BRONCHIECTASIS approach and treatment by Dr.Amira Tabidi

Pulmonolgy ,it's a common respiratory air way disease with many radiogical features that's vital to learn about it so you can reach the diagnosis easily along with a solid clinical approach

1. BRONCHIECTASIS Dr. Amira Ahmed

2.   TABLE OF CONTENTS HIGHLIGHT OVERVIEW PATHOPHISIOLOGY AETIOLOGICAL APPROACH CLINICAL APPROACH DIAGNOSIS COMMON DIFFERENTIAL DIAGNOSES OF BRONCHIECTASIS MANAGEMENT COMPLICATIONS>>>>>>>>Exacerbation REFERANCES

3. H IGHLIGHT Bronchiectasis is an end-stage of variety of pathological processes Modernization of diagnostic procedures ( CT. scan ) and definition of a clinical picture ( repeated lung infections with a chronic cough and persistent sputum production ) have raised international awareness of the prevalence of the disease, leading to increasing interest in reviewing and renewing the treatment guidelines .

4. Suspect bronchiectasis in those with recurrent bacterial lung infections . Suspect bronchiectasis in a patient with recurrent episodes of ‘bronchitis’ prior to presentation, including those with COPD with Frequent exacerbations and/ or positive sputum for P. aeruginosa.

5. The diagnostic test of choice for bronchiectasis is the high resolution CT sca n( HRCT) Sorting bronchiectasis as localized or diffuse narrows the differential diagnosis . The underlying cause of bronchiectasis should be sought after NTM which may complicate pre-existing bronchiectasis or be the primary cause .

6. OVERVIEW B ronchiectasis is a chronic suppurative lung disease characterized by a syndrome of productive cough and recurrent respiratory infections due to abnormal , irreversibly dilated thick-walled bronchi. It usually affects the proximal and medium-sized bronchi with many etiologies .

7. The management of bronchiectasis can be challenging because its pathogenic mechanisms is still evolving. Its diagnosis and management are particularly more demanding especially in resource-limited areas .   Patients with disease report worse quality of life (QOL) than do persons in the general population.

8. I n 1950 REID characterized bronchiectasis as C ylindrical the mild form that shows loss of normal airway tapering and its the commonest. V aricose shows more distortion along with more mucus and sputum production and some bronchi may appear in beaded form.

9. C ystic or saccular the most severe form in which severe loss of bronchial wall. structure leads to large balloon-like dilatations either focal or cystic distortion of the distal airways . This type of bronchiectasis often followed severe lung infections usually in childhood.

10. PATHOPHYSIOLOGY A s consequence of previous lung diseases, especially lung infections that were not treated sufficiently. This renders the lung tissue susceptible to further infections, as the mucociliary defense mechanism fails to regain its full ability to fight off infectious agents a mechanism further described as the “ vicious cycle theory ”

11. M icroorganisms produce pigments, proteases, and other toxins that injure the respiratory epithelium, which induce an inflammatory response in the host tissue . The dominant cell types involved in the inflammatory process are N eutrophils, lymphocytes, and macrophages.

12. N eutrophils are the most abundant cell type in the bronchial lumen. R eleasing mediators which destroy the bronchial elastin and other supporting lung structure . A lter the function of the cilial epithelium, leading to changes in cilia beat frequency and mucous gland hypersecretion.

13. Weakness of the airways may take many forms. Classic post infectious bronchiectasis Pulsion bronchiectasis implies that intense inflammation originating in the lumen leads to permanent airway dilation as In ABPA . Traction bronchiectasis implies that local retractile forces dilate the airways As in the lung fibrosis

14. A traditional “ wet ” versus “ dry or sicca ” bronchiectasis is now less commonly used its usually a sequalae of TB and found in the upper lobes

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16. APPROACH TO DIAGNOSIS OF BRONCHIECTASIS EATIOLOGY A diverse set of medical conditions is strongly associated with or complicated by bronchiectasis . Stduies have reported a lag time between the onset of symptoms and the formal diagnosis individuals are often diagnosed as COPD,ASTHMA,TB,CHRONIC SINUS DISEASE.

17. The causes of bronchiectasis are many and varied Determining the aetiology of the condition may lead to different management. IDIOPATHIC cause is around 50% of cases, and this is likely to be due to an (as yet unidentified) impairment in host defense . Its lower lobe predominant.

18. Different respiratory infections can cause bronchiectasis, including: Pertussis Gram negative bacteria (Pseudomonas aeruginosa, Haemophilus influenzae) Viruses (HIV, paramyxovirus, adenovirus, and flu) M.Tuberculosis Atypical mycobacteria.

19. Bronchiectasis subsequent to infection with Mycobacterium avium is a typical finding in Lady Windermere syndrome . Patients suffering from this syndrome are often older, immunocompetent women without a history of smoking or previous pulmonary pathologies in the right middle lobe or lingular lobe.

20. Mounier -Kuhn syndrome (congenital tracheobronchomegaly due to atrophy of airway elastic fibers ) Williams-Campbell syndrome (absence of cartilaginous rings in the segmental and sub segmental generations of bronchi). Weakened airways and airway collapsibility impair the effectiveness of the cough mechanism .

21. I n addition to this schema, determining the etiologic cause of bronchiectasis may be enhanced by understanding distribution patterns on HRCT imaging. The anatomic distribution of bronchiectasis associated with various etiologies is outlined below

22. FOCAL TYPE : Is a sequalae of prior infections or endobronchial obstruction DIFFUSE TYPE : Most often in patients with genetic , immunological or anatomical defects with more expansive list of possible causes

23. Left lung is involved more than right lung more in lower lobe and lingula . ( common focal type ) Lower lobes are involved more than upper lobes due to more efficient drainage of upper lobes by gravity. Smaller bronchi with less supportive cartilage are predominantly involve.

24. Infection Lower lobes, Right middle. lobe , and the lingula Obstruction Right middle lobe Tuberculosis and Upper lobes chronic fungal infections ABPA Upper lobes ( centra l bronchi -proximal BX )

25. ASSOCIATED CONDITIONS: Chronic sinusitis Yellow nail syndrome Marfan's syndrome Connective tissue diseases Rheumatoid arthritis( up to 35% of pts have bronchiectasis) Inflammatory bowel disease.

26. CLINICAL APPROACH The clinical manifestations result due to pathophysiologic mechanisms caused by following anatomic alterations : Incomplete obstruction :   Hyperinflation of the distal alveoli as a result of expiratory check-valve mechanism Complete obstruction : consolidation , collapse and fibrosis

27. F atima a 33-year-old house wife, lifelong nonsmoker from Kassala city has symptoms of chronic persistent productive cough on a daily basis for more than 1 yr. The sputum can be clear or pale yellow , and once she had haemoptysis , She also complaining of weight loss , tiredness and SOB provoked by less than 10 min walk. Prior to the current bout of symptoms, she has been having recurrent cough for 3 years , she was admitted several times and treated as pneumonia , when TB work up finally done it was found to be positive she had received two courses of anti-tuberculosis medications 1 st treatment in 2015 and second treatment in 2017 and she did not completed 6 months regimen for either. She denied that she have any other chronic systemic disease.

28. HISTORY Persistent cough with daily excessive sputum production /foul smelling sputum. Recurrent episodes of upper or lower pulmonary infections Pleuritic chest pain. Haemoptesis. mMRC SCALE grade 3

29. PAST HISTORY Childhood symptoms, previous pneumonia/viral illness or pulmonary TB , gastric aspiration, asthma, joints pain or deformity , infertility, exposure to smoke FAMILY HISTORY Positive FHx of similar condition. Primary ciliary dyskinesia (PCD) ,( Kartagener's syndrome – sinusitis, situs inversus and bronchiectasis), cystic fibrosis (CF)

30. CLINICAL ASSESSMENT GENERAL EXAMINATION Coughing (sputum pot) ill built (in advanced disease) Distended neck veins Upper airway ( nasal polyps) Breathless at rest ( Use of accessory muscles ( Barrel chest (increased AP diameter) Chest movement (B/L equal / asymmetrical) Feature of underlying disease Clubbing ( 2 %) , Tar staining ( 30% of COPD has NCFB ) Cyanosis (advanced disease )

31. VITAL SIGNS Increased RR PR &BP TEMPRETURE Oxygen saturation N/

32. CHEST ASSESSMENT FINDINGS-PRIMARILY OBSTRUCTIVE Trachea central Cricoid-notch distance normal / (reduced ) Normal chest expansion / (reduced horizontally) Norma l TVF /(Decreased due to decreased lung density)

33. Resonan t percussion note / ( Hyper resonant) Vesicular breathing / Diminished breath sounds and Prolonged expiration . Widespread expiratory Wheeze I nspiratory coarse Crackles that alter with coughing Mid Inspiratory squawks

34. CHEST ASSESSMENT FINDINGS-PRIMARILY RESTRICTIVE Trachea is midline /deviated to affected side Chest expansion is reduced in affected area. Increased TVF (increased lung density ) / reduced. Dull percussion note Bronchial breath sounds Whispering pectoriloquy (Increased lung density) End inspiratory Crackles that not alter with coughing

35. FURTHER ASSESSMENT : LN CVS : JVP Sings of Pulmonary hypertension Apex location /Apex displacement TR Limbs pitting / sacral odema. ABDOMEN : + Enlarged tender liver

36. DIFFRENTIAL DIAGNOSIS presence of a productive cough , clubbing , and coarse crackles has the following differential diagnosis: Bronchiectasis Lung abscess Carcinoma of the lung ( Tar staining , lymphadenopathy ) Pulmonary fibrosis (the crackles are not alter with coughing )

37. COMMON DIFFERENTIAL DIAGNOSIS

38. DIAGNOSIS Usually made clinically, with HRCT chest for confirmation . Investigations are aimed at: Confirming the diagnosis. Functional assessment. Identifying a treatable underlying cause for the bronchiectasis ( 50 % ) To look for comorbidity and complications. optimizing management to prevent exacerbations and lung damage

39. INITIAL INVESTIGATIONS FBC with differential Increased hematocrit and hemoglobin Hemoglobin may be low (due to anemia of chronic inflammation) Increased WBC in acute infection LFT RFT SPUTUM MICROSCOPY Standard microscopy, culture, and sensitivity(MC&S), acid- fast bacillus (AFB), and fungal cultures PFTs with reversibility testing LUNG FUNCTION TEST : FEV1/ FVC maybe normal/obstructive or restrictive

40. OBSTRUCTIVE: FEV1 ↓ FVC ↓ /N FEV1 : FVC ↓ RESTRICTIVE: FEV1 N /↓ FVC ↓ FEV1:FVC N/↑

41. FLOW VOLUME LOOP.

42. ABG Mild to moderate stages: Acute alveolar hyperventilation with hypoxemia ( Respiratory alkalosis) ↑ PH ↓ PaCO2 ↓PaO2 N /HCO3

43. ECG : As a base line to assess QTc and to look for complications Normal (usually ) Features of Right Ventricular Hypertrophy (RVH) and Cor pulmonale

44. Typical appearance of RVH: Right axis deviation  (+150 degrees). Dominant R wave in V1 (> 7 mm tall; R/S ratio > 1) Dominant S wave in V6 (> 7 mm deep; R/S ratio < 1). Right ventricular strain pattern  with ST depression and T-wave inversion in V1-4.

45. Diagnostic criteria Right axis deviation  of +110° or more. Dominant R wave in V1 (> 7mm tall or R/S ratio > 1). Dominant S wave in V5 or V6 (> 7mm deep or R/S ratio < 1). QRS duration < 120ms (i.e. changes not due to RBBB). Supporting criteria Right atrial enlargement  (P pulmonale). Right ventricular strain pattern  = ST depression / T wave inversion in the right precordial (V1-4) and inferior (II, III, aVF ) leads. S1 S2 S3 pattern = far right axis deviation with dominant S waves in leads I, II and III. Deep S waves in the lateral leads (I, aVL , V5-V6).

46. ESSANTIAL INVESTIGATIONS RADIOLOGY CXR sensitivity is only 50 % HRCT scan is the golden standard tool its 97% sensitive in detecting disease ( slices < 1mm) and (3mm slices) are needed to exclude a central airway lesion.

47. Tram line sign • Parallel line opacities (dilated , thickened bronchi)

48. Finger in glove -Mucoid impaction )

49. Tree in bud -faint reticulonodular opacities

50. Varicose and cystic bronchiectasis with mucus plugging in upper lobes .

51. Cylindrical BX and tree-in-bud pattern / lower lobes and middle lobe

52. Bunch of grapes sign

53. Tree in bud

54. Mosaic attenuation of the lung due to air trapping in segments of lung affected by bronchiectasis. The air trapping gives rise to hyperlucent (darker) pulmonary segments .

55. Bronchiectasis (straight arrows), areas of decreased attenuation and vascularity (curved arrows), and emphysema (small black arrow heads )

56. A)Chest radiograph showing dextrocardia. B) HRCT cylindrical Bx

57. ADDITIONAL INVESTIGATIONS CFTR mutation screen and SWEAT TEST . Autoantibodies (ANA, Rh.F , dsDNA) if associated arthritis/ connective tissue disease. Vaccination response to tetanus, H. influenza B , and pneumococcal antibodies. ( e.g. IgG subclass deficiency ) Detailed immunological investigation (including neutrophil and lymphocyte function studies, genetic analysis)

58. Nasal nitric oxide +/ - brushings BIOPSY (in tertiary centre ) to assess ciliary beat frequency with video microscopy to exclude PCD in those with risk factors. A1AT levels if deficiency suspected. Barium swallow/ esophageal imaging if recurrent aspiration suspected. Vasculitis screen (RF , ANCA , ANA , ENA , and anti- CCP antibodies) if connective disease/ arthritis/ vasculitis associated bronchiectasis

59. Immunoglobulins A, M, G Aspergillus precipitins, Aspergillus - specific radioallergosorbent test (RAST ), total IgE. HIV serology . Bronchoscopy t o exclude a foreign body if suggested by CT obtain microbiological samples if unusual clinical presentation or failure to respond to standard antibiotics Echocardiography.

60. MANAGEMENT T here is no cure for bronchiectasis, but it can be treated. Watch for early warning signs of a flare-up and work with a health care provider to find the best treatment plan . In a few rare cases, surgery to remove damaged lung tissue in only one area of the lung, may be curative. Research and clinical trials are taking place now to find better treatments and cure(s) for bronchiectasis.

61. Non cystic fibrosis bronchiectasis severity score: The FACED score

62. GENERAL MANAGEMENT OF NON .CF.B The main aims of management of NCFB are: Ensure the patient understanding. Treatment of any underlying medical condition and prevent its progression. Prevention of exacerbations and progression of the disease. MEASURES: Smoking cessation. Optimizing hydration status . Optimize nutrition, treat weight loss or low body mass index aggressively as this is associated with a poorer prognosis

63. D aily chest physiotherapy ,postural or autogenic drainage Exercise regimes— important to prevent general deconditioning. Nebulized saline hypertonic saline, Nebulized DNase may improve airway clearance. B 2agonist enhance airway clearance Acetylcysteine may reduce sputum viscosity. If required (6- month). Cough augmentation— using flutter valves/ positive pressure devices

64. A ntimicrobial chemotherapy Reduction of bacterial load and prevention of 2° airway inflammation and damage. Oxygen therapy if in respiratory failure. NIV as nocturnal / used as a bridge to transplantation. LTOT use the same criteria as for COPD . S teroid if indicated >> In the context of pre existing bronchial asthma or COPD. >> ABPA Remember There is no indication for inhaled steroids in isolated bronchiectasis.

65. R efer for surgery in rare cases, for localized resection of affected area .

66. R efer for lung transplantation if indicated (more in CFB) : Young less than 20yrs female with rapid deterioration Severe haemoptysis despite embolization Recurrent pneumothorax An exacerbation with inpatient admission to RICU or HDU Oxygen dependent respiratory failure , type 2.Rf and PHT FEV1 ≤30 % predicted or FEV1 >30 with rapid progressive deterioration.

67. FURTHER MANAGEMENT Self- management plan Patients need an individual plan for exacerbations , which usually involves having a supply of home antibiotics. Annual influenza / pneumococcal vaccinations. Osteoporosis prophylaxis (if on long- term steroids ). Refer for pulmonary rehabilitation if breathlessness limits activities of daily living

68. Reflux treatment if aspiration Associated rhinosinusitis is seen in up to 70% . Treat with nasal steroid ; consider antibiotics if infection likely. Immunoglobulin replacement therapy Patients found to have immunoglobulin deficiency should be referred to an immunologist.

69. ASSESSMENT OF DISEASE RESPONSE : Decreased in sputum volume and change to a mucoid Improvement in systemic symptoms CRP Spirometry and PFT

70. COMPLICATIONS Acute Infective exacerbation . Recurrent pneumonia Empyema Lung abscess Septic emboli( cerebral abscess) NTM infection ABPA . Haemoptysis : Massive hemoptysis (usually from tortuous bronchial arteries around damaged lung is a life- threatening emergency)

71. Pneumothorax. Lung collapse. Respiratory failure. Cardiovascular disease —the rate correlates with severity of disease and frequency of exacerbations . Amyloidosis.

72. A N EXACERBATION is usually a clinical diagnosis, with an increase in sputum volume and tenacity and with discoloration . It may be associated with chest pain , haemoptysis and wheeze, and systemic upset— fevers, lethargy, and anorexia. The CRP is not always elevated The deterioration in three or more of the following key symptoms for at least 48 hours.

73. Recognize an acute exacerbation with 4 out of 9 criteria : Change in sputum production Increased dyspnea Increased cough Fever Increased wheezing Malaise, fatigue, lethargy Reduced pulmonary function Radiographic changes Changes in chest sounds  

74. COMMON MICRO ORGANISMS H.Influenzae Staph .aureus S treptococcus pnemoniae P.Aeruginosa which found in patient with frequent exacerbation worst CT appearance and faster decline in PFT. Remember in stable pt. with frequent exacerbation>> P.Aeruginosa

75. MILD DISEASE WITH EXACERPATION Sputum sample for M.C and S prior to antibiotic. Empirical antibiotic initially then tailored to the colonizing organism. No prior positive microbiology : Amox 500mg-1 g TDS or / Doxycycline 100mg OD Duration 10-14 days

76. EARLY RELAPSE : Defined as sputum turned purulent with in 6-8 wks . Plan : Reassess the pathogens by sputum c/s. Long course antibiotics: PO . AMOX 500 mg BD PO.DOXYCYCLINE 100mg OD Duration 6 wks. TREATMENT FAILURE Change to iv antibiotics until clinical improving . long term prophylactic antibiotics. Start 2 days after sputum has cleared often for 2wks - 6 months And check sputum if purulent switch to iv abx .

77. SEVERE DISEASE WITH EXACERBRATION Hospital Admission. Sputum for c/s prior to antibiotics. IV.Abx often for 2 wks . / 2days after the sputum has cleared. Assess treatment response by fall in sputum volume / change to mucoid/improvement in systemic symptoms and CRP. Planned for regular cyclical IV.Abx .

78. Frequent exacerbations need Long term management with Combination of Macrolides plus Nebulized colistin or gentamicin.

79. First Isolated P .aeruginosa Oral antipseudomonal Antibiotics for 2 wks . Sputum culture if still positive give IV for 2 wks . plus nebulized antibiotics (Colistin /gentamicin/tobramycin) for 3 months . If there's no response give combination IV.Abx antipseudomonal and aminoglycosides.

80. S TATINS might alter neutrophil apoptosis. in patients with bronchiectasis without  Pseudomonas  infection showed there were clinically meaningful improvements over 6 months with an: Increase in the number of apoptotic neutrophils in sputum. Decrease in the total number of neutrophils in sputum Reduction in levels of interleukin-8 in serum . NEW THERAPIES AND NEW PRESPECTIVES

81. B RENSOCATIB is an oral reversible inhibitor of dipeptidyl peptidase 1 ( DPP-1 ) , an enzyme responsible for the activation of neutrophil serine proteases . Reduction of neutrophil serine protease activity with Brensocatib in patients with bronchiectasis was associated with improvements in bronchiectasis clinical outcomes.

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83. REFERANCES Murray & nadels textbook of respiratory medicine Oxford handbook of respiratory medicine OST Up to date .Com https://www.ncbi.nlm.nih.gov/books British Thoracic Society Guideline for bronchiectasis in adults | Thorax (bmj.com) Epomedicine .   Bronchiectasis Revision Notes  [Internet]. Epomedicine ; 2016 Feb 12 [cited 2022 Apr 5]. Available from:  https://epomedicine.com/medical-students/bronchiectasis / . https:// litfl.com/right-ventricular-hypertrophy-rvh-ecg-library 9.Bronchiectasis : a case-based approach to investigation and management - PubMed (nih.gov)

84. Dr\ Amira Ahmed