Adrenal disease

Diagnostic challenges in cyclic Cushing's syndrome: a systematic review

Lancet Diabetes EndocrinolPublished Systematic review of case reports and case series

Authors' conclusion

Variations in biochemical test results due to unpredictable cycle duration and frequency might cause diagnostic challenges resulting in misdiagnoses and missed diagnoses.

Study context

Population
Published single case reports and case series of cyclic Cushing's syndrome118 articles
Participants
212 participants
Comparison
Cyclic vs non-cyclic CS
  • Cyclic CSn = 212
Outcome
Aetiology of cyclic CS
Methodological detailsMEDLINE from inception to Oct 10, 2022
  • MEDLINE from inception to Oct 10, 2022

Pooled result

As reported in the abstract

Pituitary tumours
67% (n=143)
Ectopic tumours
17% (n=36)
Adrenal tumours
11% (n=23)
Occult tumours
2% (n=4)
Unclassified
3% (n=6)

Further pooled results

OutcomeP value
Remission rates and time to remissionP < 0.001
  • Remission rates and time to remission: remission rates lower and time to remission longer with cyclic CS

Clinical symptoms and comorbidities

No major difference between cyclic and non-cyclic CS

Bilateral inferior petrosal sinus sampling in adrenocorticotropic hormone (ACTH)-dependent cyclic CS

PPV and NPV when performed during hypercortisolism
100%
PPV irrespective of cortisolaemic status
73%
NPV irrespective of cortisolaemic status
86%

Safety

Unnecessary surgery due to misclassification — Cyclic CS
6%

Original publication

Diagnostic challenges in cyclic Cushing's syndrome: a systematic review. Lancet Diabetes Endocrinol. Published 7 July 2023. doi:10.1016/s2213-8587(23)00150-x

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