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13 bronchiectasis-dr khinchi
- 1. Bronchiectasis Dr YogRaj Khinchi
- 2. Chronic Bronchitis Definition 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 astate of increased mucus production, with or without adequate mucociliary transport, that requires cough for clearance of airway secretions.
- 4. Airway Stimulus/Injury Increasing Secretion Production Increased Effective Secretion Mucociliary Cough Inspissation Airway Clearance Obstruction Recurrent/Persistent
- 5. Chronic Bronchitis: Underlying Conditions 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. Bronchiectasis Pathogenesis Airway Injury + Secretion Stimuli Secretion Stasis Infection Airway Destruction + Airway Dilation
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- 9. CF vs. IdiopathicBronchiectasis CYSTIC IDIOPATHIC FIBROSIS BRONCHIECTATIS Multi-organ Involvement Sinopulmonary Involvement Diffuse Multifocal Pseudomones Mouth Flora Tenacious Secretions Mucoid/Coughable 50% Bronchodilator 50% Bronchodilator Response Response Progressive Outcome Variable Outcome
- 10. 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)
- 11. Bronchiectasis • Irreversible, abnormaldilatation 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
- 12. Pathogens associated withexacerbations and disease progression in bronchiectasis • Non-tuberculosis Haemophilus influenzae mycobacteria Haemophilus parainfluenzae – M. avium complex (MAC) Pseudomonas aeruginosa – M. kansasii – M. chelonae – M. fortuitum Streptococcus pneumoniae – M. malmoense Moraxella catarrhalis – M. xenopi Staphylocccus aureus • Aspergillus-related Stenotrophomonas maltophilia Gram-negative enterobacter disease
- 13. Causes / associationsof 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
- 14. Evidence for dysregulatedimmunity 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. Evidence for dysregulatedimmunity 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
- 16. Bronchiectasis associated withincreased susceptibility to specific pathogens • Non-tuberculosis Haemophilus influenzae mycobacteria Haemophilus parainfluenzae – M. avium complex (MAC) Pseudomonas aeruginosa – M. kansasii – M. chelonae – M. fortuitum Streptococcus pneumoniae – M. malmoense Moraxella catarrhalis – M. xenopi Staphylocccus aureus • Aspergillus-related Stenotrophomonas maltophilia Gram-negative enterobacter disease
- 17. Evidence for dysregulatedimmunity 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
- 18. Bronchiectasis associated with autoimmune disease • Rheumatoid arthritis • Systemic lupus erythematosus • Relapsing polychondritis • Inflammatory bowel disease - Ulcerative colitis and Crohn’s disease
- 19. Evidence for dysregulatedimmunity 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
- 20. Suppurative Lung Disease •Bronchiectasis • Lung abcess • Empyema
- 21. Brochiectasis • Def:destructive lungdisease :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.
- 22. Notes • Bronchiectasis isof 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).
- 23. Pathogenesis • Vicious cycle: infectious insult & impaired drainage &/or defect in host defence • Role of positive : genetic susceptibilty family history
- 24. Notes • Normally, inhealthy 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.
- 25. Classification Spectrum: • 1.follicular cylindrical:transmuralinflammation, 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.
- 26. Notes • Another typeof 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.
- 27. Causes of bronchiectasis Congenital:elasticbronchial wall def. other congenital abnormalities,lung sequestration Mechanical bronchial obstruction: Intrinsic and extrinsic: Lymph node or scaring :post-TB Foreign body: selective lobes Child vs adult
- 28. Notes • Causes: 1- congenital:infetus, 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).
- 29. Cont’d Notes - TBproduces 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.
- 30. *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
- 31. Cont’d Causes *Mucociliary clearancedefects -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
- 32. Notes • In cysticfibrosis, 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.
- 33. Cont’s Notes • ABPAcauses hyper immune activity. • Atypical mycobacterial infection and uncontrolled asthma can cause abnormal cilia.
- 34. *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.
- 35. Clinical manifestation ofcystic fibrosis Primary • Bronchiectasis *Nasal polyps • Bronchiolitis, bronchitis * Cirrhosis • Infertility * Meconium ileus • Pancreatic insufficiency • Pneumonia • Salt loss
- 36. Secondary *Atelectasis * Allergicbronchopulmonary *Clubbing aspergillosis *Malabsorption * cholelithiasis *Heat prostration * Cor pulmonale *Hypochloraemic * Conductive hearing loss hypokalaemic alkalosis * Diabetes mellitus
- 37. Diagnosis of Bronchiectasis •1.History: usually :muco-purulent sputum, cough. occasionally recurrent hemoptysis, recurrent fever Less specific : dyspnea, wheeze ,pleuritic pain • 2.Examination: Early: normal nowadays:clubbing 3%,crackles70% rarely :Core pulmonale( right ventricular hypertrophy due to pulmonary hypertension),CCF.
- 38. Notes The most importantsign 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.
- 39. Investigation • Sputum: gramstain,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
- 40. 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
- 41. Notes • Bronchogram: doa 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.
- 43. Notes • The previousslide 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.
- 45. Notes • The previousCT was taken for the same patient during full expiration, which is better than the full inspiratory CT.
- 46. Cont’d investigation • UGI Endoscopy • Anti protease level • Sweat test • Genetic studies • Rh. Factor
- 47. 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.
- 48. Management • Prevention: • Medicaltreatment: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)
- 49. Notes • Management ofbronchiectasis 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).
- 50. Cont’d management Physiotherapy Corner stone:Posturaldrainage, hydration ,nebulized saline *Surgery: .Massive haemoptysis: > 600ml/day. .Localized troublesome resectable bronchiectasis .Palliative for important stump.
- 51. Notes - physiotherapy: teachthe patient how to drain his lung (lungs should be drained daily). - Surgery: is indicated: 1- when there is severe hemoptysis 2- if one lobe causing problems to the patient, so you resect it to prevent damage to other lobes 3- 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.
- 52. Cont’d management Haemoptysis: *Mild:antibiotic *Severe: surgery or bronchial artery embolization Lung 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.
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- 54. “Chronic dilation ofthe bronchi marked by fetid breath and paroxysmal coughing, with the expectoration of mucopurulent matter.”
- 57. Morphological types • Cylindricalor tubular bronchiectasis • Varicose • saccular or cystic bronchiectasis
- 58. Bronchiectasis • Causes andpathogenesis • Microbiology /common pathogens • Therapeutic Goals
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- 61. Bronchiectasis Worldwide, infection isthe primary cause – M. Tuberculosis – Childhood illnesses • Rubeola, B. Pertussis
- 62. Childhood Infections M. Tb. Infection Inflammation Bronchiectasis Altered development
- 63. Inflammation Characteristics across etiologies: -Persistent - Neutrophil dominant - Pro-inflammatory cytokines (IL- 8, IL-1, TNF-a) - Low anti-inflammatory cytokines (IL-10)
- 64. Airway Damage Failure to Resolve Inflammation – Inappropriate inflammation e.g. ABPM, CF (?) – Impaired clearance of stimuli Bacteria, mucus, toxins
- 65. 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‡
- 66. Childhood Infections M. Tb. Infection Inflammation* Impaired Clearance Bronchiectasis Altered development
- 67. Impaired Clearance Altered ciliaryFunction PCD, smoking, CFTR, Young’s Mucus rheology CFTR, Mucoid Ps. A Dilated or obstructed airways Impaired cough, foreign bodies, aspiration
- 68. Impaired Immunity Ineffective inflammation Acquired Chemo-immunomodulation Congenital/innate
- 69. HIV/AIDS Chemo-Immunosuppression anti-TNF, MTX, anti-neoplastics Childhood Infections M. Tb. Innate Deficiency CGD, CVID, Ig’opathy (IFN) Infection Inflammation Impaired Clearance foreign body, CF Bronchiectasis Altered development
- 70. HIV/AIDS Chemo-Immunosuppression Childhood Infections M. Tb. Innate Deficiency Infection Auto-Immune Impaired Clearance RA, Sjogren’s, IBD Inflammation ABPM Bronchiectasis Altered development
- 71. HIV/AIDS Chemo-Immunosuppression Childhood Infections M. Tb. Innate Deficiency Infection Auto-Immune Inflammation Impaired Clearance ABPM Bronchiectasis Altered development
- 72. HIV/AIDS Chemo-Immunosuppression Childhood Infections M. Tb. Innate Deficiency Infection Auto-Immune Inflammation Impaired Clearance ABPM Bronchiectasis Altered development
- 73. Bronchiectasis Epidemiology and Etiology Who? Patients - with altered immune system - with persistent respiratory symptoms Why? Persistent inflammation in the respiratory system
- 74. Bronchiectasis Therapy Decrease inflammation antibiotics clearance – Flutter, IPPV, Vest, Bronchodilators, hypertonic saline (anti-inflammatory chemotherapy) – Steroids, macrolides, interferon-gamma, ibuprofen (surgical resection)
- 75. When to suspectbronchiectasis? Chronic cough, sputum Coarse rales Persistent respiratory symptoms Recurrent pneumonia Progressive obstructive lung disease Funny bugs
- 76. 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
- 77. Bronchiectasis Therapy Antibiotics Episodic or suppressive antibiotics? Yes. Selective pressure vs. suppression of damage
- 78. Bronchiectasis Therapy Antibiotics Con • Pro Select resistance – Decrease inflammation Cost Side effects – Slow progression adherence difficult (e.g. – Eradication? Huong et al.)
- 79. Bronchiectasis Therapy Antibiotics Con • Pro Select resistance – Decrease inflammation Cost Side effects – Slow progression adherence difficult (e.g. – Eradication? Huong et al.)
- 80. Bronchiectasis Therapy Antibiotics when to use? No: • Yes: Minimal disease without – Evidence of organism Patient Intolerant exacerbation “Unaffordable” – Progressive decline – “Frequent exacerbator” – Active inflammation (?)
- 81. Bronchiectasis Therapy Decrease inflammation Clearance Immunomodulatory chemotherapy proposed therapies: – Steroids – Macrolides, tetracyclines – interferon-gamma – ibuprofen
- 82. 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
- 83. • Maintaining adequatesystemic hydration, enhanced by nebulization with saline, • Acetylcysteine delivered by nebulizer thins secretions • aerosolized recombinant human DNase (rhDNase) in patients with cystic fibrosis
- 84. Surgery • Localised bronchiectasis • Proximal obstructive lesion • Massive hemoptysis • Recurrent infections
- 85. Vicious loops Infection Bronchial Obstruction Inflammation Bronchiectasis
- 86. Summary diagnosis 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
- 87. Antibiotics Vicious loop tobramycin Infection Bronchial Obstruction Inflammation Clearance Anti-inflammatory Albuterol, DNase, Inhaled steroids Therapy vest Bronchiectasis
- 88. Bronchiectasis Dilated airways withfrequently thickened walls
- 89. Bronchiectasis: Clinical Note: Bronchiectasismay happen 2/2 COPD or may be a separate process with very similar symptoms Clinical: • Cough (90 %) • Daily sputum production (76%) • Dyspnea (72%) • Hemoptysis (56%) • Recurrent pleurisy
- 90. Pathophysiology 2 Prerequisites: • Infectiousinsult • Impairment of drainage, airway obstruction, and/or a defect in host defense.
- 91. 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.)
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- 98. Note characteristic locationin the upper lobes and superior segments of lower lobes
- 99. Exacerbation: Etiology +Rx Colonization/infection: •Hemophilus • Pseudomonas • MAI • Aspergillus Very difficult to distinguish colonization from acute infection with these bugs. Psuedomonas colonized more bronchiectasis on CT; increased number of hospitalizations vs H. flu colonization Effect of sputum bacteriology on the quality of life of patients with bronchiectasis. Wilson CB; Jones PW; O'Leary CJ; Hansell DM; Cole PJ; Wilson R Eur Respir J 1997 Aug;10(8):1754-60. Treatment: fluoroquinolone
- 100. Prevention • Antibiotics-Controversial: Consider MacrolideTIW Cipro qd X 7-14 D/ month • Bronchial Hygiene, physiotherapy, pulmonary rehab • ?bronchodilators, and steroids • Surgery
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- 110. Introduction • Chronic dailycough 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
- 111. Pathophysiology • Permanent abnormaldilation 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
- 112. 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?
- 113. Diagnostic Evaluation • CBCw/ differential • Ig quantification • Sputum culture and smear – bacteria, mycobacteria, fungi • CXR – Linear atelectasis, tram track, ring shadow, irregular peripheral opacities • HRCT: defining test • PFT
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- 115. CXR of Bronchiectasis Hansell DM - Radiol Clin North Am - 01-JAN-1998; 36(1): 107-28
- 116. CXR of Bronchiectasis Hansell DM - Radiol Clin North Am - 01-JAN-1998; 36(1): 107-28
- 117. 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
- 118. HRCT Radiol Clin N Am 43(2005) 513-542
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- 121. Management • Infection control •↑ bronchial hygiene • Surgical resection in selected p’t
- 122. Infection Control • Acuteexacerbation – ↑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
- 123. 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
- 124. Problematic Pathogen • Pseudomonasaeruginosa – Almost impossible to irradicate – Wilson CB et al. • Reduced QoL • More extensive bronchiectasis on CT • Increased number of hospitalizations – Ciprofloxacin quickly develops resistance
- 125. Bronchial Hygiene • Oralhydration • Nebulization – Normal saline – Acetylcyteine – Recombinant DNAase – Hypertonic saline, mannitol, dextran, lactose • Physiotherapy – Chest percussion – Prone position • Bronchodilator? Steroid? NSAID?
- 126. Surgical Intervention • Removalof the most involved segments • Most common: middle and lower lobe resecton • Hemoptysis: – Bronchial a. embolization • Lung transplantation
- 127. Lung Transplantation • Overall1-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%)
- 128. Chapter 14 Bronchiectasis C A B E D Figure 14–1. 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.
- 129. Three Forms ofBronchiectasis • Varicose bronchiectasis • Cylindrical bronchiectasis • Saccular bronchiectasis
- 130. Anatomic Alterations ofthe 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
- 131. Etiology • Acquired bronchiectasis – Recurrent pulmonary infection – Bronchial obstruction • Congenital bronchiectasis – Kartagener’s syndrome – Hypogammaglobulinemia – Cystic fibrosis
- 132. Overview of theCardiopulmonary Clinical Manifestations Associated with BRONCHIECTASIS The following clinical manifestations result from the pathophysiologic mechanisms caused (or activated) by Atelectasis (see Figure 9-12), Consolidation (see Figure 9-8), Bronchospasm (see Figure 9-10), and Excessive Bronchial Secretions (see Figure 9-11)—the major anatomic alterations of the lungs associated with bronchiectasis (see Figure 14-1).
- 133. Figure 9-7. Atelectasisclinical scenario.
- 134. Figure 9-8. Alveolarconsolidation clinical scenario.
- 135. Figure 9-9. Increasedalveolar-capillary membrane thickness clinical scenario.
- 136. Figure 9-10. Bronchospasmclinical scenario (e.g., asthma).
- 137. Figure 9-11. Excessivebronchial secretions clinical scenario.
- 138. General Management of Bronchiectasis General treatment includes: • Controlling pulmonary infections • Controlling airway secretions • Preventing complications
- 139. General Management of Bronchiectasis Respiratory care treatment protocols • Oxygen therapy protocol • Bronchopulmonary hygiene therapy protocol • Hyperinflation therapy protocol • Aerosolized medication protocol • Mechanical ventilation protocol
- 140. General Management of Bronchiectasis Other medications commonly prescribed by the physician • Xanthines • Expectorants • Antibiotics
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- 142. 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)
- 143. MILD BRONCHIECTASIS Normal chestradiograph presents with hemoptysis
- 144. MODERATE BRONCHIECTASIS - Coarsewhite lines extending out from hila
- 145. TOO MANY WHITELINES
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- 149. RINGS (CYSTS) CONTAININGAIR-FLUID LEVELS
- 150. 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
- 151. Normal pulmonary artery (pearl) Dilated bronchus (ring) SIGNET-RING SIGN
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- 153. Bronchi visible within 1cm of the pleura String of beads BRONCHIECTASIS
- 154. Destroyed lung (Scarring) BRONCHIECTASIS
- 155. CAUSES OF BRONCHIECTASIS •Congenital • Acquired
- 156. CONGENITAL CAUSES OF BRONCHIECTASIS • Cystic fibrosis • Immotile cilia syndrome
- 157. CYSTIC FIBROSIS • Diffusebronchiectasis • Most severe in the upper lobes
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- 159. Worse in the upper lung zones CYSTIC FIBROSIS
- 160. IMMOTILE CILIA SYNDROME •Diffuse bronchiectasis • May have situs inversus (Kartagener’s syndrome
- 161. Bronchiectasis Dextrocardia KARTAGENER’S SYNDROME
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- 164. KARTAGENER’S SYNDROME Dextrocardia Dilated bronchus
- 165. CAUSES OF ACQUIRED BRONCHIECTASIS • Post-infectious • Post-obstructive – aspirated foreign body – slow-growing tumour
- 166. POST-INFECTIOUS BRONCHIECTASIS • Affects the part of the lung which was involved with pneumonia • Often diffuse as most commonly secondary to a viral pneumonia
- 167. POST-OBSTRUCTIVE BRONCHIECTASIS • Focal or localized because only distal to the obstructing lesion • Dilated bronchi distal to obstruction filled with mucus instead of air
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- 169. FOCAL BRONCHIECTASIS Branching tubular opacities
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- 171. Focal bronchiectasis due toslow-growing endobronchial tumour
- 172. Focal bronchiectasis due toslow-growing endobronchial tumour Dilated bronchi filled with mucus Branching tubular opacity
- 173. Dilated bronchi filled withmucus instead of air due to proximal obstruction
- 174. PULMONARY EMBOLISM RADIOLOGICAL FEATURES
- 175. Lung - Pathology AcuteLung Injury Pulmonary edema ARDS (Diffuse alveolar damage) Acute interstitial pneumonia
- 176. • 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
- 177. • 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
- 178. Bronchiectasis Gross • Distended peripheral bronchi (Due to weakening of wall)
- 179. Bronchiectasis Gross
- 180. PRIMARY ANTIBODY DEFICIENCY (PAD) & BRONCHIECTASIS (UKPIN / BTS GUIDELINES)
- 181. BRONCHIECTASIS A destructive lungdisease characterised by: Abnormal & permanent dilatation of medium sized bronchi An associated, persistent and variable inflammatory process producing damage to bronchial elastic and muscular elements
- 182. 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
- 185. 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%)
- 186. 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)
- 187. BRONCHIECTASIS Pasteur et al. Am J Respir Crit Care Med (2000) 162, 1277-1284 ASSOCIATION n % Idiopathic 80 53 ABPA 11 7 PAD 11 7 Neutrophil defect 1 <1 Rheumatoid disease 4 3 Ulcerative colitis 2 <1 Ciliary dysfunction 3 1.5 Young’s syndrome 5 3 Cystic fibrosis 4 3 Post-infectious 44 9 Aspiration/reflux 6 4 Other defineable 2 <1
- 188. BRONCHIECTASIS in PAD CVID • 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)
- 189. 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.
- 191. 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
- 192. UK PAD AUDIT1993-96 Development of bronchiectasis following diagnosis: <1980 1981-87 >1988 77% 70% 42%
- 193. CHRONIC LUNG DISEASEin PAD Damage sustained prior to active treatment and/or Continued inflammation despite treatment
- 194. AIMS Define evidence-based guidelinesrelevant to: investigation level appropriate to screen for significant antibody deficiency in all patients with bronchiectasis diagnosis & management of bronchiectasis complicating primary antibody deficiency
- 195. 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)
- 196. GUIDELINES LITERATURE 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
- 197. GUIDELINES EVIDENCE RECOMMENDATION 1 A 2 B 3 C 4 D (Good Practice Points) SIGN, RCPCH, BTS
- 198. DIAGNOSIS PRIMARY 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
- 199. PFTs - Reversible/irreversible bronchialobstruction - 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
- 200. 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
- 201. 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
- 202. 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
- 203. RADIOLOGY Kainulainen et al1999 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
- 204. RADIOLOGY - HRCT RCPSpecialty Specific Standards ‘Fit’ patients…….CT scanning should be undertaken in a minority of patients but usually not more than once a year or if respiratory function tests or symptoms deteriorate JCIA November 2001 4
- 205. MANAGEMENT – GENERALISSUES 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
- 207. 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
- 208. 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
- 210. ACUTE INFECTION MICROBIOLOGY Culture & sensitivity routinely in acute setting 3 Value unclear in chronic situation - confirm original pathogen - ? emerging resistance - additional pathogens ANTIBIOTICS Effectiveness established in exacerbations (bronchiectasis) 2 Higher doses for longer periods 4 Local treatment protocols 4
- 211. 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)
- 212. 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
- 213. 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)
- 215. 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
- 216. 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
- 218. PIN GUIDELINES Identify need for focused clinical research Encourage debate and discussion Reflect uncertainties in the field Proscriptive as necessary, flexible where possible