Incidence and late prognosis of Cushing's syndrome: A population-based study

Citation
J. Lindholm et al., Incidence and late prognosis of Cushing's syndrome: A population-based study, J CLIN END, 86(1), 2001, pp. 117-123
Citations number
32
Categorie Soggetti
Endocrynology, Metabolism & Nutrition","Endocrinology, Nutrition & Metabolism
Journal title
JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM
ISSN journal
0021972X → ACNP
Volume
86
Issue
1
Year of publication
2001
Pages
117 - 123
Database
ISI
SICI code
0021-972X(200101)86:1<117:IALPOC>2.0.ZU;2-T
Abstract
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.