Cushing's Syndrome and Hypothalamic-Pituitary-Adrenal Axis Hyperactivity in Chronic Central Serous Chorioretinopathy.

Femke M Van Haalen,Alberto M Pereira,Olaf M Dekkers,Elon H C Van Dijk,Maurice B Bizino,Camiel J F Boon,Nienke R Biermasz,Greet Dijkman

doi:10.3389/fendo.2018.00039

Abstract

Central serous chorioretinopathy (CSC), a specific form of macular degeneration, has been reported as presenting manifestation of Cushing's syndrome. Furthermore, CSC has been associated with both exogenous hypercortisolism and endogenous Cushing's syndrome. It is important to know whether CSC patients should be screened for Cushing's syndrome. Although hypothalamic-pituitary-adrenal (HPA) axis hyperactivity in CSC has been suggested, no detailed evaluation of the HPA axis has been performed in a large cohort of CSC patients. This study aimed to investigate whether Cushing's syndrome prevalence is increased among chronic CSC (cCSC) patients and whether detailed endocrinological phenotyping indicates hyperactivity of the HPA axis. Cross-sectional study. 86 cCSC patients and 24 controls. Prevalence of Cushing's syndrome, HPA axis activity. None of the cCSC patients met the clinical or biochemical criteria of Cushing's syndrome. However, compared to controls, HPA axis activity was increased in cCSC patients, reflected by higher 24 h urinary free cortisol, and accompanying higher waist circumference and diastolic blood pressure, whereas circadian cortisol rhythm and feedback were not different. Chronic CSC patients did not report more stress or stress-related problems on questionnaires. No case of Cushing's syndrome was revealed in a large cohort of cCSC patients. Therefore, we advise against screening for Cushing's syndrome in CSC patients, unless additional clinical features are present. However, our results indicate that cCSC is associated with hyperactivity of the HPA axis, albeit not accompanied with perception of more psychosocial stress.

Highlights

Cushing’s syndrome is a rare disease characterized by excessive exposure to cortisol and is associated with both metabolic and behavioral abnormalities
Data are presented as mean (SD) or as numbers, unless specified otherwise. aNot statistically significant after correction for age. bConsisting of depression, anxiety or panic disorder, posttraumatic stress disorder, burn-out, alcohol abuse, and schizophrenia. cConsisting of myocardial infarction, endocarditis, and atrial fibrillation. dConsisting of impotence, hirsutism, menstrual cycle disorders, and loss of libido. cCSC, chronic central serous chorioretinopathy
Whereas we did not find any case of Cushing’s syndrome, the activity of the HPA axis appeared to be increased in cCSC patients, without disruption of circadian rhythm

Summary

Introduction

Cushing’s syndrome is a rare disease characterized by excessive exposure to cortisol and is associated with both metabolic and behavioral abnormalities. We recently reported patients who developed visual symptoms caused by chronic central serous chorioretinopathy (cCSC) as presenting manifestation of Cushing’s syndrome [2]. Central serous chorioretinopathy (CSC) is a relatively common eye disease often affecting the macula, in which choroidal congestion, thickening, and hyperpermeability lead to retinal pigment epithelial damage and cause serous subretinal fluid accumulation. No data are available on the prevalence of CSC in patients treated with corticosteroids, up to 52% of CSC patients in different cohorts reported to use steroids during the active phase of disease [6, 7]. Clinical characteristics, circadian tests, and cortisol feedback were not included in these studies, making it impossible to conclude on the prevalence of Cushing’s syndrome

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Conclusion