CONTENTS
- Basics of bronchiectasis
- Bronchiectasis exacerbation (non-CF)
- Related topics:
- Questions & discussion
abbreviations used in the pulmonary section: 4
- ABPA: Allergic bronchopulmonary aspergillosis 📖
- AE-ILD: Acute exacerbation of ILD 📖
- AEP: Acute eosinophilic pneumonia 📖
- AFB: Acid Fast Bacilli
- AIP: Acute interstitial pneumonia (Hamman-Rich syndrome) 📖
- ANA: Antinuclear antibody 📖
- ANCA: Antineutrophil cytoplasmic antibodies 📖
- ARDS: Acute respiratory distress syndrome 📖
- ASS: Antisynthetase Syndrome 📖
- BAL: Bronchoalveolar lavage 📖
- BiPAP: Bilevel positive airway pressure 📖
- CEP: Chronic eosinophilic pneumonia 📖
- CF: Cystic fibrosis 📖
- COP: Cryptogenic organizing pneumonia 📖
- CPAP: Continuous positive airway pressure 📖
- CPFE: Combined pulmonary fibrosis and emphysema 📖
- CTD-ILD: Connective tissue disease associated interstitial lung disease 📖
- CTEPH: Chronic thromboembolic pulmonary hypertension 📖
- DAD: Diffuse alveolar damage 📖
- DAH: Diffuse alveolar hemorrhage 📖
- DIP: Desquamative interstitial pneumonia 📖
- DLCO: Diffusing capacity for carbon monoxide 📖
- DRESS: Drug reaction with eosinophilia and systemic symptoms 📖
- EGPA: Eosinophilic granulomatosis with polyangiitis 📖
- FEV1: Forced expiratory volume in 1 second 📖
- FVC: Forced vital capacity 📖
- GGO: Ground glass opacity 📖
- GLILD: Granulomatous and lymphocytic interstitial lung disease 📖
- HFNC: High flow nasal cannula 📖
- HP: Hypersensitivity pneumonitis 📖
- IPAF: Interstitial pneumonia with autoimmune features 📖
- IPF: Idiopathic pulmonary fibrosis 📖
- IVIG: Intravenous immunoglobulin 📖
- LAM: Lymphangioleiomyomatosis 📖
- LIP: Lymphocytic interstitial pneumonia 📖
- MAC: Mycobacterium Avium complex 📖
- MCTD: Mixed connective tissue disease 📖
- NIV: Noninvasive ventilation (including CPAP or BiPAP) 📖
- NSIP: Nonspecific interstitial pneumonia 📖
- NTM: Non-tuberculous mycobacteria 📖
- OHS: Obesity hypoventilation syndrome 📖
- OP: Organizing pneumonia 📖
- OSA: Obstructive sleep apnea 📖
- PAP: Pulmonary alveolar proteinosis 📖
- PE: Pulmonary embolism 📖
- PFT: Pulmonary function test 📖
- PLCH: Pulmonary Langerhans Cell Histiocytosis 📖
- PPFE: Pleuroparenchymal fibroelastosis 📖
- PPF: Progressive pulmonary fibrosis 📖
- PVOD/PCH Pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis 📖
- RB-ILD: Respiratory bronchiolitis-associated interstitial lung disease 📖
- RP-ILD: Rapidly progressive interstitial lung disease 📖
- TNF: tumor necrosis factor
- UIP: Usual Interstitial Pneumonia 📖
- Bronchiectasis is defined as irreversible dilation of the bronchi, usually associated with chronic purulent sputum production.
- Bronchiectasis may be caused by a variety of disorders. Once established, bronchiectasis may lead to a vicious spiral of worsening bronchiectasis (with ongoing infection and inflammation causing progressive worsening of bronchial dilation).
chronic cough (typically with sputum)
- Chronic cough with sputum production is the defining symptom of bronchiectasis, present in ~80% of patients. (35236562)
- Sputum quantity and tenacity correlate with disease severity.
- Milder disease may only present with sputum during exacerbations.
- Severe disease may cause chronic production of purulent sputum. (Teneback 2022)
- Dry bronchiectasis:
- Some patients with bronchiectasis have a chronic nonproductive cough.
- Nonproductive cough corresponds to milder disease, with better functional status. These patients probably won't benefit as much from mucolytics or airway clearance. (Teneback 2022)
hemoptysis
- Hemoptysis varies from minimal to massive, and is often recurrent.
- Large-volume hemoptysis results from rupture of a bronchial artery into the dilated bronchus.
recurrent pneumonia or bronchiectasis exacerbation:
- Recurrent pneumonia:
- Recurrent parenchymal pneumonia may occur, often at a site close to the region of bronchiectasis. However, sometimes patients may have bronchiectasis exacerbations which are misdiagnosed as pneumonia. (35236562)
- Pseudomonas pneumonia 📖 in particular should raise the possibility of bronchiectasis, in the absence of other risk factors for Pseudomonas. (Fishman 2023)
- Bronchiectasis exacerbations: discussed below 📖
other symptoms
- Systemic symptoms may include fever, malaise, and weight loss.
- Dyspnea.
- Wheezing.
CT scan findings to diagnose bronchiectasis
abnormalities of the bronchi
- Broncho/arterial ratio >~1.5
- The broncho/arterial ratio equals the diameter of the bronchus divided by the diameter of the adjacent pulmonary artery (when measured in cross-section).
- The normal broncho/arterial ratio is ~0.65-1.
- Values of 1-1.5 are nonspecific, especially in older people >65 years old or at high altitude. Values >1.5 are more specific for bronchiectasis. (Shepard 2019) If the pulmonary artery divides before the bronchus, this may cause the broncho/arterial ratio to be spuriously elevated. (Walker 2019)
- Signet ring sign is the classic qualitative finding on CT scan of bronchiectasis. This is generated by a combination of bronchial wall thickening plus an elevated broncho/arterial ratio.
- Lack of tapering of the bronchi: Bronchi visible within 1 cm of the pleural surface may be a very early sign of bronchiectasis. (Rosado-de-Christenson 2022)
- Other signs that are less specific:
- Bronchial wall thickening is a supportive finding (but may be caused by other disorders).
- Air-fluid levels within the bronchi, or accumulation of debris.
- Tree-in-bud centrilobular nodules may be seen.
other findings
- Mosaic attenuation may occur. Sometimes this may be seen in areas of the lung without bronchiectasis. (Walker 2019)
- Volume loss of lung tissue (especially in the right middle lobe or lingula). This may occur via various pathways: (Walker 2019)
- (a) Bronchiectasis may lead to atelectasis, due to inflammation and fibrosis around the airways.
- (b) Lung scarring could lead to secondary traction bronchiectasis.
radiographic subtypes of bronchiectasis
subtypes relate to a spectrum of severity
- Cylindrical bronchiectasis (aka tubular): Uniform dilation of bronchi occurs, without the normal narrowing.
- Varicose bronchiectasis: Irregular and beaded bronchi with alternating constriction and dilation
- Cystic/saccular bronchiectasis: Ballooning dilation of the bronchi generates clusters of round air-filled and fluid-filled cysts (which may resemble a bunch of grapes).
- This can mimic cystic lung disease.
other forms of bronchiectasis
- Traction bronchiectasis: Widening of airways without thickening of the bronchi due to surrounding fibrotic interstitial lung disease that pulls the bronchi open. These patients generally don't have symptoms related to the bronchiectasis itself (and often shouldn't be considered to have clinical bronchiectasis).
CT scan clues regarding the etiology of bronchiectasis
central distribution
- ABPA 📖 (allergic bronchopulmonary aspergillosis).
- Cartilaginous/elastin defects:
- Mounier-Kuhn syndrome.
- Ehlers-Danlos syndrome.
- Williams-Campbell syndrome. (34625182)
upper lobe distribution
- Cystic fibrosis (although cystic fibrosis may also cause diffuse bronchiectasis).
- ABPA 📖 (allergic bronchopulmonary aspergillosis).
- Tuberculosis.
- Traction bronchiectasis secondary to sarcoidosis or pneumoconiosis.
middle lobe and lingula
- Nontuberculous mycobacteria, especially Mycobacterium avium complex (MAC).
- Primary ciliary dyskinesia (PCD).
- Middle lobe syndrome (REF).
lower lobes
- Most infections other than tuberculosis tend to involve the lower lobes.
- Immunodeficiency syndrome.
- Impaired mucociliary clearance (e.g., primary ciliary dyskinesia).
- Alpha-1 antitrypsin deficiency.
- Chronic aspiration tends to cause bilateral lower lobe bronchiectasis.
- Autoimmune/collagen vascular disease.
focal bronchiectasis
- Focal central airway obstruction, for example:
- Intrinsic obstruction (e.g., tumor, foreign body).
- Bronchial stricture.
- Extrinsic compression by mass or lymphadenopathy.
- History of focal pneumonia.
- Focal lower lobe bronchiectasis may result from aspiration.
other clues:
- Hiatal hernia and/or a patulous esophagus may suggest a predisposition for aspiration. The differential diagnosis of esophageal dilation includes motility disorders (e.g., achalasia) as well as rheumatologic disorders involving the esophagus (e.g., Sjogren syndrome, scleroderma). (Teneback 2022)
clinical presentation
- Mucoid impaction can be clinically silent.
- Patients may have symptoms such as cough, dyspnea, chest pain, or expectoration of plugs or casts. (23509335)
radiologic findings
- “Finger-in-glove” sign on plain film may reflect bronchial impaction with mucus.
- Chest radiograph: may show branching tubular structures that converge on the hilum.
causes of mucus impaction of bronchi:
- ABPA (allergic bronchopulmonary aspergillosis)
- Usually occurs in the upper lobes, affecting segmental and subsegmental bronchi. (Walker 2019)
- Mucus inspissation may be transient, or can persist for weeks to months. (Walker 2019)
- Bronchiectasis of other etiologies (e.g., cystic fibrosis).
- Central airway lesion with distal obstruction, for example
- Broncholithiasis.
- Foreign body aspiration.
- Bronchial neoplasms (e.g., bronchial lipomas, endobronchial hamartomas, papillomas, carcinoid tumors, squamous cell carcinoma).
- Asthma.
- Chronic bronchitis.
- Bronchial atresia (air trapping occurs beyond the atresia).
radiological clues regarding the etiology of mucus impaction:
- High-attenuation mucus:
- Various definitions of high-attenuation mucus:
- Mucus attenuation is visually denser than para-spinal skeletal muscle.
- Hounsfield units of mucus may be >70. (27719981)
- If encountered, high-attenuation mucus is highly suggestive of ABPA. (30713049) Among patients with ABPA, high-attenuation mucus may be seen in about a third of patients. It correlates with higher peripheral eosinophil counts, higher IgE levels, and higher relapse rates. (27719981)
- Various definitions of high-attenuation mucus:
- Pattern and extent:
- Focal involvement suggests a more focal disease process (e.g., endobronchial tumor).
- Bilateral involvement suggests a more global process (e.g., ABPA).
- Features concerning for malignancy:
- Hilar lymphadenopathy.
- Endobronchial or perihilar mass. (23509335)
- Mild bronchiectasis may be associated with normal pulmonary function tests.
- Moderate bronchiectasis may cause a reduction in both FEV1 and FVC (with FEV1/FVC generally >0.7).
- Severe bronchiectasis may cause airflow obstruction and gas trapping. (35236562)
With increasing utilization of CT scans, it is increasingly common to detect the presence of bronchiectasis on CT scanning. However, this alone doesn't necessarily indicate that the patient has bronchiectasis. Clinically significant bronchiectasis has been defined as follows: (34570994)
definition of clinically significant bronchiectasis:
- Radiology: at least one of the following features:
- Bronchial/artery ratio >~1.5.
- Lack of tapering of the airways.
- Visibility of airways in the periphery.
- Clinical features: at least two of the following:
- A cough most days of the week.
- Sputum production most days of the week.
- A history of exacerbations.
infectious or postinfectious (~20-30%)
- Mycobacteria:
- Tuberculosis.
- Non-tuberculous mycobacteria.
- Allergic bronchopulmonary aspergillosis (ABPA) (~5%).
- Necrotizing pneumonia.
- Childhood viral infection (e.g., measles, adenovirus).
primary impairment in airway clearance
- Increased sputum viscosity:
- Cystic fibrosis (~2%).
- Young syndrome (similar to cystic fibrosis, but sweat chloride levels and pancreas function are normal).
- Primary ciliary dyskinesia (including Kartagener syndrome).
immunodeficiency (7%)
- Common variable immunoglobulin deficiency.
- Job syndrome.
- IgG subclass deficiency.
- HIV.
rheumatologic/inflammatory disorders (10%)
- More often:
- Rheumatoid arthritis (7%).
- Sjogren syndrome.
- Lupus.
- Inflammatory bowel disease.
- ANCA-associated vasculitis.
disorder of connective tissue integrity
- Mounier-Kuhn syndrome (tracheobronchomegaly).
- Marfan syndrome.
- Williams-Campbell syndrome (absence of cartilaginous rings in 4th-6th generation bronchi, affecting segmental and subsegmental bronchi).
- Relapsing polychondritis.
chronic central airway obstruction of any etiology, including:
- Tumor.
- Foreign body.
- Right middle lobe syndrome (compression of the right middle bronchus by lymphadenopathy).
other
- Chronic aspiration (~2%).
- Chronic obstructive pulmonary disease (~15% of bronchiectasis). (Teneback 2022)
- Asthma (~3% of bronchiectasis).
- Idiopathic (~40% of patients with bronchiectasis, most common etiology). (Tenebeck 2022)
- Yellow nail syndrome. 📖
- Alpha-1 antitrypsin deficiency. 📖
- (Traction bronchiectasis may result from any cause of pulmonary fibrosis that causes alveolar volume loss. However, traction bronchiectasis generally doesn't cause clinical symptoms of bronchiectasis – it's usually a purely radiological entity.)
initial evaluation
history
- Preceding infection(s)?
- A single severe infection can cause bronchiectasis (e.g., tuberculosis).
- Repeated infections involving the lung, sinus, and/or ear may suggest immunodeficiency.
- History of atopy, especially asthma? Consider ABPA (allergic bronchopulmonary aspergillosis).
- Rheumatologic history (Joint symptoms? Raynaud phenomenon? Sicca syndrome?).
- Infertility? (Cystic fibrosis, primary ciliary dyskinesia, Young syndrome).
- Smoking history?
CT scan clues to the etiology: ⚡️
sputum culture in bronchiectasis
- This requires two separate orders: Bacterial culture/smear and AFB culture/smear.
- Diagnostic significance:
- Organisms commonly isolated in the sputum include Pseudomonas aeruginosa, methicillin-resistant Staphylococcus aureus, Haemophilus influenzae, and Mycobacterium avium complex. (Tenebeck 2022)
- Aspergillus fumigatus may reflect ABPA (allergic bronchopulmonary aspergillosis).
- Therapeutic significance:
- For patients with bronchiectasis who first become colonized or infected with Pseudomonas or Burkholderia cepacia, eradication therapy should be considered.
other laboratory tests
- Complete blood count with differential:
- Eosinophilia may raise the possibility of ABPA (allergic bronchopulmonary aspergillosis); more on the causes of eosinophilia here: 📖
- Leukopenia or lymphopenia may indicate underlying immunodeficiency.
- Immunoglobulin levels including IgE (IgG, IgM, IgA, and IgE). (Teneback 2022)
- All immunoglobulins severely low: consider agammaglobulinemia.
- Low IgG, IgM, and/or IgA: consider CVID.
- Low IgG: consider IgG subclass deficiency.
- High IgE: 📖
- HIV screening serology.
- Evaluation for ABPA if IgE levels are elevated: 📖
advanced laboratory tests (utilize selectively)
for suspected cystic fibrosis
- Sweat chloride testing:
- <30 mM is considered normal and makes CF unlikely. However, normal sweat chloride can be seen in 1% of CF genotypes, so if there is a high suspicion for CF then genotyping is reasonable. (ERS handbook 3rd ed.)
- 30-59 mM is indeterminate and requires further testing (i.e., CFTR gene analysis).
- >59 mM is abnormal.
- CFTR gene analysis is usually definitive. This also identifies the genetic abnormality, which defines optimal CFTR-directed therapies.
other tests
- Alpha-1 antitrypsin level if deficiency is suspected. 📖
- Rheumatologic serologies (e.g., ANA, rheumatoid factor, anti-SSa, anti-SSb).
Below is a very brief overview of outpatient management.
treatment of underlying disease processes
- Causes of bronchiectasis are listed above.
- In many cases, treatment of underlying processes is fundamental to prevent disease progression.
airway hygiene
- Chest physiotherapy is indicated for patients with chronic sputum production.
- Various devices may be utilized, with selection depending on logistics and patient preference. Common examples may include:
- Oscillating positive expiratory pressure devices (e.g., Acapella).
- High-frequency chest wall oscillation with a vest. (23556995)
- Various devices may be utilized, with selection depending on logistics and patient preference. Common examples may include:
- Hypertonic saline: 7% saline inhaled twice to four times daily may improve mucus clearance and decrease exacerbations. (22018993) Mannitol 400 mg inhaled twice daily also appears to be effective. (25246664)
antimicrobial therapy
- Chronic azithromycin therapy has been shown to reduce sputum production, exacerbation frequency, and clinical symptoms. However, azithromycin must not be utilized in patients who are colonized with nontuberculous mycobacteria (NTM), as this may cause drug resistance that complicates the therapy for NTM (ideally two or three sputum cultures negative for NTM should be obtained before starting azithromycin).
- Chronic nebulized antibiotics may help reduce the frequency of exacerbations, especially among patients colonized with Pseudomonas.
definition of bronchiectasis exacerbation
- The consensus definition for research purposes requires: (28596426)
- (1) Deterioration in three or more of the following key symptoms for at least 48 hours:
- Cough.
- Sputum volume and/or consistency.
- Sputum purulence.
- Dyspnea and/or exercise tolerance.
- Fatigue and/or malaise
- Hemoptysis.
- (2) AND a clinician determines that a change in bronchiectasis treatment is required.
- (1) Deterioration in three or more of the following key symptoms for at least 48 hours:
- This definition provides a theoretical framework, but it won't always be applicable. For example, a clinician wouldn't necessarily wait 48 hours to institute therapy.
- Two important points about diagnosing bronchiectasis exacerbation:
- (1) Bronchiectasis exacerbation is a clinical diagnosis. Therefore, an asymptomatic reduction in pulmonary function tests doesn't warrant hospital admission for antibiotics. (Fishman 2023)
- (2) Bronchiectasis exacerbation is a diagnosis of exclusion, so alternative explanations for the patient's acute illness should be excluded.
differential diagnosis: other causes of clinical deterioration may include
- Community-acquired pneumonia.
- Pneumothorax.
- Hemoptysis due to bronchial artery hemorrhage.
data review
- Baseline FEV1.
- Baseline blood gas values and/or bicarbonate level (? chronic hypercapnia).
- Microbiology history.
- Antibiotic history:
- Prior clinical response to various antibiotics.
- Any antibiotic allergies.
- Prior respiratory complications.
studies to obtain
- Routine laboratory studies (including CBC with differential).
- Nasopharyngeal PCRs for relevant viral pathogens, depending on clinical context.
- Sputum:
- Bacterial Gram stain & culture.
- Acid-fast bacilli smear & culture.
- Fungal culture & Gram stain
- Chest X-ray (exclude alternative pathologies such as pneumothorax, pneumonia).
- Chest CT scan if indicated (e.g., hemoptysis, or concern for pulmonary embolism).
Treatment of non-CF bronchiectasis exacerbations is largely extrapolated from evidence regarding cystic fibrosis (since little high-quality evidence exists).
antibiotic therapy
antibiotic selection
- Sputum cultures should be ordered, but these won't return in time to guide therapy. If available, antibiotics may be directed at pathogens detected in prior sputum cultures. However, sputum cultures represent only a minority of the numerous bacteria present in bronchiectatic airways. Providing adequate coverage of organisms cultured from the sputum is theoretically desirable, but not clearly beneficial.
- If a patient previously responded well to a certain antibiotic regimen, repeating that therapy is reasonable.
- A broad-spectrum antibiotic providing coverage for Pseudomonas is often utilized (e.g., cefepime or piperacillin-tazobactam). For patients with known MRSA or Pseudomonas colonization and severe exacerbation, use of two antibiotics may be advisable (e.g., addition of MRSA coverage or an aminoglycoside for Pseudomonas). (24992394)
- Nebulized tobramycin has been demonstrated to have a substantial microbiological effect on Pseudomonas, without inducing resistance. No evidence is available regarding its use for acute bronchiectasis exacerbations, but this seems reasonable (if well tolerated; chest tightness or cough may occur). (Fishman 2023)
- (For additional discussion about antibiotic use in bronchiectasis, please see the closely related discussion of antibiotics in CF exacerbation here 📖.)
duration of therapy
- A Delphi study of experts suggested a duration of 10-14 days. (24992394) This is consistent with usual management for cystic fibrosis exacerbation.
airway clearance therapy
chest physiotherapy
- Various mechanical airway clearance devices may be helpful, e.g.:
- Oscillating positive expiratory pressure (PEP) devices (e.g., Acapella).
- High-frequency chest wall oscillation (HFCWO, aka vest therapy)
- Mobilization and aggressive physical therapy may improve secretion clearance. For example, brisk walking may provide effective chest physiotherapy.
hypertonic saline
- inhaled hypertonic saline may improve sputum viscosity and expectoration. This may be used 2-4 times per day.
- ⚠️ DNAse is useful in cystic fibrosis. However, it has not been demonstrated to be useful in non-CF bronchiectasis (with one study suggesting that it was harmful). (9596315) Consequently DNAse is not recommended in non-CF bronchiectasis.
ambulation & exercise
- Brisk walking is an effective form of chest physiotherapy.
- Aggressive physical therapy is also globally beneficial for the patient (in terms of avoiding deconditioning that may itself exacerbate dyspnea).
additional therapies
treatment of any reversible underlying process, for example:
- Patients with immunoglobulin deficiency may benefit from immunoglobulin replacement.
- Underlying allergic bronchopulmonary aspergillosis should be managed appropriately. 📖
- Surgery may rarely be considered for focal bronchiectasis refractory to medical management.
bronchodilators
- Bronchodilators may improve obstruction and aid in the clearance of secretions.
- These may be especially useful in patients with airway hyperreactivity and reversible airflow obstruction.
steroid
- Steroid is not generally a component of therapy. A short course of steroid may be considered in patients who have a combination of bronchiectasis and asthma.
- This is generally managed the same as hemoptysis in cystic fibrosis, which is discussed further here: 📖
- One difference is that for patients with focal bronchiectasis, resective surgery may be an option (unlike patients with CF, who have diffuse disease that doesn't generally allow lung resection).
basics
- Ciliary dysfunction causes impaired mucus clearance in the bronchi, middle ear, nose, and sinuses.
- Developmental abnormalities may include situs inversus and/or complex congenital heart disease.
- Primary ciliary dyskinesia may result from a variety of different genetic mutations, leading to some variability in clinical presentation.
primary clinical features
- Organ laterality defect (~50% of patients).
- Situs inversus totalis is most common (~40% of patients).
- May also have partial laterality abnormalities.
- Complex cardiovascular abnormalities may be seen in ~4% of patients. (Teneback 2022)
- Bronchiectasis:
- Patients typically have a daily productive cough. (Teneback 2022)
- Patients tend to have frequent exacerbations. (Murray 2022)
- Upper airway involvement (otitis media, sinusitis, chronic rhinitis).
- Chronic nasal congestion and sinusitis is nearly universal.
- Reduced fertility.
- Hydrocephalus.
- Neonatal respiratory distress.
- Pectus excavatum and/or scoliosis.
CT scan findings may include:
- Atelectasis and mucus plugging.
- Bronchial wall thickening.
- Bronchiectasis:
- Predominantly in the middle and lower lobes.
- Universally present by adulthood. (Teneback 2022)
clinical evaluation for ciliary dyskinesia
- Nasal nitric oxide levels may be used as a screening test (<77 nL/min suggests dyskinesia). This seems to have high performance, but there are causes of false-positive results (e.g., cystic fibrosis, acute viral infection, blood in the nasal passages, HIV, diffuse panbronchiolitis, non-atopic sinusitis, and primary immune deficiencies). (Murray 2022; Teneback 2022)
- ⚠️ Cystic fibrosis should be excluded (using sweat testing and CFTR gene analysis) prior to nasal nitric oxide measurement. (Teneback 2022)
- Genetic testing has high specificity, but may miss some genetic abnormalities (with a sensitivity of ~95% using a 32-gene panel). (Murray 2022)
- Semen analysis may be useful in men (>50% non-motile or non-progressive sperm supports the diagnosis). (Murray 2022)
management
- Treatment generally occurs along the lines of other non-CF bronchiectasis patients, but some nuances are worth noting:
- There should be a low threshold to evaluate for intercurrent ABPA (allergic bronchopulmonary aspergillosis) if patients are deteriorating. (Teneback 2022)
- Ear involvement may cause baseline hearing deficiencies, so caution should be employed to avoid ototoxicity from aminoglycosides. (Teneback 2022)
epidemiology of BCOS
- Studies vary, but ~30% of COPD patients may have radiological bronchiectasis. (36922031, 35236556)
- BOCS seems to involve roughly equal numbers of patients with emphysema-predominant COPD or chronic bronchitis-predominant COPD. (35236556)
diagnosis of BCOS
- BCOS is diagnosed on the basis of two major features:
- (1) Clinical diagnosis of COPD – which often includes tobacco exposure, compatible symptoms, and post-bronchodilator airway obstruction (as discussed further here: 📖).
- (2) CT scan showing bronchiectasis (especially if moderate or severe).
- Other clinical features may support a diagnosis of clinically relevant BCOS:
- (1) Chronic sputum production is a hallmark of chronic bronchitis, but usually the volume of sputum is relatively low (e.g., <60 ml per day). Larger volumes of daily sputum production would support a clinical diagnosis of BCOS.
- (2) Patients with BCOS are generally sicker than most COPD patients – with higher mortality, more frequent exacerbations, and more severe exacerbations. (Teneback 2022)
- (3) BCOS is associated with higher rates of sputum positivity for pathogenic bacteria (especially Pseudomonas – which should suggest the possibility of BCOS). (36922031, 35236561)
management implications
- There is little evidence regarding the optimal therapy for BCOS. Establishing evidence may be challenging because there is likely a continuum of disease composition (e.g., some patients with predominant COPD, and other patients with predominant bronchiectasis).
- Optimal therapy might be a hybrid of COPD therapies and bronchiectasis therapies, adapted to the individual patient's specifics.
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References
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Books:
- Shah, P. L., Herth, F. J., Lee, G., & Criner, G. J. (2018). Essentials of Clinical pulmonology. In CRC Press eBooks. https://doi.org/10.1201/9781315113807
- Shepard, JO. (2019). Thoracic Imaging The Requisites (Requisites in Radiology) (3rd ed.). Elsevier.
- Walker C & Chung JH (2019). Muller’s Imaging of the Chest: Expert Radiology Series. Elsevier.
- Palange, P., & Rohde, G. (2019). ERS Handbook of Respiratory Medicine. European Respiratory Society.
- Rosado-De-Christenson, M. L., Facr, M. L. R. M., & Martínez-Jiménez, S. (2021). Diagnostic imaging: chest. Elsevier.
- Murray & Nadel: Broaddus, V. C., Ernst, J. D., MD, King, T. E., Jr, Lazarus, S. C., Sarmiento, K. F., Schnapp, L. M., Stapleton, R. D., & Gotway, M. B. (2021). Murray & Nadel’s Textbook of Respiratory Medicine, 2-Volume set. Elsevier.
- Fishman's: Grippi, M., Antin-Ozerkis, D. E., Cruz, C. D. S., Kotloff, R., Kotton, C. N., & Pack, A. (2023). Fishman’s Pulmonary Diseases and Disorders, Sixth Edition (6th ed.). McGraw Hill / Medical.
- Teneback CC and Garcia B. Bronchiectasis. Humana Press, 2022.