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Bronchiectasis

Bronchiectasis is a chronic lung disease involving permanent airway dilation and scarring, resulting in mucus buildup, recurrent infections, inflammation, and often stemming from lung infections or underlying medical conditions.

Pulmonary
Bronchiectasis is a chronic lung condition characterized by permanent widening (dilation) and scarring of the airways (bronchi). This damage leads to a buildup of mucus, making the lungs more susceptible to infection and inflammation. Over time, recurring infections and inflammation can further damage the airways, creating a cycle that worsens the condition. Causes: Bronchiectasis typically develops due to a severe lung infection or an underlying medical condition that damages the airways or impairs the body's ability to clear mucus.
  • Severe Lung Infections (often the initiating event):
    • Severe pneumonia (especially viral or bacterial infections like adenovirus, influenza, pertussis, or tuberculosis).
    • Complications from measles or whooping cough (pertussis) in childhood.
  • Cystic Fibrosis (CF): The most common genetic cause of bronchiectasis in the developed world. CF causes thick, sticky mucus to block airways, leading to chronic infections and damage.
  • Alpha-1 Antitrypsin Deficiency: A genetic disorder that can lead to lung damage, including emphysema and bronchiectasis.
  • Immune System Deficiencies: Conditions that weaken the immune system, making individuals more prone to recurrent infections (e.g., common variable immunodeficiency (CVID), HIV/AIDS, specific antibody deficiencies).
  • Allergic Bronchopulmonary Aspergillosis (ABPA): An allergic reaction to Aspergillus fungus that colonizes the airways, causing severe inflammation and damage.
  • Connective Tissue Diseases: Autoimmune disorders like rheumatoid arthritis, Sjögren's syndrome, lupus, or inflammatory bowel disease can cause lung inflammation and lead to bronchiectasis.
  • Ciliary Dyskinesia Syndromes (e.g., Primary Ciliary Dyskinesia - PCD): Inherited disorders where the cilia (tiny hair-like structures that clear mucus) don't function properly.
  • Gastroesophageal Reflux Disease (GERD): Chronic aspiration of stomach acid into the lungs can cause inflammation and damage.
  • Inhaled Foreign Body: A foreign object accidentally inhaled into the airways can cause localized obstruction and recurrent infection.
  • Chronic Obstructive Pulmonary Disease (COPD): While distinct, severe COPD can sometimes be accompanied by bronchiectasis, often referred to as "COPD-bronchiectasis overlap syndrome."
  • Non-Tuberculous Mycobacterial (NTM) Infections: Certain types of mycobacteria (not causing tuberculosis) can cause chronic lung infections that lead to bronchiectasis.
Symptoms: Symptoms of bronchiectasis often develop gradually over months or years. They can fluctuate in severity and are characterized by a chronic cough and recurrent respiratory infections.
  • Chronic cough: Persistent cough that produces large amounts of sputum (phlegm), often thick and discolored (yellow, green, or even bloody).
  • Shortness of breath (dyspnea): Especially during physical activity.
  • Wheezing.
  • Chest pain or discomfort.
  • Recurrent respiratory infections: Frequent bouts of bronchitis or pneumonia.
  • Fatigue.
  • Weight loss (in severe cases).
  • Clubbing of fingers and toes: Enlargement of the fingertips and toes, less common but can occur in severe, long-standing cases.
Diagnosis: Diagnosing bronchiectasis typically involves a combination of medical history, physical examination, and specific imaging and lung function tests.
  • Medical History and Physical Exam: The doctor will ask about your symptoms, history of lung infections, smoking history, and family history. They will listen to your lungs for crackles, rhonchi (rattling sounds), or wheezing.
  • High-Resolution Computed Tomography (HRCT) Scan of the Chest: This is the gold standard for diagnosing bronchiectasis. It provides detailed images of the airways, showing the characteristic widening and scarring.
  • Sputum Culture: A sample of mucus is tested to identify the specific bacteria or fungi causing recurrent infections, which helps guide antibiotic treatment.
  • Pulmonary Function Tests (PFTs): Measure lung function (e.g., spirometry) to assess airflow obstruction and lung volumes.
  • Blood Tests: To check for underlying causes such as:
    • Alpha-1 antitrypsin deficiency.
    • Immune deficiencies (immunoglobulin levels).
    • Cystic fibrosis gene mutations.
    • Allergy testing for ABPA.
    • Tests for autoimmune diseases.
  • Bronchoscopy: A procedure where a thin, flexible tube with a camera is inserted into the airways to visualize them directly, obtain samples, or remove foreign bodies. This is done if a localized cause is suspected or for difficult-to-diagnose infections.
Treatment: Treatment for bronchiectasis aims to manage symptoms, prevent exacerbations (flare-ups), treat infections, and slow the progression of lung damage. It often involves a long-term, comprehensive approach.
  • Airway Clearance Techniques: These are essential to help remove excess mucus from the airways.
    • Chest Physiotherapy (CPT): Manual techniques (clapping, percussion) or mechanical devices (e.g., vibrating vests, oscillatory PEP devices) to loosen and move mucus.
    • Controlled breathing techniques.
  • Medications:
    • Antibiotics: To treat acute infections. Long-term (prophylactic) or inhaled antibiotics may be used for frequent infections.
    • Bronchodilators: Inhaled medications that open up the airways (similar to asthma medications) to improve airflow and mucus clearance.
    • Mucolytics: Medications (e.g., hypertonic saline, dornase alfa (for CF-related bronchiectasis)) that thin mucus, making it easier to clear.
    • Anti-inflammatory medications: Inhaled corticosteroids may be used, particularly if there's co-existing asthma or COPD.
    • Corticosteroids (oral): Short courses may be used for severe flare-ups.
    • Specific treatments for underlying causes: (e.g., antifungal medications for ABPA, enzyme replacement for CF, immunoglobulin replacement for immune deficiencies).
  • Lifestyle Management:
    • Quit smoking: Essential to prevent further lung damage.
    • Regular exercise: Helps improve lung function and overall health.
    • Hydration: Drinking plenty of fluids can help thin mucus.
    • Vaccinations: Annual flu and pneumococcal vaccines to prevent respiratory infections.
  • Oxygen Therapy: May be needed for individuals with low blood oxygen levels.
  • Surgery: In rare cases, surgery may be considered to remove a severely damaged portion of the lung (e.g., if damage is localized) or for a lung transplant in very severe, end-stage cases.
Long-term management and consistent adherence to airway clearance techniques are crucial for individuals with bronchiectasis to reduce symptoms, prevent infections, and improve quality of life.

Related Medications

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