In Cushing’s disease (thesaurus synonyms: ACTH [adrenocorticotropic hormone]-pituitary hypersecretion; ACTH [adrenocorticotropic hormone]-pituitary hypersecretion; obesity osteoporotica endocrinica; alcohol-induced pseudo-Cushing’s syndrome; Apert-Cushing’s syndrome; artificial Cushing’s syndrome; drug-induced Cushing’s syndrome; Basophilic hyperpituitarism; Basophilism; Cortico-adrenal basophilism; Crooke-Apert-Gallais syndrome; Cushing’s basophilism; Cushing’s disease; Cushing’s syndrome; Cushing’s syndrome due to ectopic ACTH [adrenocorticotropic hormone]-producing tumor; Dyscorticism; Ectopic ACTH [adrenocorticotropic hormone] syndrome; Extrapituitary ACTH [adrenocorticotropic hormone] syndrome; Hyperadrenocorticism; Hypercorticism; Hypersuprarenalism; Pituitary basophilism; Pituitary hyperadrenocorticism; Pituitary hypercorticism; Pituitary Cushing’s syndrome; Iatrogenic Cushing’s syndrome; Idiopathic Cushing’s syndrome; Cerebellopontine angle syndrome; Corticoadrenal hypersecretion; Cortisol hypersecretion; Cushing’s disease; Icenko-Cushing’s disease; Myopathy in Cushing’s syndrome; Myopathy in hyperadrenocorticism; Nelson’s syndrome; Nelson’s tumor; Thymic basophilism; ICD-10-GM E24. -: Cushing’s syndrome) is a group of disorders that lead to hypercortisolism (hypercortisolism; excess of cortisol).
The following forms can be distinguished:
- Endogenous Cushing’s syndrome (10% of cases) – this can again be subdivided into:
- ACTH-dependent (approximately 85% of endogenous cases).
- Central Cushing’s syndrome (Cushing’s disease; central hypercortisolism) – usually microadenoma of the anterior pituitary [about 65-70% of cases].
- Ectopic ACTH secretion* (adrenocorticotropic hormone) – paraneoplastic; secretion of ACTH in neoplasms, especially in bronchial carcinoma (lung cancer) [about 15-20% of cases].
- Ectopic CRH secretion* (corticotropin-releasing hormone).
- Alcohol-induced
- ACTH-independent [approximately 15% of endogenous cases].
- Adrenal Cushing’s syndrome – mainly caused by tumors of the adrenal cortex (mostly adenomas; rarely carcinomas) [about 15% of all endogenous Cushing’s syndromes]
- Primary bilateral NNR hyperplasia (adrenocortical hyperplasia/cell enlargement):
- Micronodular hyperplasia (PPNAD, primary pigmented nodular adrenocortical diesease); small, pigmented nodules of the NNR.
- Macronodular dys/hyperplasia (AIMAH, ACTH-independent macronodular adrenal hyperplasia); larger, non-pigmented nodules of the NNR.
- ACTH-dependent (approximately 85% of endogenous cases).
- Exogenous Cushing’s syndrome (iatrogenic Cushing’s syndrome) – this form occurs frequently (90% of cases) and is triggered by long-term therapy with glucocorticoids (usually prednisolone) or precursors of the latter.
* Approximately 15-20% of cases
Sex ratio of Cushing’s disease: males to females is 1: 3-4.
Frequency peak of Cushing’s disease: the disease occurs predominantly between the 20th and 40 years of age.
The incidence (frequency of new cases) of Cushing’s disease is approximately 1-2.4 cases per 100,000 inhabitants per year (in Germany).
Course and prognosis: Cushing’s syndrome can develop insidiously over years. A treated Cushing’s syndrome has a favorable prognosis in most cases. The success rate of treatment is between 50 and 80%. If Cushing’s syndrome is caused by medications, the prognosis is very good because it is possible to switch to other medications or reduce the dose. In endogenous Cushing’s syndrome, the course depends on the type and severity of the underlying disease. If left untreated, the disease leads to death after months to years. In the majority of cases, affected individuals die of myocardial infarction (heart attack) or apoplexy (stroke).
