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Related Subjects: Asthma |Acute Severe Asthma |Exacerbation of COPD |Pulmonary Embolism |Cardiogenic Pulmonary Oedema |Pneumothorax |Tension Pneumothorax |Respiratory (Chest) infections Pneumonia |Fat embolism |Hyperventilation Syndrome |ARDS |Respiratory Failure |Diabetic Ketoacidosis
Chronic destructive infective inflammatory disorder of airways. 50% are idiopathic
Cause | Description | Comments |
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Infections | Chronic or recurrent respiratory infections, such as TB, pneumonia, or whooping cough. | Infections can cause persistent inflammation, leading to damage and dilation of the airways. |
Cystic Fibrosis | A genetic disorder that causes thick, sticky mucus to build up in the lungs, leading to chronic infections and bronchiectasis. | Cystic fibrosis is the most common genetic cause of bronchiectasis. |
Primary Ciliary Dyskinesia (PCD) | A genetic disorder affecting the cilia, leading to impaired mucus clearance from the respiratory tract. | Impaired ciliary function leads to recurrent infections and bronchiectasis over time. |
Immune Deficiencies | Conditions like common variable immunodeficiency (CVID) or HIV/AIDS that weaken the immune system, making patients more susceptible to infections. | Frequent and severe infections in immune-compromised individuals can lead to bronchiectasis. |
Allergic Bronchopulmonary Aspergillosis (ABPA) | An allergic reaction to the fungus Aspergillus that can cause inflammation and damage to the airways. | Common with asthma or cystic fibrosis. |
Autoimmune Diseases | Conditions such as rheumatoid arthritis, Sjögren's syndrome, or inflammatory bowel disease (IBD) can cause inflammation in the lungs. | Can damage the airways and lead to bronchiectasis. |
Congenital Malformations | Conditions present at birth, such as bronchial atresia or congenital tracheobronchomegaly (Mounier-Kuhn syndrome). | Predispose individuals to infections and subsequent bronchiectasis. |
Inhalation of Toxic Substances | Chemicals, smoke, or foreign bodies can cause direct injury to the airways. | Acute or chronic exposure can lead to airway damage and bronchiectasis. |
Post-Obstructive Bronchiectasis | Obstruction of the airway by tumour, foreign body, or enlarged lymph nodes can lead to localized bronchiectasis. Worldwide the commonest cause is Tuberculosis | Occurs due to persistent obstruction, leading to impaired clearance and infection. |
Idiopathic | No identifiable cause; the condition develops without a known underlying trigger. | Idiopathic bronchiectasis accounts for a significant proportion of cases, particularly in older adults. |
Test | Description | Notes |
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Bloods | Raised WCC, ESR, CRP suggests acute infection. | Treat infection |
CXR | Consolidation, fibrosis, tram lines and signet ring signs | Treat infection |
High-Resolution CT (HRCT) Scan | Provides detailed images of the lungs, allowing visualization of the bronchial walls and the extent of bronchiectasis. | Gold standard for diagnosing bronchiectasis; reveals airway dilation, wall thickening, and mucus plugging. |
Spirometry | Measures lung function, specifically the volume of air that can be exhaled in one second (FEV1) and total lung capacity (TLC). | Used to assess the degree of airway obstruction and monitor lung function over time. |
Sputum Culture | Analyses sputum samples for bacteria, fungi, and mycobacteria to identify infectious agents. Organism include Haemophilus influenzae, Streptococcus pneumonia, Staphylococcus aureus, Moraxella catarrhalis, Pseudomonas aeruginosa (poorer prognosis) | Helps guide antibiotic therapy, especially in patients with recurrent infections or those not responding to initial treatments. | Aspergillus | Aspergillus precipitins. | Treat if positive |
Bronchoscopy | A procedure using a bronchoscope to directly visualize the airways and collect samples for further analysis. | Useful for identifying airway obstructions, obtaining biopsies, or removing foreign bodies. |
Blood Tests | Includes complete blood count (CBC), immunoglobulin levels, and specific tests for autoimmune diseases or allergies. | Helps identify underlying causes such as immune deficiencies, autoimmune conditions, or allergic bronchopulmonary aspergillosis (ABPA). |
Genetic Testing | Tests for specific genetic mutations associated with conditions like cystic fibrosis or primary ciliary dyskinesia. | Indicated in patients with early onset of bronchiectasis or a family history of genetic disorders. |
Alpha-1 Antitrypsin Level | Measures the level of alpha-1 antitrypsin, a protein that protects the lungs from damage. | Low levels are associated with alpha-1 antitrypsin deficiency, a genetic cause of bronchiectasis. |
Immunoglobulin Levels | Measures levels of IgG, IgA, and IgM to assess for immunodeficiencies. | Low levels may indicate an immune deficiency, increasing susceptibility to infections that can lead to bronchiectasis. |
Sweat Chloride Test | Measures the concentration of chloride in sweat, used to diagnose cystic fibrosis. | Indicated in children and young adults with bronchiectasis, particularly if there is a history of chronic lung disease and pancreatic insufficiency. |
Autoimmune Screening | Includes tests for rheumatoid factor (RF), antinuclear antibodies (ANA), and other markers of autoimmune diseases. | Used to identify underlying autoimmune conditions such as rheumatoid arthritis or Sjögren's syndrome, which can cause bronchiectasis. |
Management Option | Description | Comments |
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Airway Clearance Techniques | Includes chest physiotherapy, postural drainage, and the use of devices like oscillating positive expiratory pressure (PEP) devices to help clear mucus from the lungs. | Regular airway clearance is essential to reduce mucus build-up and prevent infections. |
Bronchodilators | Inhaled medications such as short-acting beta agonists (SABA) and long-acting beta agonists (LABA) to help open the airways and improve breathing. | Often used in patients with coexisting asthma or COPD to relieve symptoms of airway obstruction. |
Antibiotics | Used to treat acute exacerbations caused by bacterial infections. Long-term or rotating antibiotic therapy may be considered in patients with frequent exacerbations. | Choice of antibiotic is guided by sputum culture results; oral, inhaled, or intravenous antibiotics may be used depending on severity. |
Inhaled Corticosteroids | May be used in patients with coexisting asthma or COPD to reduce airway inflammation. | Not typically used as a primary treatment for bronchiectasis but may help in specific cases with significant airway inflammation. |
Vaccinations | Annual influenza vaccination and pneumococcal vaccination to prevent respiratory infections. | Vaccinations are important preventive measures to reduce the risk of infections that can exacerbate bronchiectasis. |
Surgery | Considered in localized bronchiectasis or in cases where there is persistent infection or significant bleeding that does not respond to medical treatment. | Surgical resection of the affected lung area may be performed; however, this is reserved for specific cases. |
Management of Underlying Conditions | Treatment of underlying conditions such as cystic fibrosis, primary ciliary dyskinesia, or immunodeficiency disorders. | Addressing the root cause is essential to preventing further lung damage and managing bronchiectasis effectively. |
Oxygen Therapy | Used in patients with advanced bronchiectasis and chronic respiratory failure to maintain adequate oxygen levels. | Long-term oxygen therapy may be required in patients with severe hypoxemia. |
Pulmonary Rehabilitation | A program of exercise, education, and support designed to help patients improve their physical conditioning and manage their symptoms. | Improves exercise capacity, reduces symptoms, and enhances quality of life in patients with chronic lung diseases. |
Nutritional Support | Ensuring adequate nutrition, particularly in patients with significant weight loss or malnutrition. | May involve dietary counselling, supplements, or in severe cases, enteral feeding. |
Pathogen | Commonest Causes | First-line Antibiotic Choice | Alternative Antibiotic Choices |
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Haemophilus influenzae | A common cause of respiratory infections in bronchiectasis patients. | Amoxicillin | Doxycycline or Co-amoxiclav if resistant or allergic to penicillin. |
Pseudomonas aeruginosa | Associated with more severe disease and frequent exacerbations. | Ciprofloxacin (oral) or Ceftazidime (IV) | Tobramycin (inhaled), Piperacillin-Tazobactam (IV), or Meropenem (IV) for resistant cases. |
Staphylococcus aureus | MSSA and MRSA. | Flucloxacillin for MSSA; Vancomycin or Linezolid for MRSA | Doxycycline or Co-trimoxazole for MRSA in certain cases. |
Moraxella catarrhalis | Commonly in bronchiectasis. | Amoxicillin or Doxycycline | Co-amoxiclav or Levofloxacin in cases of resistance. |
Klebsiella pneumoniae | Less common but significant in severe or hospital-acquired infections. | Co-amoxiclav or Cefuroxime | Meropenem or Ciprofloxacin in severe or resistant cases. |
Streptococcus pneumoniae | Leading cause of respiratory infections. | Amoxicillin or Penicillin V | Doxycycline or Levofloxacin if penicillin-resistant. |
Mycobacterium avium complex (MAC) | Atypical mycobacteria that can cause chronic infection in bronchiectasis patients. | Clarithromycin or Azithromycin combined with Ethambutol and Rifampicin | Consider adding Amikacin (IV) in refractory cases. |