant*Immune deficiency Primary: children: hypogammaglobinaemia:repeated sinopulmonary infection Secondary: AIDS, CA
  30. Cont’d Causes*Mucociliary clearance defects-Genetic -Primary ciliary dyskinesia :cilia abnormal 50% Kartegner’s:S.I, sinusitis Bronchiectasis. -Cystic Fibrosis: abnormal mucus content.*Acquired: -Young’s: sinusitis ,bronchiectasis ,obstructive azoospermia. Abnormal mucus.- -Post-infective bronchial damage:measles, pertussis. MAI.Viral.Mycoplasma- Toxins: SO2,smoking, lidocaine. Asthma.*Rheumatic and systemic inflammatory diseases:e.g IBD (inflammatory bowel disease).*Idiopathic (including diffuse panbronchiolitis), good % with no cause
  31. Notes• In cystic fibrosis, the cilia is normal but the mucus is abnormal because there is a block in the chlorine channel (not responsive to cyclic AMP) causing the mucus to become more sticky. This disease is characterized by increased Na and Cl in sweat, which is detected by sweating test.• Kartegner syndrome: is a type of primary ciliary dyskinesia which is also associated with sinusitis, bronchiectasis, and transposition of the viscera.• Young’s disease is characterized by high lipid content of mucus.• Idiopathic: panbronchiolitis (responsive to macrolide) is found mainly in Japanese and East Asia.
  32. Cont’s Notes• ABPA causes hyper immune activity.• Atypical mycobacterial infection and uncontrolled asthma can cause abnormal cilia.
  33. *Mucociliary clearance defects• *Genetic: Cystic fibrosis: AR, Gene mutation,chromosome 7,Transmembrane conductance regulator pr-CFTR commonest:deletion at 508 impairment of chloride channel.non respondent cyclic- AMP. Cl- and water not excreted but Na and its water absorbed ,mucus get thickened.Sweat content of Na , Cl altered.
  34. Clinical manifestation of cystic fibrosis Primary• Bronchiectasis *Nasal polyps• Bronchiolitis, bronchitis * Cirrhosis• Infertility * Meconium ileus• Pancreatic insufficiency• Pneumonia• Salt loss
  35. Secondary*Atelectasis * Allergic bronchopulmonary*Clubbing aspergillosis*Malabsorption * cholelithiasis*Heat prostration * Cor pulmonale*Hypochloraemic * Conductive hearing loss hypokalaemic alkalosis * Diabetes mellitus
  36. Diagnosis of Bronchiectasis• 1.History: usually :muco-purulent sputum, cough. occasionally recurrent hemoptysis, recurrent feverLess specific : dyspnea, wheeze ,pleuritic pain• 2.Examination: Early: normal nowadays:clubbing 3%,crackles70% rarely :Core pulmonale( right ventricular hypertrophy due to pulmonary hypertension),CCF.
  37. NotesThe most important sign is is abundant productive cough increasing with lying down.• The second important manifestations are lung abcess, infective emboli, clubbing and amyloidosis (AA type).• How hemoptysis occurs?An insult causes injury, followed by healing and neovascularization. These new formed blood vessels are weak and fragile, hence easily ruptured causing hemoptysis.- Wheeze: musical sounds produced by air passing through narrowed airways (during inspiration & expiration).- Crackles (crepitations): non-musical sounds mainly heard during inspiration caused by reopening of occluded small airways.
  38. Investigation• Sputum: gram stain,selective media ( it is done not to miss certain organisms),semi –quantitative• !!TB, fungal• CXR: early volume loss,vascular crowdening late:cylindrical tramline,cystic.• Disribution: central ABPA, LL: idiopathic UL: CF or variant
  39. Investigation• Bronchogram: surgery• HRCT: standard• Sinus X-Ray• PFT: normal,obstructive,air trapping• Specific: Ig level:IgA (in immunocompromised patients).• precipitin level is measured in skin, sputum and serum. If it is high in 2 out of the 3 sites, we think of ABPA.• Brochoscopy
  40. Notes• Bronchogram: do a bronchoscope then inject a dye. It is done when you are planning for surgery.• HRCT= high resolution CT scan.• Sinus X-ray is done to rule out sinusitis• PFT is early normal, later:obstruction/ air trappin• Bronchoscopy is indicated in case of hemoptysis and it is important to rule out obstructive lesions (e.g. foreign bodies) and TB. These three conditions are not responding to antibiotics.
  41. Notes• The previous slide shows CT taken during inspiration.• Usually, a blood vessel has a bronchus beside it that has the same size.• So, if you see a bronchus larger than the accompanied vessel then suspect bronchiectasis.
  42. Notes• The previous CT was taken for the same patient during full expiration, which is better than the full inspiratory CT.
  43. Cont’d investigation• UGI Endoscopy• Anti protease level• Sweat test• Genetic studies• Rh. Factor
  44. Complication• Mostly: infective exacerbation,haemoptysis• Rarely now: metastatic spread of infection empyema amyloidosis clubbing joint pain• Note: certain complications are rare because of early detection.
  45. Management• Prevention:• Medical treatment:control infection ,bronchial hygiene• Antibiotic:acute exacerbation: cover I.infl., strep. Prophylactic:several protocols: commoner : 10days/Month or 10 days alternate with 10 free days.• Bronchodilator• IV Ig if deficient• Oral Steroid:only ABPA vs inhaled steroids• Mucolyte :debatable.role of ACC ( N-acetylcystin which is antioxidant), DNAs• Anti-fungal :itracanazole: ABPA• Future treatment:Antiprotease replacement• CF treatment (very complicated)
  46. Notes• Management of bronchiectasis includes:- 1- Giving Igs for immunocompromised patients.- 2- Vaccination for H. influenza, pneumococcus and influenza.- 3- Screening for TB (very important).- 4- Bronchodilators (almost always given).- 5- Steroids (only for ABPA because of the hyperimmunity).- 6- mucolytics: make sputum more liquid and less viscus (not important except DNAs in cystic fibrosis patients to improve their lung function).
  47. Cont’d managementPhysiotherapyCorner stone:Postural drainage,hydration ,nebulized saline*Surgery: .Massive haemoptysis: > 600ml/day. .Localized troublesome resectable bronchiectasis .Palliative for important stump.
  48. Notes- physiotherapy: teach the patient how to drain his lung (lungs should be drained daily).- Surgery: is indicated:1- when there is severe hemoptysis2- if one lobe causing problems to the patient, so you resect it to prevent damage to other lobes3- if you have a diffuse lung disease and one particular area which is more problematic, then you resect it.- Mild hemoptysis: loss of 200-300ml/day.- Moderate hemoptysis: loss of 300-600ml/day.- Severe hemoptysis: loss of > 600ml/day.
  49. Cont’d management Haemoptysis:*Mild: antibiotic*Severe: surgery or bronchial artery embolizationLung Transplant: always double.- Indication for transplantation: bilateral, advanced, and bleeding bronchiectasis.- 2 lungs should be transplanted because if you transplant one lung only, the diseased lung you left in the body can affect the new transplanted lung to become also infected.
  50. Bronchiectasis
  51. “Chronic dilation of the bronchi marked by fetid breath and paroxysmal coughing, with the expectoration of mucopurulent matter.”
  52. Morphological types• Cylindrical or tubular bronchiectasis• Varicose• saccular or cystic bronchiectasis
  53. Bronchiectasis• Causes and pathogenesis• Microbiology /common pathogens• Therapeutic Goals
  54. Ct/cxr
  55. Ct/cxr
  56. BronchiectasisWorldwide, infection is the primary cause – M. Tuberculosis – Childhood illnesses • Rubeola, B. Pertussis
  57. Childhood Infections M. Tb. Infection Inflammation Bronchiectasis Altered development
  58. InflammationCharacteristics across etiologies:- Persistent- Neutrophil dominant- Pro-inflammatory cytokines (IL- 8, IL-1, TNF-a)- Low anti-inflammatory cytokines (IL-10)
  59. Airway Damage Failure to Resolve Inflammation– Inappropriate inflammation e.g. ABPM, CF (?)– Impaired clearance of stimuli Bacteria, mucus, toxins
  60. Airway Damage Failure to Resolve Inflammation– Altered Airway Milieu Proteolytic damage -e.g. cleaved receptors -impaired macrophage function* Oxidant stress -low antioxidants associated with worse disease -dysregulation of signaling and cellular function‡
  61. Childhood Infections M. Tb. Infection Inflammation* Impaired Clearance Bronchiectasis Altered development
  62. Impaired ClearanceAltered ciliary Function PCD, smoking, CFTR, Young’sMucus rheology CFTR, Mucoid Ps. ADilated or obstructed airways Impaired cough, foreign bodies, aspiration
  63. Impaired Immunity Ineffective inflammationAcquiredChemo-immunomodulationCongenital/innate
  64. HIV/AIDS Chemo-Immunosuppression anti-TNF, MTX, anti-neoplasticsChildhood Infections M. Tb. Innate Deficiency CGD, CVID, Ig’opathy (IFN) Infection Inflammation Impaired Clearance foreign body, CF Bronchiectasis Altered development
  65. HIV/AIDS Chemo-ImmunosuppressionChildhood Infections M. Tb. Innate Deficiency InfectionAuto-Immune Impaired Clearance RA, Sjogren’s, IBD InflammationABPM Bronchiectasis Altered development
  66. HIV/AIDS Chemo-ImmunosuppressionChildhood Infections M. Tb. Innate Deficiency Infection Auto-Immune Inflammation Impaired Clearance ABPM Bronchiectasis Altered development
  67. HIV/AIDS Chemo-ImmunosuppressionChildhood Infections M. Tb. Innate Deficiency Infection Auto-Immune Inflammation Impaired Clearance ABPM Bronchiectasis Altered development
  68. Bronchiectasis Epidemiology and EtiologyWho?Patients - with altered immune system - with persistent respiratory symptomsWhy?Persistent inflammation in the respiratory system
  69. Bronchiectasis Therapy Decrease inflammationantibioticsclearance – Flutter, IPPV, Vest, Bronchodilators, hypertonic saline(anti-inflammatory chemotherapy) – Steroids, macrolides, interferon-gamma, ibuprofen(surgical resection)
  70. When to suspect bronchiectasis?Chronic cough, sputumCoarse ralesPersistent respiratory symptomsRecurrent pneumoniaProgressive obstructive lung diseaseFunny bugs
  71. Clinical Characteristics– Focal – Systemic • Sputum production • Malnutriton/wasting – Mild <15 cc/d • Chronic Inflammation – Moderate 15-150 – “gammaglobulinemia” cc/d – CRP – Sed’ rate – Severe >150 cc/d – anemia • Hemoptysis • Dyspnea • Chest pain
  72. Bronchiectasis Therapy AntibioticsEpisodic or suppressive antibiotics? Yes.Selective pressure vs. suppression of damage
  73. Bronchiectasis Therapy Antibiotics Con• Pro Select resistance – Decrease inflammation Cost Side effects – Slow progression adherence difficult (e.g. – Eradication? Huong et al.)
  74. Bronchiectasis Therapy Antibiotics Con• Pro Select resistance – Decrease inflammation Cost Side effects – Slow progression adherence difficult (e.g. – Eradication? Huong et al.)
  75. Bronchiectasis Therapy Antibiotics when to use? No:• Yes: Minimal disease without – Evidence of organism Patient Intolerant exacerbation “Unaffordable” – Progressive decline – “Frequent exacerbator” – Active inflammation (?)
  76. Bronchiectasis Therapy Decrease inflammationClearanceImmunomodulatory chemotherapyproposed therapies: – Steroids – Macrolides, tetracyclines – interferon-gamma – ibuprofen
  77. Bronchopulmonary Hygiene• removal of respiratory secretions is beneficial• chest percussion and postural drainage• chest clapping or cupping• inflatable vests or mechanical vibrators• Oral devices that apply positive end- expiratory pressure maintain the patency of the airway during exhalation
  78. • Maintaining adequate systemic hydration, enhanced by nebulization with saline,• Acetylcysteine delivered by nebulizer thins secretions• aerosolized recombinant human DNase (rhDNase) in patients with cystic fibrosis
  79. Surgery• Localised bronchiectasis• Proximal obstructive lesion• Massive hemoptysis• Recurrent infections
  80. Vicious loops Infection BronchialObstruction Inflammation Bronchiectasis
  81. Summarydiagnosis management• History • Treat bronchiectasis – Prior infections, exposures – Clearance – Time course – Antibiotics – Other manifestations? – Immune-modulation• Chest Imaging • Balance burden of disease vs – Define region, pattern burden of therapy• Sputum culture (Sputum, Symptoms, PFT’s, Weight, X-rays)• Determine causal disease • Other… – sweat testing – Underlying disease therapy – immune testing – serologic testing – Transplantation – Management of complications • Collapse, plugs, hemoptysis
  82. Antibiotics Vicious loop tobramycin Infection Bronchial Obstruction InflammationClearance Anti-inflammatoryAlbuterol, DNase, Inhaled steroidsTherapy vest Bronchiectasis
  83. BronchiectasisDilated airways with frequently thickened walls
  84. Bronchiectasis: ClinicalNote: Bronchiectasis may happen 2/2 COPD or may be a separate process with very similar symptomsClinical:• Cough (90 %)• Daily sputum production (76%)• Dyspnea (72%)• Hemoptysis (56%)• Recurrent pleurisy
  85. Pathophysiology2 Prerequisites:• Infectious insult• Impairment of drainage, airway obstruction, and/or a defect in host defense.
  86. Pathophys Continued• Infection:Bacterial, mycobacterial, esp. ABPA central airway bronchiectasis• Airway obstruction:intraluminal tumor, foreign body, lymph nodes, COPD• Immunodeficiency:ciliary dyskinesia, HIV, hypogammaglobulinemia, cystic fibrosis (obstruction and immunodef.)
  87. Characteristic central bronchiectasis 2/2 ABPA
  88. Note characteristic location in the upper lobes and superior segments oflower lobes
  89. Exacerbation: Etiology +RxColonization/infection:• Hemophilus• Pseudomonas• MAI• AspergillusVery difficult to distinguish colonization from acute infection with these bugs. Psuedomonas colonized more bronchiectasis on CT; increased number of hospitalizations vs H. flu colonizationEffect of sputum bacteriology on the quality of life of patients with bronchiectasis. Wilson CB; Jones PW; OLeary CJ; Hansell DM; Cole PJ; Wilson R Eur Respir J 1997 Aug;10(8):1754-60.Treatment:fluoroquinolone
  90. Prevention• Antibiotics-Controversial:Consider Macrolide TIWCipro qd X 7-14 D/ month• Bronchial Hygiene, physiotherapy, pulmonary rehab• ?bronchodilators, and steroids• Surgery
  91. CXR08/21
  92. CXR08/23
  93. CXR08/28
  94. CXR08/31
  95. HRCT08/31
  96. CXR09/01
  97. CXR09/09
  98. CXR09/15
  99. Bronchiectasis
  100. Introduction• Chronic daily cough w/ viscid sputum• Bronchial wall thickening and luminal dilation on CT• Prevalence varies – Associated w/ ↑age, female• Management – Infection control – ↑ bronchial hygiene – Surgical resection in selected p’t
  101. Pathophysiology• Permanent abnormal dilation and destruction of bronchial walls• Two factors – Infection – Impairment of drainage, airway obstruction, and/or defect in host defense• Biomarkers: inflammatory cells or 8-iso- prostaglandin F(2α) in sputum
  102. Etiology• Pulmonary infections – viral, mycoplasma, TB, MAC• Airway obstruction• Defective host defenses• ABPA (allergic bronchopulmonary aspergillosis)• Rheumatic and other systemic dz – RA, Sjogren’s syndrome – Ulcerative colitis• Dyskinetic cilia• Cystic fibrosis ← rare in TW• Cigarette smoking?
  103. Diagnostic Evaluation• CBC w/ differential• Ig quantification• Sputum culture and smear – bacteria, mycobacteria, fungi• CXR – Linear atelectasis, tram track, ring shadow, irregular peripheral opacities• HRCT: defining test• PFT
  104. CXR of Bronchiectasis
  105. CXR of Bronchiectasis Hansell DM - Radiol Clin North Am - 01-JAN-1998; 36(1): 107-28
  106. CXR of Bronchiectasis Hansell DM - Radiol Clin North Am - 01-JAN-1998; 36(1): 107-28
  107. HRCT• Sensitivitiy ~97%• Findings – Airway dilation – Lack of tapering of bronchi – Bronchial wall thickening – Mucopurulent plugs or debris – Cyst – Pneumonia, LAP, emphysema• Distributions – Upper lobe – Central distribution
  108. HRCT Radiol Clin N Am 43(2005) 513-542
  109. HRCT
  110. HRCT
  111. Management• Infection control• ↑ bronchial hygiene• Surgical resection in selected p’t
  112. Infection Control• Acute exacerbation – ↑viscous, dark sputum, lassitude, SOB, pleurisy – Fevers and chills generally absent – CXR rarely show new infiltrates – H. influenzae and P. aeruginosa – FQ is reasonable (eg. ciprofloxacin) for 7~10 days
  113. Prevention• Daily ciprofloxacin (500~1500mg) in 2~3 doses• Macrolide daily or three times weekly• Daily use of a high dose oral antibiotic, such as amoxicillin 3 g/day• Aerosolization of an antibiotic• Intermittent intravenous antibiotics
  114. Problematic Pathogen• Pseudomonas aeruginosa – Almost impossible to irradicate – Wilson CB et al. • Reduced QoL • More extensive bronchiectasis on CT • Increased number of hospitalizations – Ciprofloxacin quickly develops resistance
  115. Bronchial Hygiene• Oral hydration• Nebulization – Normal saline – Acetylcyteine – Recombinant DNAase – Hypertonic saline, mannitol, dextran, lactose• Physiotherapy – Chest percussion – Prone position• Bronchodilator? Steroid? NSAID?
  116. Surgical Intervention• Removal of the most involved segments• Most common: middle and lower lobe resecton• Hemoptysis: – Bronchial a. embolization• Lung transplantation
  117. Lung Transplantation• Overall 1-year survival : 68% (54-91%)• Overall 5-year survival : 62% (41-83%)• Subgroup – SLTX : 1 yr survival 57% (20%-94%) n=4 • Mean FEV1 : 50% predicted (34%-61%), • Mean FVC : 53% predicted (46-63%) – 2 lungs : 1 yr survival 73% (51-96%) n = 10 • Mean FEV1 : 73% predicted (58%-97%), • Mean FVC : 68% predicted (53%-94%)
  118. Chapter 14 Bronchiectasis C A B E D Figure 14–1. Bronchiectasis. A, Varicose bronchiectasis. B, Cylindrical bronchiectasis. C, Saccularbronchiectasis. Also illustrated are excessive bronchial secretions (D) and atelectasis (E), which are both common anatomic alterations of the lungs in this disease.
  119. Three Forms of Bronchiectasis• Varicose bronchiectasis• Cylindrical bronchiectasis• Saccular bronchiectasis
  120. Anatomic Alterations of the Lungs• Chronic dilation and distortion of bronchial airways• Excessive production of often foul-smelling sputum• Smooth muscle constriction of bronchial airways• Hyperinflation of alveoli (air-trapping)• Atelectasis, consolidation, and parenchymal fibrosis• Hemorrhage secondary to bronchial arterial erosion
  121. Etiology• Acquired bronchiectasis – Recurrent pulmonary infection – Bronchial obstruction• Congenital bronchiectasis – Kartagener’s syndrome – Hypogammaglobulinemia – Cystic fibrosis
  122. Overview of the Cardiopulmonary Clinical Manifestations Associated with BRONCHIECTASISThe following clinical manifestations result fromthe pathophysiologic mechanisms caused (oractivated) by Atelectasis (see Figure 9-12),Consolidation (see Figure 9-8), Bronchospasm(see Figure 9-10), and Excessive BronchialSecretions (see Figure 9-11)—the major anatomicalterations of the lungs associated withbronchiectasis (see Figure 14-1).
  123. Figure 9-7. Atelectasis clinical scenario.
  124. Figure 9-8. Alveolar consolidation clinical scenario.
  125. Figure 9-9. Increased alveolar-capillary membrane thickness clinical scenario.
  126. Figure 9-10. Bronchospasm clinical scenario (e.g., asthma).
  127. Figure 9-11. Excessive bronchial secretions clinical scenario.
  128. General Management of BronchiectasisGeneral treatment includes:• Controlling pulmonary infections• Controlling airway secretions• Preventing complications
  129. General Management of BronchiectasisRespiratory care treatment protocols• Oxygen therapy protocol• Bronchopulmonary hygiene therapy protocol• Hyperinflation therapy protocol• Aerosolized medication protocol• Mechanical ventilation protocol
  130. General Management of BronchiectasisOther medications commonly prescribedby the physician• Xanthines• Expectorants• Antibiotics
  131. BRONCHIECTASISRADIOLOGICAL FEATURES
  132. BRONCHIECTASIS THE CHEST RADIOGRAPH• Often normal if not severe• Too many white lines extending from the hila = tram-tracks• Elongated (tubular) opacities (white)• Small circles containing air (black) or fluid and air (air-fluid level)
  133. MILD BRONCHIECTASISNormal chest radiographpresents with hemoptysis
  134. MODERATE BRONCHIECTASIS- Coarse white linesextending out from hila
  135. TOO MANY WHITE LINES
  136. SEVERE BRONCHIECTASIS
  137. SEVERE BRONCHIECTASISCircle filledwith air
  138. SEVERE BRONCHIECTASIS
  139. RINGS (CYSTS) CONTAINING AIR-FLUID LEVELS
  140. BRONCHIECTASIS THE CT SCAN• Signet ring sign• Tram-tracks• String of beads• Circles filled with air or air and fluid• Tubular and branching opacities• Bronchi visible within 1 cm of the pleura• Scarring
  141. Normal pulmonary artery (pearl) Dilated bronchus (ring)SIGNET-RING SIGN
  142. Dilated bronchusBRONCHIECTASIS
  143. Bronchi visiblewithin 1 cm ofthe pleura String of beads BRONCHIECTASIS
  144. Destroyed lung (Scarring)BRONCHIECTASIS
  145. CAUSES OF BRONCHIECTASIS• Congenital• Acquired
  146. CONGENITAL CAUSES OF BRONCHIECTASIS• Cystic fibrosis• Immotile cilia syndrome
  147. CYSTIC FIBROSIS• Diffuse bronchiectasis• Most severe in the upper lobes
  148. CYSTIC FIBROSIS
  149. Worse in the upper lung zonesCYSTIC FIBROSIS
  150. IMMOTILE CILIA SYNDROME• Diffuse bronchiectasis• May have situs inversus (Kartagener’s syndrome
  151. BronchiectasisDextrocardia KARTAGENER’S SYNDROME
  152. BronchiectasisKARTAGENER’S SYNDROME
  153. KARTAGENER’S SYNDROME
  154. KARTAGENER’S SYNDROME Dextrocardia Dilated bronchus
  155. CAUSES OF ACQUIRED BRONCHIECTASIS• Post-infectious• Post-obstructive – aspirated foreign body – slow-growing tumour
  156. POST-INFECTIOUS BRONCHIECTASIS• Affects the part of the lung which was involved with pneumonia• Often diffuse as most commonly secondary to a viral pneumonia
  157. POST-OBSTRUCTIVE BRONCHIECTASIS• Focal or localized because only distal to the obstructing lesion• Dilated bronchi distal to obstruction filled with mucus instead of air
  158. FOCAL BRONCHIECTASIS
  159. FOCAL BRONCHIECTASIS Branching tubular opacities
  160. FOCALBRONCHIECTASIS
  161. Focal bronchiectasisdue to slow-growingendobronchial tumour
  162. Focal bronchiectasisdue to slow-growingendobronchial tumour Dilated bronchi filled with mucus Branching tubular opacity
  163. Dilated bronchifilled with mucusinstead of air due toproximal obstruction
  164. PULMONARY EMBOLISM RADIOLOGICAL FEATURES
  165. Lung - PathologyAcute Lung Injury Pulmonary edema ARDS (Diffuse alveolar damage) Acute interstitial pneumonia
  166. • Pulmonary Edema• 1. Hemodynamic disturbances(↑ capillary hydrostatic pressure), MCC- LVF, Heart failure cells• 2. ↓ oncotic pressure (Hypoalbuminemia)  Renal failure, Malnutrition, Cirrhosis• 3. Microvascular injury - ↑ capillary permeability, – Seen in ARDS following a) Infection -viral pneumonia b) Gases, Aspiration, Drugs--heroin, Paraquat (herbicide) c) Shock, Trauma, Sepsis, DIC• ARDS:• Also known as ***Diffuse Alveolar Damage (DAD)  pulmonary edema (protein rich)  hyaline membrane disease  hypoxemia (refractory to oxygen Rx )• Mortality in 50% of cases• Clinical Manifestations: respiratory insufficiency, Cyanosis, arterial hypoxemia  multi-organ failure• Acute interstitial pneumonia Cause is unknown (Unlike ARDS) but Radiographic and Pathologic features, mortality similar to ARDS
  167. • Bronchiectasis• Infection + permanent dilatation of bronchi• Causes:• infections and causes of bronchial obstruction (FB, mucus plugs, tumors, sequestrations, cystic fibrosis)• immotile cilia (Kartagener’s )syndrome (Bronchiectasis, dextrocardia -situs inversus, chronic sinusitis, and infertility)• Clinically:• Chronic cough, productive of purulent sputum• Dyspnea and orthopnea in severe cases• later obstructive respiratory insufficiency & Corpulmonale
  168. Bronchiectasis Gross• Distended peripheral bronchi (Due to weakening of wall)
  169. Bronchiectasis Gross
  170. PRIMARY ANTIBODY DEFICIENCY (PAD) & BRONCHIECTASIS (UKPIN / BTS GUIDELINES)
  171. BRONCHIECTASISA destructive lung disease characterised by: Abnormal & permanent dilatation of medium sized bronchi An associated, persistent and variable inflammatory process producing damage to bronchial elastic and muscular elements
  172. PATHOLOGY Neutrophil proteases (acute infection in a normal or compromised host)  Epithelial injury + Structural protein damage  Damaged, dilated airway Mucous retention / chronic, recurrent infection Ongoing inflammation / tissue damage / repair
  173. BRONCHIECTASIS - aetiology Infection - pertussis, influenza, measles, TB, necrotising peumonia Bronchial obstruction - mucoid impaction, ABPA Congenital anatomical lung abnormality Inherited disorders - ciliary dysfunction - cystic fibrosis - alpha-1 AT deficiency• Undefined (29 - 49%)
  174. BRONCHIECTASIS OF UNDEFINED REF. AETIOLOGY NOS. ANALYTE % age ABN. Hilton & Doyle 53 IgG/A/M 0 - 1978 Murphy et al 23 IgG/A/M, Gsub 0 - 1984 Barker et al 30 IgG/A/M, Gsub 37 Panhypoγ (9/30) 1987 IgM (2/30) De Gracia et al 65 Gsub, Hib 48 IgG2 1996 Hib (10/19) Hill et al 89 Gsub 6 IgG4 1998 Stead et al 56 IgG/A/M, Gsub 23 IgG4 2002 Hib, Pneum Pneum (1/29)
  175. BRONCHIECTASIS Pasteur et al. Am J Respir Crit Care Med (2000) 162, 1277-1284ASSOCIATION n %Idiopathic 80 53ABPA 11 7PAD 11 7Neutrophil defect 1 <1Rheumatoid disease 4 3Ulcerative colitis 2 <1Ciliary dysfunction 3 1.5Young’s syndrome 5 3Cystic fibrosis 4 3Post-infectious 44 9Aspiration/reflux 6 4Other defineable 2 <1
  176. BRONCHIECTASIS in PADCVID• 53% (Hausser et al 1983)• 44% (Watts et al 1986)• 18% (Hermazewski & Webster 1993)• 20% (UK PAD Audit 1993-96)• 27% (‘chronic lung disease’) (Cunningham Rundles 1999)• 58% (Garcia 2001)• 43% (Busse et al 2002)XLA• 7% (Hermazewski & Webster 1993)• 12% (UK PAD Audit 1993-96)• 20% (Quartier et al 1999)
  177. RESPIRATORY INFECTIONS Figure 2 Types of infection in the 37 patients receiving immunoglobulin replacement treatment. The numbers of each type of infection are listed by each chart section.
  178. DIAGNOSTIC DELAY Average: diagnosis - 6.3 years treatment - additional 3.9 Diagnostic delay > 2 years:  risk of bronchiectasis sinusitis iron deficiency (UK PAD Audit 1993-96) 3 Strongest predictor of chronic pulmonary disease in treated patients is established lung disease at time of presentation n= XLAx10, CVIDx12 IMIg x 18, IVIg x 3, FFP x 1 (all + daily antibiotic) (Sweinberg et al 1991) 3
  179. UK PAD AUDIT 1993-96Development of bronchiectasis following diagnosis:<1980 1981-87 >1988 77% 70% 42%
  180. CHRONIC LUNG DISEASE in PAD Damage sustained prior to active treatment and/or Continued inflammation despite treatment
  181. AIMSDefine evidence-based guidelines relevant to: investigation level appropriate to screen for significant antibody deficiency in all patients with bronchiectasis diagnosis & management of bronchiectasis complicating primary antibody deficiency
  182. GUIDELINES Simple  Valid Evidence-based  Reproducible Consistent with  Reliable existing, recognised standards • Involving & representative of Realistic key disciplines Explicit  Clinically applicable Clear & well documented  Clinically flexible Credible & widely supported  Scheduled for review Results orientated (outcomes)
  183. GUIDELINESLITERATURE REVIEW DATABASES  Ovid Online Collection Meta-analyses - Medline, preMedline Systematic reviews - CINAHL, EMBASE RCTs - Journals@ovid  EBM Reviews Longitudinal studies - Cochrane Systematic Reviews Case control/cohort studies - Cochrane Controlled Trials Case reports/case series - Effectiveness Reviews Abstracts - ACP Journal Club Expert opinions  Allied & Complementary Medicine  Specialty Contacts
  184. GUIDELINESEVIDENCE RECOMMENDATION 1 A 2 B 3 C 4 D (Good Practice Points)SIGN, RCPCH, BTS
  185. DIAGNOSISPRIMARY ANTIBODY DEFICIENCY Humoral abnormalities are common in bronchiectasis 3 Respiratory Physician + Immunologist 4 Diagnosis of significant antibody deficiency should entail use of established and widely accepted criteria: 4 - Primary Immunodeficiency Diseases. Report of an IUIS Scientific Group Clinical & Experimental Immunology 1999 (118), Suppl 1:1-34 - Diagnostic Criteria for Primary Immunodeficiencies. Clinical Immunology 1999 (93), 190-197 - Practice parameters for the Diagnosis & Management of Immunodeficiency. Annals of Allergy, Asthma & Immunology 1996 (76), 282-294
  186. PFTs- Reversible/irreversible bronchial obstruction- Granulomatous disease etc. Correlate poorly with Radiology (bronchiectasis) 3 - Pulmonary abnormalities in patients with primary hypogammaglobulinaemia Kainulainen et al. Jounal of Allergy & Clinical Immunology (1999) 104, 1031-1036 - Pulmonary manifestations of hypogammaglobulinaemia Dukes et al. Thorax (1978) 33, 603-607 - Radiologic findings of adult primary immunodeficiency disorders: contribution of CT Obregon et al. Chest (1994) 106, 490-495• Static volumes/flow-volume loops
  187. RADIOLOGY - CXR Bronchiectasis - vessel ‘crowding’ - loss of vessel markings - tramline/ring shadows - cystic lesions/ air-fluid levels - evidence of TB Poor:  diagnostic sensitivity  monitoring of progression 3
  188. RADIOLOGY - HRCT - bronchial dilatation - bronchial wall thickening - classification (pathology)  sensitivity (97%) > CXR 3  chromosomal radiosensitivity - plain CXR (x 3 days background) - HRCT: x 30-40 - conventional CT: x 200 • ? routine baseline • ? (a)symptomatic monitoring
  189. UNSUSPECTED DISEASE (Clinical v CXR v HRCT) Bronchiectasis in Hypogammaglobulinaemia - A Computed Tomography assessment. Curtin et al. Clinical Radiology (1991) 44, 82-84 Radiologic Findings of Adult primary Immunodeficiency Disorders. Obregon et al. Chest (1994)106, 490-495 Chest High Resolution CT in Adults with Primary Humoral Immundeficiency. Feydy et al. British Journal of Radiology (1996) 69, 1108-1116 Clinical Utility of High-Resolution Pulmonary Computed Tomography in Children with Antibody Deficiency. Manson et al. Pediatric Radiology (1997) 27, 794-798 The Value of Computed Tomography in the Diagnosis & Management of Bronchiectasis. Pang et al. Clinical Radiology (1989) 40, 40-44 Review Article: Imaging in Bronchiectasis. Smith et al. British Journal of Radiology (1996) 69, 589-593 3
  190. RADIOLOGYKainulainen et al 1999 CVID x 18, XLA x 4  3 year follow-up  CXR HRCT Disease progression (5)Bronchiectasis 3 16 Serum IgG Case No T=0 T=36 1 9.9 10.0 2 4.6 6.1 8 3.7 5.1 10 3.7 4.9 21 3.1 5.7
  191. RADIOLOGY - HRCTRCP Specialty Specific Standards‘Fit’ patients…….CT scanning should be undertaken ina minority of patients but usually not more than once ayear or if respiratory function tests or symptomsdeteriorateJCIA November 2001 4
  192. MANAGEMENT – GENERAL ISSUES Shared Care (Immunologist/Respiratory Physician) optimal 4 Bronchodilators (reversible airflow obstruction) Mucolytics - insufficient evidence to evaluate routine use (Cochrane Database of Systematic Reviews. 3, 2003) Physical therapy - insufficient evidence to support or refute usage (Cochrane Database of Systematic Reviews. 3, 2003) Anti-inflammatory agents
  193. REPLACEMENT THERAPY Risk/benefit assessment 4 IV/Sc routes optimal 2  pulmonary infections in XLA/CVID (v untreated) 2 Optimal dosing/frequency/serum IgG level not established Tailor route/dose/infusion frequency 3--------------------------------------------------------------- Maintain IgG >5g/l 2 Paediatric target: mid reference range 4 IgG: >8g/l   infection (v 5g/l, XLA, children) 3 9.4 g/l   infection (v 6.5g/l, XLA/CVID, children/adults) 3 High v standard doses   infections (no. & duration) 2  days hospitalised  serum IgG Insidious disease progression despite ‘adequate’ replacement 3
  194. REPLACEMENT THERAPY High dose v low dose: secondary outcome, pulmonary function Eijkhout et al 2001 (randomised, double-blind, multicentre, crossover, n=43) High dose (mean trough IgG 9.4 g/l): PEFR 37.3 l/min Standard dose (mean trough IgG 6.5 g/l): PEFR 11.4 l/min NS Roifman & Gelfand 1988 (ramdomised, crossover, n=12) High dose   FVC & FEV1 p<0.01 Roifman et al 1987 (randomised, crossover, n=12) Mean FEV1 & FVC high dose phase v low dose phase p<0.01 Bernatowska et al 1987 (two-dose, crossover, non-randomised, n=13) High dose  Max. expiratory flow & FEV1 NA
  195. ACUTE INFECTIONMICROBIOLOGY Culture & sensitivity routinely in acute setting 3 Value unclear in chronic situation - confirm original pathogen - ? emerging resistance - additional pathogensANTIBIOTICS Effectiveness established in exacerbations (bronchiectasis) 2 Higher doses for longer periods 4 Local treatment protocols 4
  196. ANTIBIOTIC PROPHYLAXIS Chronic bronchitis - no place in routine treatment (Cochrane Database of Systematic Reviews. 3, 2003) Cystic fibrosis benefits - principally staphylococci - infancy  3/6 years - ? older children/adults - ? > 3years treatment (The Cochrane Library, Oxford. 2, 2003) (Cochrane Database of Systematic Reviews. 3, 2003)• Bronchiectasis - limited meta-analysis (6 RCTs) - marginal benefit / cautious support (Evans et al. Thorax 2001)
  197. ANTIBIOTIC PROPHYLAXIS No robust data v placebo No substantial data v (or additional to) IVIg/SCIg (Silk et al. 1990) ? Single intervention in mild antibody deficiency - not in more severe phenotypes / tissue damage Papworth protocol: consider if: > 3 exacerbations / year 4 radiological / PFT deterioration ? Eradication/clean-up therapy prior to prophylaxis - no clear evidence of benefit in antibody deficiency + structural lung damage Development of local protocols for management of infections (esp. with Primary Care) and initiating prophylaxis 4
  198. ANTIBIOTIC PROPHYLAXIS Percentage of sputum samples growing pathogens before and after prophylactic ciprofloxacin 70 60 50 all pathogens 40 30 H. Infl (all % isolates) 20 H Infl. (resistant 10 to ciprofloxacin) 0 Prior to On ciprofloxacin ciprofloxacin(Heelan et al., ESID 2002)
  199. SURGERY Diagnostic delay > 2 years:  need for surgical procedures Adequate treatment:  lobectomy/pneumonectomy by 95% (UK PAD Audit 1993-96) 3 Important treatment option with favourable outcomes especially in focal bronchiectasis (Cohen et al 1994, Mansharamani & Koziel 2003) 3
  200. QUESTIONS / ISSUES HRCT in routine screening & monitoring Radiological changes a primary therapeutic target - Does HRCT modify our current assumptions about criteria for adequate treatment of antibody deficiency disorders? Correct level of Ig treatment - arbitrary target serum level (evidence) or individualised (clinical + HRCT factors) - single intervention universally applicable in all patients (probably not) - higher doses: expense, complications, limited commodity Roles of: antibiotics anti-inflammatory agents bronchodilators aids to airway clearance Role of co-factors (e.g. 1AT) Selective IgA deficiency
  201. PIN GUIDELINES Identify need for focused clinical research Encourage debate and discussion Reflect uncertainties in the field Proscriptive as necessary, flexible where possible

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