Non-Cystic Fibrosis Bronchiectasis

Non-Cystic Fibrosis Bronchiectasis

Tampa General Hospital is one of the nation’s foremost academic medical institutions. The distinguished team in our Pulmonology and Lung Center includes industry-leading experts in advanced diagnostics and treatment, and we have extensive experience in treating bronchiectasis and other airway diseases.

There are two main types of bronchiectasis: non-cystic fibrosis bronchiectasis and cystic fibrosis bronchiectasis. Non-cystic fibrosis bronchiectasis is a chronic inflammatory condition that causes the lungs’ airways (bronchi) to permanently widen. The inner walls of the bronchi are normally coated with a thin layer of sticky mucus, which traps inhaled particles and prevents them from damaging the lungs. In the widened bronchi, excess mucus can build up, harbor bacteria and make the lungs more susceptible to infection.

Cystic fibrosis bronchiectasis is a genetic condition that causes the body to produce thick mucus in the lungs and other organs. The excess mucus can clog the bronchi, which can cause breathing difficulty and progressive lung damage.

What causes non-cystic fibrosis bronchiectasis?

Non-cystic fibrosis bronchiectasis can result from:

  • A previous respiratory infection – Influenza, pneumonia, whooping cough, adenovirus or tuberculosis
  • A bronchial obstruction – An inhaled foreign object or enlarged lymph gland
  • A hereditary condition – Primary ciliary dyskinesia (PCD) or Marfan syndrome
  • An autoimmune condition – Rheumatoid arthritis, systemic lupus erythematosus (SLE), Sjogren's syndrome, relapsing polychondritis or human immunodeficiency virus (HIV)
  • An inflammatory bowel disease – Crohn’s disease or ulcerative colitis
  • An inhalation injury – Breathing in noxious gases or fumes

In some cases, a specific cause cannot be determined.

What are the signs of non-cystic fibrosis bronchiectasis?

The symptoms of non-cystic fibrosis bronchiectasis can take months or years to develop and usually worsen over time. The primary symptom is a persistent cough that produces phlegm (sputum). Other symptoms can include:

  • Shortness of breath
  • Wheezing
  • Coughing up blood
  • Chest pain due to the increased effort needed to breathe
  • Fever and chills
  • Fatigue
  • Clubbing (skin thickening under the nails)

The symptoms can vary in severity and typically worsen if a lung infection develops.

How is non-cystic fibrosis bronchiectasis diagnosed?

If non-cystic fibrosis bronchiectasis is suspected, a physician will typically perform a medical history review and order tests to check for underlying conditions. Because non-cystic fibrosis bronchiectasis is an airway disease, the physician will also perform lung function testing. Additionally, a sputum culture can identify a bacterial infection in the lungs.

To confirm or rule out a diagnosis of non-cystic fibrosis bronchiectasis, the physician will order a chest X-ray or CT scan. Both tests create detailed images of the lungs, airways and heart.

How is non-cystic fibrosis bronchiectasis treated?

The goals of treatment are to control the symptoms, prevent lung infections and improve quality of life. This may involve a combination of:

  • Oral or intravenous antibiotics
  • Mucus-thinning medications
  • Airway clearance techniques (chest physical therapy)
  • Handheld airway clearance devices, such as oscillating positive expiratory pressure (PEP), intrapulmonary percussive ventilation (IPV) and postural drainage
  • Avoidance of airborne irritants, such as tobacco smoke

Receive comprehensive care and support at TGH

TGH’s highly qualified medical team is committed to improving the lives of patients who are living with pulmonary and lung conditions, such as non-cystic fibrosis bronchiectasis.

Contact us at 800-822-3627 to request a consultation with a member of our team.