The main purpose was to assess the incidence and late outcome of Gushing's
syndrome, particularly in Gushing's disease. Information for all patients d
iagnosed with Gushing's syndrome during an 11-yr period in Denmark was retr
ieved. The incidence was 1.2-1.7/ million yr (Gushing's disease), 0.6/milli
on.yr (adrenal adenoma) and 0.2/million.yr (adrenal carcinoma). Other types
of Gushing's syndrome were rare. In 139 patients with nonmalignant disease
, 11.1% had died during follow-up (median, 8.1 yr; range, 3.1-14.0), yieldi
ng a standard mortality ratio (SMR) of 3.68 [95% confidence interval (CI),
2.34-5.33]. The SMR was partly attributable to an increased mortality withi
n the first year after diagnosis. Eight patients died before treatment coul
d be undertaken. The prognosis in patients with malignant disease was very
poor.
Patients in whom more than 5 yr had elapsed since initial surgery were stud
ied separately, including a questionnaire on their perceived quality of hea
lth. In 45 patients with Gushing's disease who had been cured through trans
sphenoidal neurosurgery, only 1 had died (SMR, 0.31; CI, 0.01-1.72) compare
d with 6 of 20 patients with persistent hypercortisolism after initial neur
osurgery (SMR, 5.06; GI, 1.86-11.0). In patients with adrenal adenoma, SMR
was 3.95 (CI, 0.81-11.5). The perceived quality of health was significantly
impaired only in patients with Gushing's disease and appeared independent
of disease control or presence of hypopituitarism. It is concluded that 1)
Gushing's syndrome is rare and is associated with increased mortality, in p
atients with no concurrent malignancy also; 2) the excess mortality was mai
nly observed during the first year of disease; and 3) the impaired quality
of health in long-term survivors of Gushing's disease is not fully explaine
d.