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EP197 - ECE_1768 - Effective cortisol synthesis control with markedly low-dose osilodrostat in ACTH-independent Cushing syndrome: a case report

G. Handzlik, Anna Chudek, Maria Świerkot, Ewa Płoskonka, J. Staniszewska-Mróz, J. Chudek

European Journal of Endocrinology, 2026

Abstract

Surgical adrenalectomy is the treatment of choice in ACTH-independent Cushing syndrome (CS). However, it may be contraindicated in patients with advanced age or severe comorbidities. Pharmacological treatment options include osilodrostat, an oral 11β-hydroxylase inhibitor approved for ACTH-dependent CS, which has been used sporadically in ACTH-independent CS. We present a case of ACTH-independent CS successfully controlled with extremely low doses of osilodrostat.

A 64-year-old female was admitted to the endocrinology unit for evaluation of resistant hypertension with persistent hypokalemia. The initial evaluation suggested primary aldosteronism, but subsequent hormonal work-up established ACTH-independent CS, with elevated 24-hour urinary free cortisol, loss of the diurnal cortisol rhythm, unresponsiveness to 1 mg overnight dexamethasone suppression test, and decreased plasma ACTH level. Abdominal CT imaging revealed a 17-mm adrenal tumour of the right gland with benign radiological features. The patient presented with multiple, potentially CS-related comorbidities, including obesity (BMI 35.7 kg/m²), type 2 diabetes mellitus, severe hypertension with kidney disease, osteoporosis (with low-trauma vertebral fractures), and severe obstructive sleep apnea. Her medical history was complicated by life-threatening infections, including severe COVID-19 pneumonia requiring ICU admission (2020) and septic shock in the course of necrotizing soft tissue infection of the lower…

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