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- 2. Definition Bronchiectasis - uncommon disease, most often secondary to an infectious process, that results in the
- 3. ERS guidelines for the management of adult bronchiectasis (Eva Polverino, Pieter C. Goeminne, Melissa J. European
- 4. Classification by etiology 1. Genetic disorders (cystic fibrosis, primary ciliary dyskinesia, alpha1-antitrypsin deficiency) 2. Post infectious
- 5. Classification by etiology 29% idiopathic 14% post-infective 15% - COPD (predominantly not numerous, local*) 7% asthma
- 6. Classification: by shape Assoc Prof Frank Gaillard, Radiopaedia.org . From the case rID: 8863
- 7. Classification by shape: Normal bronchus; no bronchoectases American Journal of Roentgenology > Volume 193, Issue 3
- 8. Cylindric bronchiectasis with lack of bronchial tapering Assoc Prof Frank Gaillard, Radiopaedia.org . From the case
- 9. varicose bronchiectasis with string-of-pearls appearance Assoc Prof Frank Gaillard, Radiopaedia.org . From the case rID: 8863
- 10. cystic bronchiectasis American Journal of Roentgenology > Volume 193, Issue 3 > BronchiectasisSeptember 2009, Volume 193,
- 11. Classification: etiology and location Focal (congenital bronchial atresia, extrinsic compression, extrabronchial malignancy, foreign body, broncholothiasis, airway
- 12. Figure 1 The Lancet 2018 392, 880-890DOI: (10.1016/S0140-6736(18)31767-7) Copyright © 2018 Elsevier Ltd Terms and Conditions
- 13. Components Neutrophilic inflammation – destruction of wall by elastases Ciliary disfunction (primary or secondary) – retention
- 14. Inflammation: neutrophilic Neutrophils recruitment acceleration: degradation of elastins; increase of neutrophilic proteolytic molecules, damage and structural
- 15. Importance of this mechanism for control the disease block of neutrophilic elastase: NE inhibitor AZD9668 in
- 16. Ciliary disfunction: primary and secondary Cilia Dysfunction in Lung Disease Ann E. Tilley,1,2 Matthew S. Walters,1
- 17. Genes Encoding Major Components of Airway Motile Cilia Axoneme – outer dynein arm – Dyenin axoneal
- 18. Secondary ciliary disfunction Viruses Bacterial mediators - H. influenzae, P. aeruginosa, Streptococcus pneumoniae (direct damage) Smoking
- 19. Primary and secondary mucociliary clearance disturbance leads to airway dehydration, excess mucus volume and viscosity. Increase
- 20. Primary anatomical changes, promoting clearance disorders due to bronchi deformities or compression Traction bronchoectases – advanced
- 21. Flora Haemophilus influenzae (29%–70%) Pseudomonas aeruginosa (12%–31%). No pathogenic bacteria (30%–40% ) Best preserved lung function:
- 22. Effects of flora promoting bronchoectases inhibition of the mucociliary clearance: mediators of H. influenzae, P. aeruginosa,
- 23. Immune dysfunction Malnutrition Extremes of age hypogammaglobulinemia, human immunodeficiency virus (HIV), interferon gamma receptor deficiency, type
- 24. Figure 1 The Lancet 2018 392, 880-890DOI: (10.1016/S0140-6736(18)31767-7) Copyright © 2018 Elsevier Ltd Terms and Conditions
- 25. Clinical manifestations Chronic productive cough - 98% of patients Sputum - produced on a daily basis
- 26. Dyspnea – 62%-72% of patients, mixed (obstruction + restriction due to fibrosis) Wheezing – rare (more
- 27. In whom should be suspected? Persistent mucopurulent/purulent sputum + risk factors rheumatoid arthritis + chronic productive
- 28. Diagnosis: to confirm baseline chest X-ray in patients with suspected bronchiectasis. Thin section computed tomography scan
- 29. CT features of bronchiectasis bronchial dilatation as suggested by one or more of the following: Bronchoarterial
- 30. Diagnosis: general + flora full blood count incl ESR In all patients: specific antibodies against capsular
- 31. Diff: COPD Bronchiectases Sputum >50 ml, more purulent Hemopthisis common Fever more common Dullness zones may
- 32. Diff: cancer Bronchiectases Sputum >50 ml, more purulent Hemopthisis common Fever more common Dullness zones may
- 33. Diff: embolism Bronchiectases Sputum >50 ml, more purulent Hemopthisis common Fever more common Not typical pleural
- 34. Rare syndroms (ciliary disfunction, cystic fibrosis) Cystic fibrosis Fetal meconium ilius may be Start in early
- 35. Cystic fibrosis predominance of cystic bronchiectasis (arrows) volume loss (fibrosis) diffuse heterogeneous attenuation enlarged lung volumes
- 36. Adult cystic fibrosis (milder case) cylindric bronchiectasis (white arrows) bronchiolitis (black arrows) – tree in bud
- 37. Kartagener's syndrome Dextrocardia (here + cardiomegaly) Here - left middle lobe bronchiectasis, volume loss. Arrow points
- 38. Other endotypes Alpha -1 antitripsin deficiency Panacinar basal emphysema in non-smokers Liver cirrhosis Non-TB mycobacteria post-menopausal
- 39. Sarcoidosis Diffuse fibrosis traction bronchiectasis (arrows, B) predominantly upper lobes. American Journal of Roentgenology > Volume
- 40. Usual interstitial pneumonia (idiopathic pulmonary fibrosis; rheumatoid arthritis, more rare Sjogren, scleroderma) Bibasilar and subpleural reticulation
- 41. Scleroderma and other connective tissue diseases – more typical NSIP Dilated esophagus(white arrow) ground-glass opacities (NSIP)
- 42. Bronchiolitis obliterans after lung transplantation. Transverse images of right lung in deep inspiration (A) and end
- 43. Other endotypes ABPA Blood eosinophilia thick sputum with black Bronchial obstruction with wheeze, Asthma in case
- 44. Allergic bronchopulmonary aspergillosis Asthma Presence of transient pulmonary infiltrates (fleeting shadows) Elevated total serum IgE Peripheral
- 45. Same central bronchiectasis and mucoid impaction, so-called finger-in-glove appearance
- 46. Other investigations: endotypes assessment Co-morbidities and past medical history to identify relevant and possibly causative disease
- 47. Tests for: cystic fibrosis - early onset, male infertility, malabsorption, pancreatitis Primary Ciliary Dyskinesia if supporting
- 48. Other investigations Spirogram/functional investigation of lungs, oxygen saturation, blood gases Daily protein loss, GFR, urine analysis
- 49. Focal idiopathic in left lower lobe non-TB mycobacteria Perimenopausal females Usually dry bronchoectases Focal bronchiectasis frequently
- 50. subtle idiopathic bibasilar cylindric bronchiectasis shows signet-ring sign (arrows). https://www.ajronline.org/doi/10.2214/AJR.09.3053 marked idiopathic left lower bronchiectasis with
- 51. Mycobacterium avium- intracellulare infection Bronchiectasis (arrows) predominantly involves right middle lobe and lingula. American Journal of
- 52. Obstruction Tumor More gradual onset (1-3 mo) Dyspnea progression from expiratory to inspiratory Dry cough, hemopthisis
- 53. Carcinoid. Endobronchial growth May arise berofe bifurcation of lobar bronchi Serotonin secretion symptoms as following: Flushes
- 54. Carcinoid Distal bronchiectases American Journal of Roentgenology > Volume 193, Issue 3 > BronchiectasisSeptember 2009, Volume
- 55. Tumors: postradiation fibrosis Right paramediastinal fibrotic changes, developed after treatment of lung cancer, are associated with
- 56. Broncholithiasis: post TB Calcified left upper lobe endobronchial broncholithiasis Results of lymph node calcification (compression and
- 57. Congenital abnormalities Congenital stenosis (left mainstem bronchus Bronchial atresia focal bronchiectasis (arrow) distal to bronchial atresia
- 58. Other causes Mounier-Kuhn's syndrome. Enlarged trachea (arrow). Enlarged mainstem bronchi (black arrows) and distal bronchiectasis (white
- 59. Williams-Campbell mostly varicose and cystic central bronchiectasis (arrows). American Journal of Roentgenology > Volume 193, Issue
- 60. Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneityProf Patrick A Flume, MD, Prof James D
- 61. Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneityProf Patrick A Flume, MD, Prof James D
- 62. Cystic fibrosis Cystic fibrosis (CF) is an autosomal recessive disease Loss of function of the cystic
- 63. Pathogenesis: hypothesis chemical shield’ hypothesis: in normal condition airway epithelium produces low salt ( importance of
- 64. Periciliary liquid layer The mucus layer as a liquid reservoir. Upper panel depicts normal geometry of
- 65. Advanced Drug Delivery Reviews Volume 54, Issue 11, 5 December 2002, Pages 1359-1371 An overview of
- 66. CFTR mutations classifications Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneity The lancet Volume 392,
- 67. Median age at diagnosis- 6-8 months; two thirds of patients are diagnosed by 1 year of
- 68. Primary ciliary dyskinesia multiple genes
- 69. Idiopathic bronchiectasis associated with non-tuberculous mycobacteria (NTM) post-menopausal non-smoker females chronic cough No predisposing factors share
- 70. Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneityProf Patrick A Flume, MD, Prof James D
- 71. Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneityProf Patrick A Flume, MD, Prof James D
- 72. T Hill A, L Sullivan A, D Chalmers J, et al British Thoracic Society Guideline for
- 73. Initial treatment European Respiratory Society guidelines for the management of adult bronchiectasis Eva Polverino, Pieter C.
- 74. Offer annual influenza immunisation to all patients with bronchiectasis. (D) Offer polysaccharide pneumococcal vaccination to all
- 75. Physiotherapy – drainage promotion AD: autogenic drainage; ELTGOL: total slow expiration with open glottis and infralateral
- 76. Airway clearance techniques should be taught by a respiratory physiotherapist. Patients admitted with an exacerbation of
- 77. Consider autogenic drainage, positive expiratory pressure, high frequency chest wall oscillation and intrapulmonary percussive ventilation as
- 78. Airway clearance techniques during an acute exacerbation Manual techniques may be offered to enhance sputum clearance
- 79. Mucoactives in bronchiectasis Do not routinely use recombinant human DNase in adults with bronchiectasis. Consider the
- 80. T Hill A, L Sullivan A, D Chalmers J, et al British Thoracic Society Guideline for
- 81. T Hill A, L Sullivan A, D Chalmers J, et al British Thoracic Society Guideline for
- 82. Inhaled GCS: Do not offer long-term oral corticosteroids for patients with bronchiectasis without other indications (such
- 83. PDE inhibitors, CXCR2 antagonists, statins etc Do not routinely offer phosphodiesterase type 4 (PDE4) inhibitors, methylxanthines
- 84. Antibiotics European Respiratory Society guidelines for the management of adult bronchiectasis Eva Polverino, Pieter C. Goeminne,
- 85. Consider long term antibiotics in patients with bronchiectasis who experience 3 or more exacerbations per year.
- 86. Safety Prior to starting long term macrolides, for safety reasons: (1) ensure no active NTM infection
- 87. Ps.aeruginosa European Respiratory Society guidelines for the management of adult bronchiectasis Eva Polverino, Pieter C. Goeminne,
- 88. Offer patients with bronchiectasis associated with clinical deterioration and a new growth of P. aeruginosa (1st
- 89. Offer patients with bronchiectasis associated with clinical deterioration and a new growth of methicillin-resistant S. aureus
- 90. Consider long term oxygen therapy for patients with bronchiectasis and respiratory failure, using the same eligibility
- 91. Consider lung resection in patients with localised disease whose symptoms are not controlled by medical treatment
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