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Cushing's disease (also known as Cushing disease, tertiary or secondary hypercortisolism, tertiary or secondary hypercorticism, Itsenko-Cushing disease) is a cause of Cushing's syndrome characterised by increased secretion of adrenocorticotropic hormone (ACTH) from the anterior pituitary (secondary hypercortisolism). This is most often as a result of a pituitary adenoma or due to excess production of hypothalamus CRH (Corticotropin releasing hormone) (tertiary hypercortisolism/hypercorticism). Pituitary adenomas are responsible for 70% of endogenous Cushing's syndrome, when excluding Cushing's syndrome from exogenously administered corticosteroids.
Signs and Symptoms
The symptoms related to Cushing's disease and Cushing's syndrome are the same, since both are related to an excess of cortisol. Patients with Cushing's disease usually present with one or more signs and symptoms secondary to the presence of excess cortisol or ACTH. Although uncommon, some patients with Cushing's disease have large pituitary tumors (macroadenomas). In addition to the severe hormonal effects related to increase blood cortisol levels, the large tumor can compress adjacent structures. The eye conditions glaucoma and cataracts also may occur in Cushing's syndrome. In Cushing's disease (tumors on the pituitary gland), your field of vision can be affected. You may have loss of side, or peripheral, vision.
The clinical diagnosis must be based on the presence of one or more of the symptoms listed below, because the syndrome itself has no true pathognomonic signs or symptoms.
Less Common Symptoms
The less-common symptoms include:
Following the 8 mg dexamethasone suppression test plasma cortisol should be low; while plasma ACTH should be at the higher end of normal or should exceed the reference range. This will distinguish whether the cause is an ectopic ACTH secreting tumour, an adrenal carcinoma or a pituitary adenoma - ranges differ between different labs. A more accurate but invasive test used to differentiate pituitary from ectopic or adrenal cushing's syndrome is inferior petrosal sinus sampling.
A CT or MRI of the pituitary may also show the ACTH secreting tumor if present.
Administration of corticotropin releasing hormone (CRH) can differentiate this condition from ectopic ACTH secretion. If ectopic, the plasma ACTH and cortisol levels should remain unchanged; if this is pituitary related, levels of both would rise.
Treatment of a pituitary adenoma usually involves removal of the tumor via transphenoidal surgery (TSS). Access to the sphenoidal sinus can either be transnasal (through the nostril) or sublabial (through an incision under the top lip). A study of 3,525 cases of TSS for Cushing's disease in the U.S. between 1993 and 2002 found the following results: the in-hospital mortality rate was 0.7%; the complication rate was 42.1%. Diabetes insipidus (15%), fluid and electrolyte abnormalities (12.5%), and neurological deficits (5.6%) were the most common complications reported. The study also found that complications were more likely in patients with pre-operative comorbidities. Patients older than 64 years were much more likely to have an adverse outcome and prolonged hospital stay.
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