Systemic amyloidosis in cystic fibrosis.

We report two siblings with cystic fibrosis and systemic amyloidosis. The major 
clinical problem in both cases was recurrent respiratory infection with pulmonary 
fibrosis and bronchiectasis prior to death at ages 20 and 22 
years. Findings from postmortem examinations disclosed diffuse amyloidosis. In addition, amyloid 
infiltration developed in both patients, with enlargement of the thyroid gland, 
and one required thyroidectomy. An autopsy review of 17 additional cases 
of cystic fibrosis failed to disclose any other instances of systemic 
amyloidosis. 