Chronic infectious or inflammatory diseases lead to amyloid infiltrati
on and dysfunction of many organs, including the kidney, liver, heart,
and gastrointestinal tract, Subclinical amyloid infiltration of the t
hyroid gland has been described in over 80% of such patients. However,
symptomatic involvement of the thyroid gland by amyloid is rare, We d
escribe a euthyroid patient with cystic fibrosis and widespread amyloi
dosis who presented with a rapidly enlarging goiter and symptoms of lo
cal compression that compromised his pulmonary status, Fine needle asp
iration of the goiter was nondiagnostic. At surgery he proved to have
replacement of the thyroid gland by amyloid. A review of the literatur
e reveals only five previous cases of amyloid goiter in patients with
cystic fibrosis, However, as more patients survive into adulthood, amy
loid goiter may become a more common complication of cystic fibrosis,
In contrast to other patients with reactive amyloidosis and goiter, pa
tients with cystic fibrosis may require thyroid surgery to relieve air
way compression that can compromise pulmonary function.