Endocrine Abstracts

Background: True Cushing’s syndrome (CS) is an exceedingly rare condition, thus to avoid unnecessary and costly testing, a diagnostic investigation should only start based on solid clinical suspicion. The current obesity epidemic has become a hurdle in discriminating subjects at real risk for CS from those with the metabolic syndrome. Moreover, many CS patientsrecently diagnosed do not fit the typical clinical profile of ‘at risk subjects’.

Aim and Design: To characterize the clinical presentation of patients with confirmed CSat Tel Aviv Sourasky Medical Center between 2000–2018. Relevantclinical, laboratory, imaging and pathological datawere retrospectively retrieved and analyzed fromCS patients’ charts.

Results: We identified 76 patients with CS (79%women) with a clear increased incidence over the years. The subtypes comprised: 49 Cushing’s disease (CD) 64.5%, 16 benign adrenal process (BA) 21.1%, 7 ACC 9.2%, and 4 cases of ectopic ACTH secretion (ECT) 5.3%. Mean age was 47.9 ± 15.3 y (range: 18–79), by ANOVA (P = 0.003), CD subjects being significantly younger than subjects with ECT. Clinical suspicion of CS initiated a diagnostic workup in only 15 subjects (19.7%). In 48 (63%), an investigation started either for incidental imaging or laboratory findings, or for nonspecificreasons(i.e obesity). Once the diagnosis was known, in 50% of the cases an endocrinologist noted the patient did not appear to suffer from CS. The most common co-morbidities were hypertension (62.7%), hyperlipidemia (48.6%), obesity (48.6%), osteoporosis, and diabetes (36.8%).The most common symptoms were muscle weakness in both genders (29.6%), and menstrual disturbances in women of reproductive age (46.8%). Acute weight gain was reported by only 17.1% of subjects. Finally, the most common signs were abdominal obesity (70%), buffalo hump (38%), and a round/moon face (37.5%). Typical striae were noted in only 20.8% of subjects. All these features were less frequent than usually quoted in the literature. We generated a disease severity score which was positively correlated with serum cortisol after an overnignt dexamethasone suppression test (r = 0.42, P = 0.003), but not with urinary free cortisol excretion.

Conclusions: Our tertiary institution’s experience suggests the incidence of diagnosed CS is rising. Nonetheless, clinicians should be aware that the majority of modern era CS patients might escape diagnosis due their non-classical presentation. Our findings add further uncertainty as to which patients should be screened for CS.