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bronchiectasis

Chronic dilation of bronchi or bronchioles as a sequel of inflammatory disease or obstruction. Etiology: 1) infection a) pneumonia (reversible cause) b) COPD, chronic bronchitis (reversible cause) c) organisms 1] bacterial a] tuberculosis (especially affecting upper lobes) b] Mycobacterium avium complex [12,13] b] pertussis as child may lead to bronchiectasis as adult [5] 2] viral a] adenovirus in adults b] measles or influenza in children c] HIV1 infection 3] fungal (chronic mycoses) (affects upper lobes) a] *histoplasmosis (not typical) [12] b] coccidioidomycosis c] allergic bronchopulmonary aspergillosis d) acute exacerbation - Haemophilus influenzae, Moraxella catarrhalis, Staphylococcus aureus, Pseudomonas aeruginosa 2) ciliary dyskinesia (immotile cilia syndrome) a) inherited b) acquired (especially smoking) 3) immunoglobulin disorders a) Bruton's agammaglobulinemia b) selective IgA deficiency c) hyperimmunoglobulin E (Job's syndrome) d) common variable immune deficiency 3) HIV1 infection 4) right middle lobe syndrome (Mycobacterium avium complex) 5) allergic bronchopulmonary aspergillosis (asthma, eosinophilia, high serum IgE) a) affects upper lobes b) reversible cause 6) yellow nail syndrome 7) congenital - Young's syndrome - Mounier-Kuhn syndrome 8) unilateral hyperlucent lung syndrome 9) cystic fibrosis (affects upper lobes) 10) uncommon causes 1] alpha-1 antitrypsin deficiency 2] connective tissue diseases - rheumatoid arthritis (Felty's syndrome) - scleroderma - Sjogren's syndrome - systemic lupus erythematosus 3] toxic inhalation 4] chronic tracheobronchial stenosis 5] recurrent pulmonary aspiration 6] heroin 7] inflammatory bowel disease - ulcerative colitis, Crohn's disease 8] foreign body - asbestosis 9] sequestrated lung 10] chronic tracheoesophageal fistula 11] heart-lung transplantation - lung transplantation rejection 12] chronic granulomatous disease 13] chronic hypersensitivity pneumonitis 14] radiation therapy 15] sarcoidosis 16] asthma 17] Marfan syndrome 18] lymphadenpathy [5] 19] neoplasms Epidemiology: - occurs in both smokers & non-smokers [5] - non cystic fibrosis patients - 79% women, 75% diagnosed age 50-75 years [10] Pathology: 1) most commonly occurs in lower lung fields 2) in most cases 2nd to 4th order bronchi are involved 3) bilateral involvement in 30% 4) ~20% of patients with bronchiectasis have eosinophilic inflammation [20] Clinical manifestations: 1) chronic productive cough - cough not always productive [10] 2) sputum a) purulent b) frequently copious c) may be foul smelling (fetor oris) 3) recurrent bronchial infection, pneumonia 4) sinusitis may be present 5) hemoptysis may occur (especially in areas of old tuberculosis) 6) coarse rales at lung bases 7) clubbing of fingers 8) arthralgia 9) hypertrophic pulmonary osteoarthropathy 10) anorexia & weight loss 11) dyspnea common 12) fatigue common Laboratory: 1) sputum cultures yield a mixture of organisms - non-tuberculous acid-fast mycobacterium, Mycobacterium avium complex - Pseudomonas [10] 2) sputum forms layers on standing 3) serum IgA, serum IgG, serum IgM 4) complete blood count - eosinophil count (> 300/uL eosinophilic bronchiectasis)* 5) antibodies to Aspergillus [11] a) Aspergillus IgE in serum b) Aspergillus IgG in serum * eosinophil count < 100/uL associated with greater disease severity & mortality [20] * higher eosinophilic counts were associated with shorter time to exacerbations [20] Special laboratory: 1) angiography by interventional radiology for massive hemoptysis - 200-1000 mL in 24 hours or > 100 mL in one event [14] 2) pulmonary function testing: - generally shows obstructive lung disease (50%) - 20% with restrictive pattern [10] 3) flexible bronchoscopy with bronchoalveolar lavage - identify obstruction - testing for infections missed by sputum culture [5] 4) skin testing for Aspergillus fumigatus (immediate cutaneous reactivity) Radiology: 1) chest X-ray a) may appear normal even with advanced disease b) patches of increased density at lung bases c) crowding of bronchi d) segmental atelectasis e) honeycombing with cystic spaces measuring < 2.0 cm f) loss of lung volume g) air-fluid level (if cystic bronchiectasis is present) h) 'tram lines' from inciting event, i.e. pneumonia in childhood i) nodular opacities in right middle lobe - Lady Windermere syndrome (Mycobacterium avium complex) 2) high-resolution computed tomography (CT) of thorax has replaced bronchography as the diagnostic modality of choice a) signet-ring shadows - dilated bronchus (ring) with bronchial artery (stone) b) bronchial wall thickening c) dilated bronchi extending to the periphery - airway larger than accompanying blood vessel - lack of distal airway tapering d) bronchial obstruction secondary to inspissated purulent secretions e) loss of lung volume f) air-fluid level (if cystic bronchiectasis is present) Complications: 1) severe hemoptysis 2) progressive respiratory failure with hypoxemia 3) cor pulmonale 4) secondary infection a) generally saprophytic infection with fungi &/or mycobacteria b) Pseudomonas aeruginosa 1] most commonly isolated organism 2] impossible to erradicate 3] infection with Pseudomonas correlates with more extensive disease Management: 1) determine if underlying treatable cause(s) - treat underlying disorders aggressively 2) treatment aimed at controlling symptoms, clearing airway, treating infection & preventing exacerbations [5] a) postural drainage b) chest physiotherapy c) humidification d) acute exacerbations - respiratory fluoroquinolone (levofloxacin, moxifloxacin) to ensure coverage for Haemophilus influenzae, Moraxella catarrhalis, Staphylococcus aureus, & Pseudomonas aeruginosa for 10-14 days [5,11] - ciprofloxacin may also be used [5] - despite its non-respiratory fluoroquinolone status [5] - best activity among fluoroquinolones against Pseudomonas aeruginosa - use of glucocorticoids not recommended in the absence of asthma or allergic bronchopulmomary aspergillosis e) in hospital-acquired pneumonia &/or recent antibiotic use (within 90 days) coverage for multi-drug-resistant Pseudomonas & MRSA - vancomycin + cefepime, Zosyn or meropenem + fluroquinolone - vancomycin + aztreonam or meropenem + fluroquinolone if penicillin allergy [23] e) cyclic antibiotic therapy f) daily azithromycin 250 mg results in fewer exacerbations at a cost of antibiotic resistance [6] g) no benefit for routine use of bronchodilators [5] h) no benefit of long-term systemic glucocorticoids [5] 3) pulmonary rehabilitation [5] a) improves exercise capacity b) reduces emergency department & outpatient visits 4) surgery 5) lack of evidence-based interventions [10] 6) no role for statins [11]

Related

allergic bronchopulmonary aspergillosis; allergic bronchopulmonary mycosis (ABPA) Bruton type agammaglobulinemia ciliary dyskinesia; immotile cilia syndrome (Kartagener's syndrome) hyperimmunoglobulin E (Job's syndrome) right middle lobe syndrome selective IgA deficiency yellow nail syndrome (lymphedema & yellow nails) Young's syndrome (obstructive azoospermia)

Specific

congenital bronchiectasis

General

chronic lung disease obstructive lung disease

References

  1. Stedman's Medical Dictionary 26th ed, Williams & Wilkins, Baltimore, 1995
  2. Guide to Physical Examination & History Taking, 4th edition, Bates B, JB Lippincott, Philadelphia, 1987
  3. DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 870
  4. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 746-48
  5. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2015, 2018, 2022.
  6. Altenburg J et al. Effect of azithromycin maintenance treatment on infectious exacerbations among patients with non-cystic fibrosis bronchiectasis: The BAT randomized controlled trial. JAMA 2013 Mar 27; 309:1251 PMID: 23532241 - Serisier DJ et al. Effect of long-term, low-dose erythromycin on pulmonary exacerbations among patients with non-cystic fibrosis bronchiectasis: The BLESS randomized controlled trial. JAMA 2013 Mar 27; 309:1260 PMID: 23532242
  7. Chalmers JD, Smith MP, McHugh BJ et al Short- and long-term antibiotic treatment reduces airway and systemic inflammation in non-cystic fibrosis bronchiectasis. Am J Respir Crit Care Med. 2012 Oct 1;186(7):657-65. PMID: 22744718
  8. McShane PJ, Naureckas ET, Tino G, Strek ME. Non-cystic fibrosis bronchiectasis. Am J Respir Crit Care Med. 2013 Sep 15;188(6):647-56 PMID: 23898922
  9. Pasteur MC, Bilton D, Hill AT et al British Thoracic Society guideline for non-CF bronchiectasis. Thorax. 2010 Jul;65 Suppl 1:i1-58. Review. PMID: 20627931
  10. Aksamit TR et al. Adult patients with bronchiectasis: A first look at the US Bronchiectasis Research Registry. Chest 2017 May; 151:982 PMID: 27889361
  11. Anello J, Feinberg B, Heinegg J et. al. New Guidelines and Recommendations, October 2017 Medscape. Oct 06, 2017. http://reference.medscape.com/viewarticle/886616_15
  12. NEJM JWatch Question of the Week. March 27, 2018 https://knowledgeplus.nejm.org/question-of-week/562/
  13. Ebihara T, Sasaki H. Bronchiectasis with Mycobacterium avium Complex Infection. N Engl J Med 2002; 346:1372 PMID: 11986411 Free full text http://www.nejm.org/doi/full/10.1056/NEJMicm010899
  14. NEJM Knowledge+ Question of the Week. June 19, 2018 https://knowledgeplus.nejm.org/question-of-week/1699/
  15. Milliron B, Henry TS, Veeraraghavan S, Little BP. Bronchiectasis: Mechanisms and Imaging Clues of Associated Common and Uncommon Diseases. Radiographics. 2015 Jul-Aug;35(4):1011-30. Review. PMID: 26024063
  16. Smith MP. Diagnosis and management of bronchiectasis. CMAJ. 2017 Jun 19;189(24):E828-E835. Review. PMID: 28630359 Free PMC Article
  17. NEJM Knowledge+ Question of the Week. April 16, 2019 https://knowledgeplus.nejm.org/question-of-week/561/
  18. Barker AF. Bronchiectasis. N Engl J Med 2002 May 3; 346:1383. PMID: 11986413 https://www.nejm.org/doi/full/10.1056/NEJMra012519
  19. Feldman C. Bronchiectasis: new approaches to diagnosis and management. Clin Chest Med. 2011 Sep;32(3):535-46. Review. PMID:21867821
  20. Shoemark A et al. Characterization of eosinophilic bronchiectasis: A European multicohort study. Am J Respir Crit Care Med 2022 Apr 15; 205:894. PMID: 35050830 https://www.atsjournals.org/doi/10.1164/rccm.202108-1889OC - Singh D, Brightling C. Bronchiectasis, the latest eosinophilic airway disease: What about the microbiome? Am J Respir Crit Care Med 2022 Apr 15; 205:860. PMID: 35213295 https://www.atsjournals.org/doi/10.1164/rccm.202201-0105ED
  21. NEJM Knowledge+ Question of the Week. Nov 8, 2022 https://knowledgeplus.nejm.org/question-of-week/233/ - Thompson GR, Young JAH. Aspergillus infections. PMID: 34644473 N Engl J Med 2021; 385:1496-1509 https://www.nejm.org/doi/full/10.1056/NEJMra2027424 - Patel G, Greenberger PA. Allergic bronchopulmonary aspergillosis. Allergy Asthma Proc 2019 Nov 1; 40:421-424. PMID: 31690385 - Shah A, Panjabi C. Allergic bronchopulmonary aspergillosis: a perplexing clinical entity. Allergy Asthma Immunol Res 2016 Jul; 8:282-97. PMID: 27126721 PMCID: PMC4853505 Free PMC article
  22. O'Donnell AE. Bronchiectasis. Chest. 2008 Oct;134(4):815-823. PMID: 18842914 Review.
  23. NEJM Knowledge+