Cushing's syndrome (overview) E24.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Apert-Cushing's Syndrome; Apert-Gallais Syndrome; basophile; basophilic adenoma; Crooke-Apert-Gallais Syndrome; Cushing purpura; Cushing's basophilism; Cushing\'s basophilism; Cushing's disease; hyperadrenocorticism; Hypercorticism; Hypercortisolism; Hyperpituitarism; Hyperpituitarism basophile; Incenko-Cushing's disease; Purpura Cushing purpura; Purpura with M. Cushing

History
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Apert, 1910; Gallais, 1912; Cushing, 1932

Definition
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Hypercortisolism with characteristic clinical symptoms due to exogenous or endogenous excess supply of glucocorticoids and possibly androgens

Classification
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  • Exogenous Cushing's syndrome (frequent)
    • Iatrogenic Cushing's syndrome (through long-term regular, systemic administration of ACTH or glucocorticoids).
  • Endogenous Cushing's syndrome (rarely 2-3 cases/1 million inhabitants)
    • Central Cushing's Syndrome (Cushing's Disease: 72% of all cases): Increased production of ACTH in the anterior pituitary gland followed by increased corticoid release from the adrenal cortex (see also pituitary diseases, skin changes).
    • Adrenal Cushing's Syndrome (about 20% of all cases, 80% of women are affected): increased secretion of gluco- or mineralocorticoids from the adrenal cortex in the course of neoplasia (adrenal cortex adenomas: 50% of cases or adrenal cortex carcinomas: 50% of cases) with subsequent suppressed release of ACTH from the anterior pituitary gland); so-called "white Cushing's" s.a. " white Addison's". In very rare cases of adrenal adenomatosis (<1% of all cases) multiple small andenomas are also detectable.
    • Ectopic (paraneoplastic) Cushing's syndrome (formation of corticoids in ectopic tissue, e.g. in small cell bronchial carcinoma).
    • Hypothalamic-pituitary Cushing's syndrome (disturbances of the hypothalamic-pituitary regulation).
    • Pseudo-Cushing's syndrome, so-called Cushingoid (passagere Cushing's symptoms e.g. after severe cranial injuries).
    • Iatrogenic Cushing's syndrome: As a result of long-term therapy with ACTH or glucocorticosteroids.

Clinical features
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Clinical-dermatological impress, weight gain with centripedal obesity with the typical full moon face and buffalo neck, striae cutis distensae (abdomen, thighs, axillary folds), fatigue, muscle weakness, plethora, arterial hypertension, general inefficiency, impotence, oligo-bis amenorrhoea, depressive moods, sleep disorders, anxiety and other personality disorders as well as steroid acne. Hypertrichosis to hirsutism exists in 20% of patients (mainly women). Children suffer from growth disorders due to the oversupply of corticosteroids.

Furthermore osteoporosis with back pain, as well as loss of potency and libido.

Occasionally, hyperpigmentation (with ACTH and MSH overproduction) and purpura are present. The adrenal Cushing's syndrome shows no hyperpigmentation ("white Cushing's").

Diagnosis
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Possible by modern imaging techniques such as MRT, CT, sinus-petrosus-inferior catheter under CRH-stimulation, CRH-stimulation test and dexamethasone suppression test

Therapy
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In case of metabolic disorders and tumours: treatment of the underlying disease by internists or surgeons. With glucocorticoid medication: Reduction of the dose to below the Cushing's threshold (5-7.5 mg/day prednisolone equivalent) or, if necessary, change the medication to other immunosuppressive drugs.

Literature
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  1. Apert E (1910) Dystrophies en relation avec des lésions des capsules surrénales. Hirsutisme et progenia. Bulletin of the Société de pédiatrie de Paris 12: 501-518
  2. Cushing HW (1932) The basophil adenomas of the pituitary body and their clinical manifestations (pituitary basophilism). Bull Johns Hopkins Hosp 50: 137-195
  3. Gallais A (1912) Le syndrome génitosurrénal, étude anatomo-clinique. Thesis, Paris, p. 224
  4. Kolyvanos Naumann U et al (2003) Cushing syndrome/Cushing disease. Switzerland Rundsch Med Prax 92: 1763-1777
  5. Lacroix A, Bolte E, Tremblay J et al (1992) Gastric inhibitory polypeptide-dependent cortisol hypersecretion - A new cause of Cushing's syndrome. N Engl J Med 327: 974-980
  6. Magialou MA et al (1994) Cushing's syndrome in children and adolescents. Presentation, Diagnosis and Therapy. N Engl J Med 331: 629-636
  7. Mönig H, Schulte HM (1992) Ectopic ACTH syndrome. Dtsch med Wschr 117: 1605-1610
  8. Nieman LK (2002) Medical therapy of Cushing's disease. Pituitary 5: 77-82
  9. Oldfield EH, Doppman JL, Nieman L et al (1991) Petrosal sinus sampling with and without corticotropin-releasing hormones for the differential diagnosis of Cushing's syndrome. N Engl J Med 325: 897-905
  10. Orth DN (1992) Corticotropin-releasing hormones in humans. Endocr Rev 13: 164-191
  11. Pereira AM et al (2003) Long-term predictive value of postsurgical cortisol concentrations for cure and
  12. risk of recurrence in Cushing's disease. J Clin Endocrinol Metab 88: 5858-5864
  13. Reznik Y, Allali-Zerah V, Chayvialle JA et al. (1992) Food-dependent Cushing's syndrome mediated by aberrant adrenal sensitivity to gastric inhibitory polypeptides. N Engl J Med 327: 981-986
  14. Saeger W (2003) Adrenocortical tumours. Pathologist 24: 272-279

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020