Idiopathic isolated adrenocorticotropic hormone deficiency: a systematic review of a heterogeneous and underreported disease
Authors' conclusion
We report the largest case series of acquired idiopathic IAD to date. Our systematic review highlights the lack of a clear definition and diagnostic work-up.
Study context
- Population
- Adults with acquired idiopathic IAD: ACTH deficiency on dynamic testing, no other pituitary axis deficiency, normal brain/pituitary MRI42 articles
- Participants
- 85 adults
- Outcome
- Clinical characteristics of acquired idiopathic IAD
Pooled result
As reported in the abstract
- Sex distribution (F:M)
- 47:38
- Lethargy as presenting symptom
- 38%
- Weight loss as presenting symptom
- 25%
- Anorexia as presenting symptom
- 22%
- Myalgia/arthralgia as presenting symptom
- 12%
- Presented with Addison crisis
- 8 cases (9.5%)
- Autoimmune disease at diagnosis (Hashimoto hypothyroidism most frequent)
- 31%
Further pooled results
Cases
- Dynamic testing repeated
- 4
- Recovery of the adrenal axis
- 2
- Follow-up data scarce
Original publication
Idiopathic isolated adrenocorticotropic hormone deficiency: a systematic review of a heterogeneous and underreported disease. Pituitary. Published 27 December 2023. doi:10.
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