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Incidence and Late Prognosis of Cushing’s Syndrome: A Population-Based Study¹

Department of Medicine, Holstebro Hospital (J.L.), 7500 Holstebro; Department of Epidemiology and Social Medicine, Aarhus University (S.J.), and Departments of Medicine and Endocrinology (J.O.L.J., J.W.) and Neurosurgery (J.A.), Aarhus University Hospital, 8000 Aarhus; Departments of Neurosurgery (P.B.) and Endocrinology (C.H.), Odense University Hospital, 5000 Odense; Departments of Endocrinology and Neurosurgery (M.K.), Copenhagen University Hospital (U.F.R.), 2100 Copenhagen; Departments of Neurosurgery (J.J.) and Endocrinology (P.L.), Aalborg Hospital, 9000 Aalborg; Department of Medicine and Endocrinology, Herlev University Hospital (L.Ø.K.), 2730 Herlev; and Department of Neurosurgery, Glostrup University Hospital (K.S.), 2600 Glostrup, Denmark

Address all correspondence and requests for reprints to: Dr. J. Lindholm, Department of Medicine, Holstebro Hospital, 7500 Holstebro, Denmark. E-mail: j.lindholm{at}forum.dk

The main purpose was to assess the incidence and late outcome of Cushing’s syndrome, particularly in Cushing’s disease. Information for all patients diagnosed with Cushing’s syndrome during an 11-yr period in Denmark was retrieved. The incidence was 1.2–1.7/million·yr (Cushing’s disease), 0.6/million·yr (adrenal adenoma) and 0.2/million·yr (adrenal carcinoma). Other types of Cushing’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), yielding 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 within the first year after diagnosis. Eight patients died before treatment could 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 studied separately, including a questionnaire on their perceived quality of health. In 45 patients with Cushing’s disease who had been cured through transsphenoidal neurosurgery, only 1 had died (SMR, 0.31; CI, 0.01–1.72) compared with 6 of 20 patients with persistent hypercortisolism after initial neurosurgery (SMR, 5.06; CI, 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 Cushing’s disease and appeared independent of disease control or presence of hypopituitarism. It is concluded that 1) Cushing’s syndrome is rare and is associated with increased mortality, in patients with no concurrent malignancy also; 2) the excess mortality was mainly observed during the first year of disease; and 3) the impaired quality of health in long-term survivors of Cushing’s disease is not fully explained.

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