bronchiectasis
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Introduction
Chronic dilation of bronchi or bronchioles as a sequel of inflammatory disease or obstruction.
Etiology
- infection
- pneumonia (reversible cause)
- COPD, chronic bronchitis (reversible cause)
- organisms
- bacterial
- tuberculosis (especially affecting upper lobes)
- Mycobacterium avium complex[12][13]
- pertussis as child may lead to bronchiectasis as adult[5]
- viral
- adenovirus in adults
- measles or influenza in children
- HIV1 infection
- fungal (chronic mycoses) (affects upper lobes)
- *histoplasmosis (not typical)[12]
- coccidioidomycosis
- allergic bronchopulmonary aspergillosis
- bacterial
- acute exacerbation
- ciliary dyskinesia (immotile cilia syndrome)
- inherited
- acquired (especially smoking)
- immunoglobulin disorders
- HIV1 infection
- right middle lobe syndrome (Mycobacterium avium complex)
- allergic bronchopulmonary aspergillosis (asthma, eosinophilia, high serum IgE)
- affects upper lobes
- reversible cause
- yellow nail syndrome
- congenital
- Young's syndrome
- Mounier-Kuhn syndrome
- unilateral hyperlucent lung syndrome
- cystic fibrosis (affects upper lobes)
- uncommon causes
- alpha-1 antitrypsin deficiency
- connective tissue diseases
- rheumatoid arthritis (Felty's syndrome)
- scleroderma
- Sjogren's syndrome
- systemic lupus erythematosus
- toxic inhalation
- chronic tracheobronchial stenosis
- recurrent pulmonary aspiration
- heroin
- inflammatory bowel disease
- ulcerative colitis, Crohn's disease
- foreign body - asbestosis
- sequestrated lung
- chronic tracheoesophageal fistula
- heart-lung transplantation
- lung transplantation rejection
- chronic granulomatous disease
- chronic hypersensitivity pneumonitis
- radiation therapy
- sarcoidosis
- asthma
- Marfan syndrome
- lymphadenpathy[5]
- neoplasms
Epidemiology
- occurs in both smokers & non-smokers[5]
- non cystic fibrosis patients
- 79% women, 75% diagnosed age 50-75 years[10]
Pathology
- most commonly occurs in lower lung fields
- in most cases 2nd to 4th order bronchi are involved
- bilateral involvement in 30%
- ~20% of patients with bronchiectasis have eosinophilic inflammation[20]
Clinical manifestations
- chronic productive cough
- sputum
- purulent
- frequently copious
- may be foul smelling (fetor oris)
- recurrent bronchial infection, pneumonia
- sinusitis may be present
- hemoptysis may occur (especially in areas of old tuberculosis)
- coarse rales at lung bases
- clubbing of fingers
- arthralgia
- hypertrophic pulmonary osteoarthropathy
- anorexia & weight loss
- dyspnea common
- fatigue common
Laboratory
- sputum cultures yield a mixture of organisms
- sputum forms layers on standing
- serum IgA, serum IgG, serum IgM
- complete blood count
- eosinophil count (> 300/uL eosinophilic bronchiectasis)*
- antibodies to Aspergillus[11]
* eosinophil count < 100/uL associated with greater disease severity & mortality[20]
* higher eosinophilic counts were associated with shorter time to exacerbations[20]
Diagnostic procedures
- angiography by interventional radiology for massive hemoptysis
- pulmonary function testing:
- generally shows obstructive lung disease (50%)
- 20% with restrictive pattern[10]
- flexible bronchoscopy with bronchoalveolar lavage
- identify obstruction
- testing for infections missed by sputum culture[5]
- skin testing for Aspergillus fumigatus (immediate cutaneous reactivity)
Radiology
- chest X-ray
- may appear normal even with advanced disease
- patches of increased density at lung bases
- crowding of bronchi
- segmental atelectasis
- honeycombing with cystic spaces measuring < 2.0 cm
- loss of lung volume
- air-fluid level (if cystic bronchiectasis is present)
- 'tram lines' from inciting event, i.e. pneumonia in childhood
- nodular opacities in right middle lobe
- high-resolution computed tomography (CT) of thorax has replaced bronchography as the diagnostic modality of choice
- signet-ring shadows
- dilated bronchus (ring) with bronchial artery (stone)
- bronchial wall thickening
- dilated bronchi extending to the periphery
- airway larger than accompanying blood vessel
- lack of distal airway tapering
- bronchial obstruction secondary to inspissated purulent secretions
- loss of lung volume
- air-fluid level (if cystic bronchiectasis is present)
- signet-ring shadows
Complications
- severe hemoptysis
- progressive respiratory failure with hypoxemia
- cor pulmonale
- secondary infection
- generally saprophytic infection with fungi &/or mycobacteria
- Pseudomonas aeruginosa
- most commonly isolated organism
- impossible to erradicate
- infection with Pseudomonas correlates with more extensive disease
Management
- determine if underlying treatable cause(s)
- treat underlying disorders aggressively
- treatment aimed at controlling symptoms, clearing airway, treating infection & preventing exacerbations[5]
- postural drainage
- chest physiotherapy
- humidification
- 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
- 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]
- cyclic antibiotic therapy
- daily azithromycin 250 mg results in fewer exacerbations at a cost of antibiotic resistance[6]
- no benefit for routine use of bronchodilators[5]
- no benefit of long-term systemic glucocorticoids[5]
- pulmonary rehabilitation[5]
- improves exercise capacity
- reduces emergency department & outpatient visits
- surgery
- lack of evidence-based interventions[10]
- no role for statins[11]
More general terms
More specific terms
Additional terms
- 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)
References
- ↑ Stedman's Medical Dictionary 26th ed, Williams & Wilkins, Baltimore, 1995
- ↑ Guide to Physical Examination & History Taking, 4th edition, Bates B, JB Lippincott, Philadelphia, 1987
- ↑ DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 870
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 746-48
- ↑ 5.00 5.01 5.02 5.03 5.04 5.05 5.06 5.07 5.08 5.09 5.10 5.11 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2015, 2018, 2022.
- ↑ 6.0 6.1 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: https://www.ncbi.nlm.nih.gov/pubmed/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: https://www.ncbi.nlm.nih.gov/pubmed/23532242 - ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/22744718
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/23898922
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/20627931
- ↑ 10.0 10.1 10.2 10.3 10.4 10.5 Aksamit TR et al. Adult patients with bronchiectasis: A first look at the US Bronchiectasis Research Registry. Chest 2017 May; 151:982 PMID: https://www.ncbi.nlm.nih.gov/pubmed/27889361
- ↑ 11.0 11.1 11.2 11.3 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
- ↑ 12.0 12.1 12.2 NEJM JWatch Question of the Week. March 27, 2018 https://knowledgeplus.nejm.org/question-of-week/562/
- ↑ 13.0 13.1 Ebihara T, Sasaki H. Bronchiectasis with Mycobacterium avium Complex Infection. N Engl J Med 2002; 346:1372 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/11986411 Free full text <Internet> http://www.nejm.org/doi/full/10.1056/NEJMicm010899
- ↑ 14.0 14.1 NEJM Knowledge+ Question of the Week. June 19, 2018 https://knowledgeplus.nejm.org/question-of-week/1699/
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/26024063
- ↑ Smith MP. Diagnosis and management of bronchiectasis. CMAJ. 2017 Jun 19;189(24):E828-E835. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/28630359 Free PMC Article
- ↑ NEJM Knowledge+ Question of the Week. April 16, 2019 https://knowledgeplus.nejm.org/question-of-week/561/
- ↑ Barker AF. Bronchiectasis. N Engl J Med 2002 May 3; 346:1383. PMID: https://www.ncbi.nlm.nih.gov/pubmed/11986413 https://www.nejm.org/doi/full/10.1056/NEJMra012519
- ↑ Feldman C. Bronchiectasis: new approaches to diagnosis and management. Clin Chest Med. 2011 Sep;32(3):535-46. Review. PMID:21867821
- ↑ 20.0 20.1 20.2 20.3 Shoemark A et al. Characterization of eosinophilic bronchiectasis: A European multicohort study. Am J Respir Crit Care Med 2022 Apr 15; 205:894. PMID: https://www.ncbi.nlm.nih.gov/pubmed/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: https://www.ncbi.nlm.nih.gov/pubmed/35213295 https://www.atsjournals.org/doi/10.1164/rccm.202201-0105ED - ↑ NEJM Knowledge+ Question of the Week. Nov 8, 2022 https://knowledgeplus.nejm.org/question-of-week/233/
Thompson GR, Young JAH. Aspergillus infections. PMID: https://www.ncbi.nlm.nih.gov/pubmed/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: https://www.ncbi.nlm.nih.gov/pubmed/31690385
Shah A, Panjabi C. Allergic bronchopulmonary aspergillosis: a perplexing clinical entity. Allergy Asthma Immunol Res 2016 Jul; 8:282-97. PMID: https://www.ncbi.nlm.nih.gov/pubmed/27126721 PMCID: PMC4853505 Free PMC article - ↑ O'Donnell AE. Bronchiectasis. Chest. 2008 Oct;134(4):815-823. PMID: https://www.ncbi.nlm.nih.gov/pubmed/18842914 Review.
- ↑ 23.0 23.1 NEJM Knowledge+