Cycle characterisation and clinical complications in patients with cyclic Cushing's syndrome: insights from an international retrospective cohort study
Authors' conclusion
Even in specialised centres, cCS diagnosis and management remain challenging with high rates of spontaneous adrenal insufficiency, inappropriate surgeries, and poor outcomes. Ectopic cCS showed the most frequent and severe peaks. These findings might help to guide imaging localisations or the timing of BIPSS in patients with active occult ACTH-dependent cCS. Hypercortisolism needs to be biochemically confirmed before BIPSS to enable correct tumour localisation. Patients with suspected or proven cCS should be equipped with salivary cortisol collection kits to capture dynamic changes as well as being prescribed glucocorticoids to be used as a precaution.
Study context
- Population
- Patients with confirmed CS showing ≥2 hypercortisolaemic peaks and ≥1 spontaneous eucortisolaemic or hypocortisolaemic trough; 43 endocrine centres in 21 countries116 potentially eligible
- Participants
- 110 patients
- Outcome
- Cycle characteristics and complications of cCS
Methodological detailsobservational, international, multicentre, Data collected between Dec 1, 2023 and Feb 2, 2025
- Observational
- International
- Multicentre
- Data collected between Dec 1, 2023 and Feb 2, 2025
Main finding
As reported in the abstract
- Female
- 84 [76%] of 110
- Median age at diagnosis
- 44.0 years (IQR 31.8-58.3)
- Origin pituitary / ectopic / adrenal / occult
- 70 (64%) / 25 (23%) / three (3%) / 12 (11%)
- Median 24 h UFC peaks
- 7.40 × ULN (range 0.44-299)
- Median 24 h UFC troughs
- 0.31 × ULN (0.02-0.98)
- Median peak count
- 3.0 (IQR 2.0-4.0)
- Irregular intervals
- 55 [86%] of 64
- Symptoms worsened during peaks
- 87 (81%) of 108
- Symptoms improved during troughs
- 79 (74%) of 107
- Bilateral inferior petrosal sinus sampling (BIPSS) performed during troughs
- 14 patients (18% of the 78 procedures done)
- Imaging missed tumours
- 35 (32%) of the 110
- Delayed diagnosis
- 45 [41%] of 110
- Delayed therapy
- 47 [43%]
Observational data: associations, not effects.
Further findings
Outcomes at median follow-up 5.8 years (IQR 2.6-10.5)
- Complete biochemical surgical remission
- 55 (50%) of 110
- Spontaneous remission
- seven (6%)
- Medically controlled
- 22 (20%)
- Partial remission
- six (5%)
- Uncontrolled
- 11 (10%)
- Lost to follow-up
- nine (8%)
Safety
Complications
- Spontaneous AI
- 31 (28%) of 110
- Unwarranted surgery at wrong anatomical site due to misclassification
- nine (8%)
- Died during entire observation period
- 3% (3/110)
Original publication
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