MANAGEMENT OF ENDOCRINE DISEASE: Cushing's syndrome: a structured short- and long-term management plan for patients in remission

Abstract

One hundred years have passed since Harvey Williams Cushing presented the first patient with the syndrome that bears his name. In patients with Cushing's syndrome (CS), body composition and lipid, carbohydrate and protein metabolism are dramatically affected and psychopathology and cognitive dysfunction are frequently observed. Untreated patients with CS have a grave prognosis with an estimated 5-year survival of only 50%. Remission can be achieved by surgery, radiotherapy and sometimes with medical therapy. Recent data indicate that the adverse metabolic consequences of CS are present for years after successful treatment. In addition, recent studies have demonstrated that health-related quality of life and cognitive function are impaired in patients with CS in long-term remission. The focus of specialised care should therefore be not only on the diagnostic work-up and the early postoperative management but also on the long-term follow-up. In this paper, we review the long-term consequences in patients with CS in remission with focus on the neuropsychological effects and discuss the importance of these findings for long-term management. We also discuss three different phases in the postoperative management of surgically-treated patients with CS, each phase distinguished by specific challenges: the immediate postoperative phase, the glucocorticoid dose tapering phase and the long-term management. The focus of the long-term specialised care should be to identify cognitive impairments and psychiatric disorders, evaluate cardiovascular risk, follow pituitary function and detect possible recurrence of CS.

Using subscales when scoring the Cushing's quality of life questionnaire

“…If transsphenoidal surgery for ACTH-dependent CD leaves a residual mass, radiotherapy or medical therapy might be the treatment of choice. Medical therapy is also an optionsymptoms regress substantially, although QoL typically does not return to premorbid levels (8,9). Because impaired QoL is a persistent complaint from patients in remission from CS, it is important to accurately assess which aspects of QoL are affected in order to develop more effective patient interventions.…”

The association between urinary cortisol excretion and cardiovascular risk factors, bone status and quality of life in the population

“…Patients with Cushinǵ s syndrome have increased cardiovascular risk [3], decreased bone mineral density, increased fracture risk [4,5], cognitive dysfunction and impaired quality of life (QoL) [6,7]. Even mildly, pathologically increased endogenous cortisol production has negative impact on QoL [8], cardiovascular [9] and skeletal health [10,11].…”

Mental fatigue and executive dysfunction in patients with Cushing's syndrome in remission

“…The MFS is possibly better than general QoL questionnaires since it captures symptoms that are of great importance for general health in patients treated for CS and provides at least additional and valuable information on the patients' wellbeing in comparison to the disease specific questionnaire [15]. In addition, one of the aims in the longitudinal follow-up of patients treated for CS is to improve QoL [4]. The MFS is probably a valuable instrument for this purpose and can be used to detect mental fatigue at diagnosis and during the follow-up to objectively compare changes before and after treatment.…”

“…Despite remission, as defined by biochemical control of the disease after treatment, a large number of patients suffer from impaired quality of life, fatigue, and cognitive dysfunction [3][4][5][6][7]. Also, van Aken et al have shown that 45% of patients with CD in remission are depressed [8].…”