Bronchiectasis

Bronchiectasis is a chronic lung condition that occurs when the tubes (bronchi) that carry air to your lungs are damaged. This damage causes them to widen permanently and fill with mucus, making it harder to clear germs and increasing the risk of infection. This is a common condition that some people are born with, while others develop later in life.

Pulmonologists, specialists in the respiratory system, diagnose and treat lung conditions like bronchiectasis. At Penn Medicine, pulmonary specialists in chronic lung disorders care treat all types of lung disease.

Pulmonologists classify bronchiectasis based on how and where the airways are damaged:

• Cylindrical bronchiectasis: Also called tubular bronchiectasis, it’s the most common and least severe type. The airways are widened but still mostly retain their shape.
• Varicose bronchiectasis: The airways have an irregular, stretched look, similar to varicose veins.
• Cystic bronchiectasis: This most severe type of bronchiectasis occurs when the airways form large, cyst-like sacs filled with mucus.
• Focal bronchiectasis: This happens when a large airway is blocked, often due to swollen lymph nodes from a mycobacterial infection.
• Diffuse bronchiectasis: In this case, damage affects multiple areas in both lungs, leading to permanent widening. Inflammation can spread and cause scarring in nearby lung tissue.
• Traction bronchiectasis: Scarring in the lungs pulls the airways out of shape. It’s often seen in interstitial lung diseases (ILD) and usually happens at the lung’s outer edges, where the bronchi have less support.

Bronchiectasis symptoms develop gradually and may appear months or even years after the condition begins. The main symptom is a chronic cough that produces large amounts of foul-smelling phlegm. Other symptoms may include:

• Frequent lung infections, coughing, and trouble breathing
• Shortness of breath that worsens with exercise
• Wheezing
• Fatigue and weight loss
• Breath odor
• Coughing up blood (less common in children)
• Low-grade fever and night sweats
• Pale skin
• Clubbing of the fingers (rare and depends on the cause)

Bronchiectasis develops when a cycle of inflammation, airway damage, poor mucus clearance, and infections leads to permanent lung changes.

It can start in childhood after a severe lung infection or result from inhaling a foreign object or food particles. Cases where the cause is unknown are called idiopathic bronchiectasis.

Other possible causes include:

• Cystic fibrosis, which leads to thick, sticky mucus buildup in the lungs
• Rheumatoid arthritis, Sjögren syndrome, inflammatory bowel disease, or other autoimmune disorders
• Allergic lung diseases, like allergic bronchopulmonary aspergillosis (ABPA)
• Certain cancers, including leukemia 
• Immune deficiency syndromes that weaken the body’s ability to fight infections
• Primary ciliary dyskinesia, a genetic condition that affects airway clearance
• Infections with non-tuberculous mycobacteria
• Serious lung diseases like bronchiolitis obliterans, which can lead to scarring
• Asthma or chronic obstructive pulmonary disease (COPD) 

To diagnose bronchiectasis, your provider may recommend tests to check for infections, immune deficiencies, or underlying conditions. There are a variety of specialized tests, including imaging tests, that can help pinpoint a diagnosis.

Tests include:

• Complete blood count (CBC)
• Immunoglobulin test
• Sputum culture
• PPD skin test
• Pulmonary function tests
• Genetic tests
• Chest x-ray
• Computed tomography (CT) scan

You may not be able to prevent bronchiectasis completely, but you can reduce your risk by promptly treating lung infections. Childhood vaccines, the yearly flu shot, and COVID-19 vaccines can help prevent certain infections.

Quitting smoking can also reduce your risk. The Harron Lung Center’s comprehensive Stop Smoking Program provides a supportive, encouraging environment to help you quit.

Managing a chronic condition like bronchiectasis requires a coordinated approach from several specialists. At Penn Medicine, our bronchiectasis, mycobacterial lung disease, and chronic lung infection departments are staffed by physicians, nurses, respiratory therapists, and pharmacists specializing in pulmonary medicine and infectious diseases. These integrated teams work together to provide advanced diagnostic testing and expert guidance on the best ways to treat lung infections.

Penn Medicine’s Harron Lung Center offers more leading-edge treatment options than any other lung program in the southeastern Pennsylvania and New Jersey regions. Many people seek our expert care for mild to severe lung conditions.