CUSHING’S SYNDROME IN WESTERN SWEDEN

Abstract

Abstract Cushing’s syndrome in western Sweden Degree project, Programme in Medicine Sofie Wengander Supervisor: Oskar Ragnarsson, MD, Associate professor 2018, Department of Endocrinology, Sahlgrenska University Hospital, Gothenburg Introduction: Cushing’s syndrome (CS) is a rare endocrine syndrome caused by elevated cortisol levels. Endogenous CS is most often caused by adrenocorticotrophic hormone (ACTH) secreting pituitary tumours i.e. Cushing’s disease (CD), ACTH-secreting neuroendocrine tumours of other origin i.e. ectopic CS, cortisol producing adrenal adenomas, and in rarer cases adrenal carcinoma. Due to its rarity, epidemiological studies on CS are few. Objectives: The primary aim of this study was to analyze the incidence and prevalence of endogenous CS in western Sweden. Secondary aims were to validate the diagnostic codes used for CS and to evaluate the time span from debut of symptoms to remission. Methods: Medical records from patients diagnosed with CS in the West Götaland region 2002-2017 were reviewed. In total, 234 patients had received a diagnostic code for CS. Of these, 80 patients had endogenous CS and were included in the study. Results: The incidence of endogenous CS was 3.1 per million per year, 1.5 for CD, 0.7 for benign adrenal CS, 0.1 for adrenal carcinoma, and 0.8 for ectopic CS. Validation of the diagnostic codes showed a sensitivity of 100% and a specificity of 70% for CD, and a sensitivity of 62% and a specificity of 93% for ectopic CS. Patients with adrenal CS were inconsistently coded with diagnostic codes of other specified CS and unspecified CS. For patients with CD, the time from debut of symptoms to remission was 30-46 months and 5 24 months in patients with cortisol producing adrenal adenoma. Conclusions: 1) Benign adrenal CS and ectopic CS are more common than previously reported. 2) Specific diagnostic codes are needed for adrenal CS. 3) Patients with CS may have active disease for up to 4 years before adequate treatment has been provided.