Cushing’s disease is a condition that occurs when the pituitary gland— the endocrine system’s organ— releases an excess amount of adrenocorticotropic hormone (ACTH), said Medline Plus, a trusted health information website. Cushing’s disease is a form of Cushing syndrome.
Other forms of the said condition also include exogenous Cushing syndrome, Cushing syndrome caused by adrenal tumor, and ectopic Cushing syndrome. ACTH triggers the adrenal glands— which are located on top of the kidneys— to produce cortisol, also known as the stress hormone, stated UCLA Health.
Clinical Practice Patterns In Diagnosing and Managing Cushing’s Disease In Iran (2016)
The questionnaire created by Mojtaba Malek and colleagues of biomedical and life sciences journal PMC was appraised by the members of a scientific committee comprising of nine endocrinologists. The endocrinologists are experts in the diagnosis and management of Cushing’s disease. 90 endocrinologists were invited to take part in the survey, with 76 (84.4%) participating and 53 (69.7%) answering all questions.
75% and 86.8% of respondents obtained an assessment of 24-hour urinary free cortisol (UFC) and fasting serum cortisol after an overnight dexamethasone suppression test (ONDST), respectively. 59% chose both of the above-mentioned screening tests simultaneously, whereas only 9.2% obtained UFC as a single screening test. 19.7% chose the measurement of midnight serum cortisol while midnight salivary cortisol in combination with the other tests was acquired by 7.9% of participants.
Taking into account the assessment of current clinical practice for localizing the primary lesion, classic high dose dexamethasone suppression test may be obtained alone by 22.4% of participants. 64.5% said that the use of both serum ACTH and classic high dose dexamethasone suppression test whereas 15.8% chose modified high dose dexamethasone suppression test for localizing the primary lesion.
When asked to provide their diagnostic approach if the preliminary evaluations indicated an ectopic Cushing’s disease, 93.4% chose a high resolution CT scan of the thorax, 2.9% preferred to use a spiral CT scan of the abdomen, and 76.3% said they would use both tests to assess an ectopic source. 69.7% chose to perform an anoctreotide scan, whereas 11.8% chose to use inferior petrosal sinus sampling.
The respondents were instructed to specify their primary choice of treating an index case of ACTH-dependent Cushing’s disease with a 6 mm pituitary adenoma reported in dynamic brain MR. The respondents said they would treat it with Trans-sphenoidal pituitary surgery (TSS) (5.3%), long acting somatostatinanalogues, metyrapone, mitotane (6.6%), or bilateral adrenalectomyetoconazole (1.3%)
The index case was operated via a TSS (Trans-sphenoidal pituitary surgery) approach. No evidence of hypo or hypercortisolism was found for the next two years after the two years following the operations when recurrence proved to be ACTH-dependent. When the respondents were asked to specify their next options in treating the index case, 51.3% selected medical treatment as the preferred treatment modality for the recurrence of Cushing's disease in this clinical setting. Meanwhile, 31.6% chose bilateral adrenalectomy and 18.4% preferred pituitary re-surgery.
The index case was treated by ketoconazole. Three months later, liver enzymes increase to five times the upper limit of the normal range. The participants were then asked to select their course of action. Their preferred treatment options for addressing Cushing’s disease post-pituitary surgery and ketoconazole failure were bilateral adrenalectomy (51.3%) and pasireotide (36.8%). 9.2% chose combination therapy with cabergoline and pasireotide, while only 5.3% preferred to perform another pituitary surgery.
The participants were asked to rate the success in the treatment of ACTH-dependent Cushing’s disease in Iran. 48.7% said it had a success rate of 30% to 49% while 38.2% rated it as 50 to 79%. When asked to provide a priority list of dogs they would like to be made available for treating Cushing’s disease in Iran, they answered Ketoconazole (67.9%) and Pasireotide (38.5%).
What Are the Symptoms of Cushing’s Disease?
Signs can include upper body obesity (above the waist) and having thin arms and legs. Having a round, red, full face is also another sign of this condition. Kids may have Cushing’s disease if they experience a slow growth rate. Skin changes such as acne or skin infections and purple stretch marks or striae on the skin of the abdomen, upper arms, breasts, and thighs are also a result of Cushing’s disease.
Women who have this condition may have excess hair growth on the face, chest, abdomen, neck, and thighs. Their menstrual cycle can stop or become irregular. For men, they can suffer from decreased libido or no desire for sexual activity, including erection problems. Those with Cushing’s disease may experience depression, anxiety, or changes in behavior. Increased thirst and urination and high blood pressure are telltale signs of Cushing’s disease.
How Is Cushing’s Disease Diagnosed?
Your doctor will perform a physical examination and ask about your symptoms. The tests are performed to see if there is excess cortisol in your body, as well as to determine its cause. Tests such as the 24-hour urine cortisol are used to confirm excess levels of cortisol. To determine the cause, your doctor can perform a brain MRI scan, a dexamethasone suppression test, an ACTH blood test, and more.
How Is Cushing’s Disease Treated?
If possible, surgery can be performed to remove the pituitary tumor. The pituitary gland may slowly start to function normally after the procedure. When recovering from the surgery, you may need to cortisol replacement treatments since the pituitary gland needs time to start producing ACTH. If the tumor is not completely removed, radiation treatment of the pituitary gland can be done.
Undergoing radiation treatment can lead to delayed pituitary failure, which usually happens several years after the treatment. Therefore, it is recommended to have a continued long-term follow-up with your endocrinologist.
Your doctor will prescribe medications to stop your body from producing cortisol if the tumor fails to respond to surgery or radiation. If the aforementioned treatments are ineffective, your adrenal glands may have to be removed to prevent the excess production of cortisol. However, removing the adrenal glands can make the pituitary tumor bigger (Nelson syndrome). If Cushing’s disease is not treated, it can cause severe health complications and death. Removing the tumor may lead to full recovery, but it can also grow back.
Cushing’s disease occur when ACTH is produced excessively. Symptoms range from changes in skin and mental health to high blood pressure. If you think you exhibit signs of this condition, it is recommended to call your doctor immediately.