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 & 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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    • "Las operaciones secundarias tienden a disminuir el éxito de curación. En casos en que se irradia al paciente y en tanto los resultados son a largo plazo, se controlan los síntomas con enzimas adrenales inhibitorias como el ketoconazol , la metirapona, el mitotane o la aminoglutatimida , proporcionadas ya sea solas o combinadas (Orth, 1995; Buchfelder y Schlaffer, 2010; Lindsay, Nansel, Baid, Gumowski y Nieman, 2006; Losa, Picozzi, Redaelli, Laurenzi y Mortini, 2010). "
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    ABSTRACT: El objetivo de este estudio exploratorio es analizar la relación entre el bienestar psicológico y las condiciones médicas y terapéuticas de personas con diagnóstico de síndrome de Cushing. Los instrumentos utilizados fueron el Índice de Bienestar subjetivo (The Psychological Well-Being Index; Dupuy, 1984) y el Cushing’s Syndrome Severity Index (CSI). La selección de la muestra se realizó por conveniencia; considerando que es un síndrome de muy baja incidencia, se estableció contacto con cinco pacientes. Se utilizaron estadísticos descriptivos que facilitaron la obtención de información sobre la muestra. Los resulatdos per- miten concluir que la severidad de los síntomas asociados al síndorme, evaluados a través del CSI, implican malestar moderado en el bienestar psicológico, corroborando lo señalado por la literatura, así como que las alteraciones de orden psicológico influyen en el proceso de enfermedad o recuperación.Palabras clave: síndrome de Cushing (SC 5117073), bienestar psicológico (SC56603), psicología de la salud (SC22398).
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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.
    Preview · Article · Apr 2013 · Pituitary
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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.
    Preview · Article · Apr 2013 · The patient
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