Williams-Campbell syndrome

Williams-Campbell syndrome is a rare congenital (present from birth) lung condition in which the cartilage that normally supports the medium-sized airways (bronchi) of the lungs is missing or severely underdeveloped. Cartilage acts like a scaffold that keeps your airways open, especially when you breathe out. Without it, the affected airways become floppy and tend to balloon out (a condition called bronchiectasis) and then collapse during breathing. This leads to air trapping, chronic coughing, repeated lung infections, and progressive breathing difficulties. The main symptoms include a persistent productive cough (often with mucus), wheezing, shortness of breath, and frequent respiratory infections such as pneumonia or bronchitis. Over time, the repeated infections and airway damage can worsen lung function. Some patients may also experience poor growth in childhood and reduced exercise tolerance. There is currently no cure for Williams-Campbell syndrome. Treatment focuses on managing symptoms and preventing complications. This includes chest physiotherapy (airway clearance techniques), prompt treatment of lung infections with antibiotics, bronchodilator medications to help open the airways, and in some cases supplemental oxygen. Vaccinations against influenza and pneumococcal disease are strongly recommended. In very severe cases, lung transplantation may be considered. Early diagnosis and consistent management can help slow the progression of lung damage and improve quality of life.

Bring these to your next appointment

• Q1.How severe is the airway involvement, and which parts of the lungs are most affected?,What airway clearance techniques do you recommend, and how often should they be done?,Should my child or I be on preventive (prophylactic) antibiotics to reduce infections?,How often should lung function tests and CT scans be repeated to monitor progression?,Are there any activities or environments I should avoid to protect my lungs?,At what point would you consider referring for lung transplant evaluation?,Are there any clinical trials or research studies I could participate in?

Common questions about Williams-Campbell syndrome

Related conditions

Other rare diseases commonly researched alongside Williams-Campbell syndrome.

• Cystic fibrosis→
• Idiopathic bronchiectasis→
• Primary ciliary dyskinesia→