Long-Term Impaired Quality of Life in Cushing’s Syndrome despite Initial Improvement after Surgical Remission

Reproductive Biology and Medicine Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Clinical Research Center, Room 1-3140, 10 Center Drive, Bethesda, MD 20892-1109, USA.
Journal of Clinical Endocrinology &amp Metabolism (Impact Factor: 6.21). 03/2006; 91(2):447-53. DOI: 10.1210/jc.2005-1058
Source: PubMed


Cushing's syndrome (CS) is associated with symptoms that may impair health-related quality of life (HRQL).
We used the short-form 36 survey to evaluate HRQL in 23 patients with Cushing's disease before and after transsphenoidal surgery (age, 42.7 +/- 12.0 yr; 19 women and four men) and in a cross-section of 343 CS patients (age, 48.2 +/- 14.1 yr; 265 women and 78 men) in remission for up to 25.8 yr after surgery (adrenal, 5%; ectopic, 6%). The z-scores were calculated for short-form 36 domains, and physical (PCS) and mental (MCS) summary scores were compared with those of age- and sex-matched controls (n = 6742).
Active Cushing's disease was associated with low PCS and MCS scores (P < 0.05). Despite residual postoperative impairment, primarily of physical domains, all HRQL parameters improved after treatment with transsphenoidal surgery (3.2 +/- 1.5 yr; P < 0.05). In the cross-section in remission at follow-up, there was a small, but significant (P < 0.05), impairment of both PCS and MCS. A longitudinal postoperative analysis confirmed stable, but impaired, HRQL over time. Logistic regression demonstrated that previous pituitary radiation and current glucocorticoid use had little effect on HRQL outcomes.
CS is associated with impaired HRQL, which partially resolves after treatment. At longer-term follow-up, however, there is residual impairment of HRQL. Determination of modifiable factors that contribute to impaired HRQL may help reduce the physical and psychosocial burden of this disease.

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    • "In relation to the measurement of change over time with these instruments, it is of note that earlier studies suggested a ‘ceiling’ for HRQoL in CS patients, at least using a generic HRQoL instrument [24]. It would be interesting to determine whether a similar effect exists with disease-specific instruments, as may be suggested by the fact that both pasireotide and TSS appeared to achieve similar CushingQoL scores after 6 months. "
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    ABSTRACT: Cushing's syndrome (CS) has a considerable negative impact on patient health-related quality of life (HRQoL). Two disease-specific instruments (the CushingQoL and the Tuebingen CD-25 questionnaire) are now available to assess the impact of the disease and its treatment on HRQoL. The purpose of this review was to summarize the characteristics of the studies which have used these two instruments to date and summarize their findings regarding (a) the determinants of disease-specific HRQoL in patients with CS and (b) the impact of treatment for CS on disease-specific HRQoL. A total of 7 studies were identified, 5 with the CushingQoL and 2 with the Tuebingen CD-25. Most were observational studies, though the CushingQoL had been used in one randomized clinical trial. In terms of clinical factors, there was some evidence for an association between UFC levels and disease-specific HRQoL, though the presence and strength of the association varied between studies. There was also some evidence that a more recent diagnosis of CS could lead to poorer HRQoL, and that length of time with adrenal insufficiency may also affect HRQoL. There was no evidence for an impact on disease-specific HRQoL of etiology or of the clinical signs and symptoms associated with CS, such as bruising, rubor, and fat deposits. One factor which did have a significant negative effect on HRQoL was the presence of depression. No clear picture emerged as to the effect of demographic variables such as age and gender on HRQoL scores, though there was some evidence for poorer HRQoL in female patients. As regards treatment, the two interventions studied to date (transsphenoidal surgery and pasireotide) both showed significant gains in HRQoL, with moderate to large effect sizes. This type of review is useful in summarizing knowledge to date and suggesting future research directions.
    Pituitary 04/2013; 17(2). DOI:10.1007/s11102-013-0484-2 · 3.20 Impact Factor
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    • "Successful treatment of CD often ameliorates clinical symptoms and leads to an improvement in health-related quality of life (HRQOL). Published studies have reported significant improvements in patients’ postoperative physical and mental functioning [5, 6]. "
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    ABSTRACT: Objectives To construct a prediction model of preference-adjusted health status (SF-6D) for Cushing’s syndrome using a disease-specific health-related quality of life (HRQOL) measure (CushingQoL). Methods Data were obtained from the original multicenter, multinational study to validate the CushingQoL questionnaire. HRQOL was measured using the CushingQoL and the SF-36 questionnaires. SF-6D scores were calculated from responses on the SF-36. Sociodemographic and clinical data were also collected. Various predictive models were tested and the final one was selected on the basis of four criteria: explanatory power, consistency of estimated coefficients, normality of prediction errors, and parsimony. Results For the mapping analysis, data were available from 116 of the 125 patients included in the original validation study. Mean (SD) age was 45.3 (13.1) years and the sample was predominantly (83 %) female. Patients had a mean (SD) CushingQoL score of 52.9 (21.9), whereas the SF-6D (derived from SF-36) was skewed towards better health with a mean of 0.71 (median 0.74) on a scale of −0.704 to 1. Of the various models tested, a model which included the intercept (0.61), CushingQoL overall score, level one in CushingQoL item 2 (always have pain preventing me from leading a normal life), and level one in CushingQoL item 10 (my illness always affects my everyday activities) best met the four criteria for model selection. The model had an adjusted R2 of 0.60 and a root mean square error of 0.084. Conclusions Although the mapping function finally selected appears to be able to accurately map CushingQoL scores onto SF-6D outcomes at the group level, further testing is required to validate the model in independent patient samples.
    The patient 04/2013; 6(2). DOI:10.1007/s40271-013-0010-7 · 1.90 Impact Factor
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    • "Adjunctive treatments following surgical management may include medical therapy, radiosurgery or other radiation-based treatments, and/or bilateral adrenalectomy. Despite improvements in overall survival and quality of life following multimodality treatment and achievement of hormonal remission, many patients with treated Cushing's Disease continue to have persistently diminished quality of life, as compared to normal age- and sex-matched controls [10, 29, 30]. "
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    ABSTRACT: Cushing's Disease is caused by oversecretion of ACTH from a pituitary adenoma and results in subsequent elevations of systemic cortisol, ultimately contributing to reduced patient survival. The diagnosis of Cushing's Disease frequently involves a stepwise approach including clinical, laboratory, neuroimaging, and sometimes interventional radiology techniques, often mandating multidisciplinary collaboration from numerous specialty practitioners. Pituitary microadenomas that do not appear on designated pituitary MRI or dynamic contrast protocols may pose a particularly challenging subset of this disease. The treatment of Cushing's Disease typically involves transsphenoidal surgical resection of the pituitary adenoma as a first-line option, yet may require the addition of adjunctive measures such as stereotactic radiosurgery or medical management to achieve normalization of serum cortisol levels. Vigilant long-term serial endocrine monitoring of patients is imperative in order to detect any recurrence that may occur, even years following initial remission. In this paper, a stepwise approach to the diagnosis, and various management strategies and associated outcomes in patients with Cushing's Disease are discussed.
    International Journal of Endocrinology 01/2013; 2013(2):893781. DOI:10.1155/2013/893781 · 1.95 Impact Factor
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