1. Yatheendra V

2. DefinitionDefined as- It is an abnormal dilatation of bronchi. Itmaybe either focal, involving theairways supplying a limited region of lungparenchyma or diffuse, airways including a morewidespread distribution. 3. HISTORYRen Laennec, the man who inventedthe stethoscope, used his invention to firstdiscover bronchiectasis in 1819.The disease wasresearched in greater detail by Sir William Osler inthe late 1800s; it is suspected that Osler actuallydied of complications from undiagnosedbronchiectasis. 4. EpidemiologyNo systematic data are available but it is considered asthe reflection of the socio-economic conditions of thepopulation under study.In 20th century the cases of bronchiectasis significantlyreduced due to emergence of anti-biotics and vaccines.Race, sex and age related demography :-no racial prelidiction.-CF related bronchiectasis more common in whitefemales older than 60yrs and is often caused by MACinfection. Its called as Lady-Winderrnere syndromenamed after a character in novel by Oscar Wilde. 5. - In pre antibiotic era the disease used to start asearly as in the first decade. But now the disease hasfound itself in the age group > 60yrs. 6. Etiology1.Focal:-obstruction. For eg: Aspirated foreign body,tumor mass.2. Diffuse:- Cystic fibrosis, Necrotising and suppurativepneumonia (Staphylococcus Aureus andKlebsiella), Post tubercular sequale. 7. 1. ChildhoodPertussis, measles, Necrotizingpneumonia2. Primary infectionsklebsiella speciesstaphylococcal speciesMycoplasma pneumoniaeMycobacterium tuberculosisMeasles virusInfluenza virusHerpes simplex virusAdenovirusPertusis virus 8. MAC infections has increased propensity to occur inHIV infection as well as in immuno compromisedindividual.It is found mainly in women >60yrs who are non-smokers,who dont have any predisposing pulmonarydisorder and who tend to suppress cough.3. ObstructionForeign body4. TumorLaryngeal papillomatosis, adenoma,bronchogenic , carcinoma 9. 5. Hilar adenopathyTuberculosis, histoplasmosis6. Mucoid impactionAllergic bronchopulmonary aspergillosis,bronchocentric granulomatosis, fibrinous bronchitis7.Congenital anatomic defectWilliams-Campbell syndrome(congenital cartilage deficiency), Mounier-Kuhnsyndrome (tracheo-bronchomegaly), Swyer-Jamessyndrome(unilateral hyperlucent lung), pulmonarysequestration, pulmonary artery aneurysm, yellownail syndrome(Hypoplastic lymphatics, pleural effusion,yellow nails ) 10. 8.Immunodeficiency stateIgG subclass deficiency, X-linked,agammaglobulinemia, selective IgA, IgM, or IgEdeficiency, bare lymphocyte syndrome, chronicgranulomatous disease, Nezelof syndrome (Thymicdysplasia with normal immunoglobulins)9.Hereditary abnormalityDyskinetic cilia syndrome, Kartagenerssyndrome (situs inversus, nasal polyposis andbronchiectasis) ,a 1-antitrypsin deficiency, cysticfibrosis, ADPKDK 11. PathogenesisBronchieactasis is a consequence of inflammatory anddestruction of structural component of the bronchialtree mainly mid sized airways, segmental and sub-segmentalbronchi.Micro-organisms such as staphylococcus aureus ,klebsiella, psuedomonas aeruginosa,haemophilus influenzae etc.protease and other toxins 12. Up-regulation of neutrophils(Elastase and Matrix metallo-protinases)Destruction of wall structures- cartilage,muscle,elastictissue and replaced by fibrous connective tissueDilatation of the bronchi leading to accumulation ofthe thick pool of purulent material and occlusion dueto fibrous thickeningIncreased vascularity due to enlargement of bronchialarteries and increased anastonosis between bronchialartery and pulmonary artery 13. pathology 14. Clinical FeaturesSymptoms1.Persistent and recurrent cough with purulentand sputum production.2.Repeated respiratory tract infection.3.Haemoptysis 50-70% of cases.4.Systemic symptoms like : fatigue, weight lossand myalgia5.Pneumonia type- chronic cough and sputumproduction. 15. 6.Few patients give an history of insidious onset ofsymptoms.7.Some remain asymptomatic.8.Some give history of non-productive coughtermed as Dry-bronchiectasis.9.Dyspnea in around 72% cases.10.Wheezing11.Pleuretic chest pain 16. In past the severity of the bronchiectasis was classifiedaccording the amount of sputum production.150ml Sever Bronchiectasis. At present it is classified accorrding to the radiologicalfindings 17. Signs1. Many a times combination of crackles(73%), wheezeand ronchi are heard.2. Clubbing(2-3%) may be present.3. In severe cases with hypoxemia it may be associatedwith cor-pulmonale and features of right ventricularfailure.4. Cyanosis and plethora are a rare finding secondaryto polycythemia from chronic hypoxia. 18. WORK-UP 19. Patient historyChildhood infections, exposure to pulmonarypathogens, aspiration of foreign bodies, pulmonarysymptoms in siblingsPhysical examinationAuscultation for focal wheezes or other adventitialsounds, examination of nares and upper respiratorytract for polyps or evidence of chronic sinusitisLaboratory testsRoutine hematology is non specific but may showanaemia and increased white blood count. It may alsoshow polycythemia as a response to chronic hypoxia. 20. Quantitative immunoglobulin levels of IgG, IgM and IgA areuseful to exclude hypogammaglobulinemia.Quantitative serum alpha 1 anti trypsin levels used torule out AAT deficiencySputum analysiswhen sputum is allowed to settle may reveal Dittrichplugs, small, white or yellow concretions.Grams stain may reveal Pseudomonas and E-colisuggestive of CF but not diagnostic.Presence of non-mucoid sputum is suggestive ofpseudomonas aeruginosa, while presence of eosinophiliaand golden plugs containing hyphae is suggestive ofaspergillous species. 21. Routine bacterial, fungal, and mycobacterial culturesmay reveal other organisms.Pilocarpine ionophoresis (Sweat test)for the evaluation of CFSkin test*Aspergillus antigenAspergillus Precipitin testDiagnostic criteria 1000 IU/ml or a greater than 2folds increase from the base-line 22. Auto-immune screening testFor RA and other auto-immune diseases. For ex ANAantibody assay.Computerized tomographythe HRCT is has almost completely replacedbronchography. The sensitivity and specificity are 84-89%and 82-99% respectively.additional advantages include non invasiveness,avoidance of possible allergen to contrast media andinformation regarding other pulmonary processes. 23. Reids classification :depending on the findings of the CT scan it isclassified as :1.Cylindical bronchiectasis has a tram track lines inlongitudinal section or signet ring in case of a horizontalsection and the adjacent pulmonary artery representing thestone.2.Varicose bronchiectasis : has irregular or beadedbronchi with alternating dilatation and constriction.3.Cystic bronchiectasis has large cystic spaces and ahoney comb appearance. This contrasts with blebs ofemphysema. 24. This CT scan depicts areasof both cysticbronchiectasis and varicosebronchiectasis. 25. Varicose bronchiectasiswith alternating areas ofbronchial dilatation andconstriction 26. Cylindrical bronchiectasis with signet-ringappearance. Note that theluminal airway diameter is greaterthan the diameter of the adjacentvessel. 27. Cystic and cylindricalbronchiectasis of theright lower lobe on aposterior-anterior chestradiograph 28. RadiographyP-A and lateral chest radiographs should be taken.Expected findings :1. Increased pulmonary markings2. Honey combing3. Atelectasis4. Plueral changesSpecific findings Tram tracking, dilated bronchi,clustered cysts. 29. Pulmonary function tests :Patients with bronchiectasis show yearlyrapid deterioration in FEV-1 than a patient withoutbronchiectasis. More rapid decline is seen inPseudomonas aerugenosa infection and in increasedpro inflammatory markers.Electron microscopy examinationto observe the evidence of primary ciliarystructural abnormalities and dyskinesia. 30. BronchographyIts being significantly replaced by CT. It isperformed by instilling contrast via a catheter orbronchoscope and performing plane radiographicimaging. In current practice, its only of potential valuein conforming the location of focal bronchiectasis andexcluding the disease elsewhere in the setting ofpossible surgical resection.It carries a high risk of acute broncho-constriction. 31. BronchoscopyNot helpful in diagnosing bronchiectasis butuseful in identifying abnormalities such as tumors,foreign bodies or other lesions. It may also used alongwith lavage for collection of specimens for culture andstaining. 32. Treatment 33. Supportive TreatmentCessation of smokingAvoidance of second-hand smokingAdequate nutritional intakeImmunizations for influenza and pneumococcalpneumoniaConformation of immunizations for measles, rubellaand pertusisOxygen therapy is reserved for patients withhypoxemia and end stage complications such as cor-pulmonale 34. 1. Treatment of infection2. Clearance of the secretion3. Reduction of the inflammation4. Treatment of the underlying problem 35. Antibiotics- Initially during the acute phaseamoxicillin,TMP or levofloxacin should be startedand later proper antibiotic should be chosenaccordingly to the Sputum culture and Gramsstain.When pseudomonas is the organism oralQuinolone or parentral therapy withaminoglycosides, carbapenamor third generationcephalosporins should be given.There is no firm guidelines for therapy and henceshould be continued for 10-14 days. 36. Aerosolized antibioticsIt delivers relatively high concentration of drugswith relatively lesser systemic side-effects.It is beneficial in treating Pseudomonas infection.Currently, inhaled Tobramycin is most widelyused. Gentamycin and Colistin have also been used 37. Bronchial hygieneProper mechanical and devices with proper positioningof the patient can help the patients with copioussecretions.Postural drainage with percussion and vibration helps ineffective clearance.Devices like Flutter device, Intrapulmonic percussiveventilation device and incentive spirometry areavailable.Newest device is the Vest system wherein a pneumaticcompression vest is worn by the patient periodicallythroughout the day. 38. Tapping of the chest wall todislodge the secretions 39. Positional drainage andphysiotherapy 40. Positive expiratory thera- PEP 41. Use of oscillator 42. Use of Flutter 43. Acapella Device 44. Use of a vest 45. Cornet device 46. Use of mucolytics can help thinning out the thickmucous secretions.Use of recombinant DNAse which help in thedestruction of the DNA released by the neutrophiles hasshown improvement in the PF in case of CF.Bronchodilators help in the obstruction and clearancee of the bronchus.Use of nebulizations concentrated with 7% NaCl haveshown beneficial in CF-related Bronchiectasis. 47. Anti-inflammatory therapyReduce the inflammation caused by theorganisms and subsequently reduce the tissue damage.Inhaled corticosteroids, Leucotriene inhibitors andNSAID can be given.Studies have shown use of inhaled corticosteroids haveshown qualitative improvement in the quality of life.Azithromycin has known anti-inflammatoryproperties and its long term use has shown ,markedimprovement in CF and non-CF bronchiectasis. 48. Surgical resectionHelpful in advanced or complicated disease.Indications :1.Patients who have focal disease that is poorlycontrolled by anti-biotics.2.Reduction of acute infective episodes3.Massive haemoptysis(Alternatively bronchial arteryembolization may be attempted)4. Foreign body or tumor removal5. Consideration in the treatment of MAC orAspergillus specific infections 49. Complications :empyema, haemmorrhage, prolonged air leak andpersistent atelectasis. Mortality is