Affected patients present with a nonproductive
cough that is associated with dyspnea, chest pain, and cyanosis. The disease
evolves over many years toward hypoxemia, respiratory failure, and right
heart failure. It can be exacerbated by spontaneous pneumothorax and
recurrent infections. Clubbing and pulmonary osteoarthropathy are usually
present. Patients become very disabled in the third or fourth decade, and
death usually occurs in their 50s or earlier if they are victims of
complications.