Understanding Disease
Cushing’s Syndrome is a rare condition that happens when your body produces too much cortisol, a hormone linked to stress, metabolism, and immune function. This is often due to a tumour in the pituitary gland, which signals the adrenal glands to release excess cortisol.
Cushing’s syndrome is an uncommon condition.
Facts & figures:
Affects 350 - 1200 people every year worldwide2
40 in 1,000,000 people have Cushing’s syndrome worldwide3
Most often affects adults who are usually between 30 and 50 years old1
More common in women than men
3 out of 4 patients who are affected by Cushing’s syndrome are women1
Most cases are caused by a non-cancerous tumour in the pituitary gland.
Other less common causes include:
Tumours in other parts of the body (ectopic ACTH syndrome)
Tumours directly affecting the adrenal glands
CD Causes Elevated Cortisol
CD is a rare hormonal disorder caused by a pituitary adenoma that secretes excess adrenocorticotropic hormone (ACTH).5 Excess ACTH stimulates the adrenal glands to overproduce cortisol, leading to the clinical manifestations of CD.
Patients with CD experience comorbidities at a higher rate than the general population.5 Although biochemical remission or a surgical cure is usually associated with clinical improvement, some comorbidities may not completely normalize.1,6
Hypertension and diabetes are the main long-term controllable risk factors for cardiovascular events and mortality; repeated long-term follow-up is recommended.7,8
What does cortisol do?
Control blood pressure
Manage blood sugar
Help the body respond to stress
Cushing’s symptoms may be subtle or mistaken for other conditions. Key signs include:
Red face
Easy bruising
Stretch marks
Proximal muscle weakness
Rounding of the face
Excessive body and facial hair
Acne
Weight gain
Fat pads
Symptoms can vary greatly by patient, further complicating the path to diagnosis1
Symptoms of hypercortisolism can mimic other common conditions.1 While thin skin, easy bruising, and muscle weakness are key indicators, not all patients exhibit these features. Confirming Cushing’s disease (CD) requires additional screening, including laboratory tests, imaging, and potentially, procedures.2
PATIENTS WITH CHRONIC EXPOSURE TO EXCESS CORTISOL are at increased risk for morbidity and mortality12
The signs and symptoms of CD can be confusing8 :
Signs and symptoms vary from patient to patient12
Not all signs and symptoms are obvious and can depend on extent and persistence of disease8
In some cases, hypercortisolism may not present classically; patients may present with only isolated symptoms4
*Some patients may have hypercortisolism despite not having these typical phenotypes.4
Cushing’s symptoms can look like everyday problems — making diagnosis tricky.
Left untreated, it may lead to:
Venous
Thromboembolism
≈6.8x
higher risk
Heart
Failure
≈6.0x
higher risk
Stroke
≈4.5x
higher risk
Acute Myocardial
Infarction
≈2.1x
higher risk
Predicted Mortality Rate
The predicted mortality rate is nearly 2x higher for patients with uncured CD vs that for patients in remission13. When patients are not appropriately treated, their mortality rate is ~4-fold higher than the general population6.
The goal is to restore normal hormone levels and improve quality of life.
Options may include:
Surgery to remove the tumour (1st line treatment)
Medication to control cortisol levels (2nd line treatmet)
Radiation therapy (if needed)
LEARN MORE
Regulating our body’s stress response
During times of stress, our body can release cortisol after releasing its “fight or flight” hormones, like adrenaline. This happens so you continue to stay on high alert. Cortisol also triggers the release of glucose (sugar) from your liver. This provides fast energy during times of stress.
Regulating metabolism and blood sugar levels
Metabolism refers to the chemical processes in our body that allow us to live and function. Thousands of metabolic processes happen at all times. Cortisol affects our metabolism by helping regulate how our body uses glucose (sugar) for energy. The hormone does this in many ways. For example, cortisol triggers our pancreas to decrease insulin and increase glucagon. Insulin lowers blood glucose (sugar). Glucagon raises it. Cortisol also acts on other bodily tissues to manage glucose use, including your body fat tissue (adipose tissue), liver, and muscle.
Suppressing inflammation
In short spurts, cortisol can boost our immunity by limiting inflammation. But if you have consistently high levels of cortisol, your body can get used to it. This can lead to inflammation and a weakened immune system.
Regulating blood pressure
The exact way in which cortisol regulates blood pressure in humans is unclear. But elevated levels of cortisol can cause high blood pressure. And lower-than-normal levels of cortisol can cause low blood pressure.
Helping controls your sleep-wake cycle
Most people have lower cortisol levels in the evening when they go to sleep. And they have peak levels in the morning right before they wake up. This suggests that cortisol plays a key role in your circadian rhythm and how your body wakes up.
The most common causes of Cushing’s syndrome are those that involve excessive secretion of the hormone ACTH (also called ACTH-dependent causes). These actually account for 8 out of 10 cases of Cushing’s syndrome and occur when a growth (tumour) produces too much of the hormone ACTH. This in turn causes the adrenal glands to produce too much cortisol.
Other organs where tumours can be found in ectopic ACTH syndrome:
Thyroid (A small butterfly-shaped gland found on the neck)
Lungs (A pair of organs located on either side of the chest that allows you to breathe)
Pancreas (A long, flat gland behind the stomach that plays a role in digestion)
Causes of Cushing’s syndrome that do not involve the hormone ACTH are less common and account for about 2 out of 10 cases of the condition. The examples here are found at the top of the kidneys:
A non-cancerous (benign) tumour found on one adrenal gland
Benign tumours found on both adrenal glands
A cancerous (malaignant) tumour found only on one gland
Malignant tumours found on both adrenal glands
Lumps (nodules) that measure more than 1cm found in one or both adrenal glands that causes to increase in size
Lumps (nodules) that measure less than 1cm found in one or both adrenal glands; adrenal glands are usually normal in size
WHAT YOU CAN DO
Know what to do before your visit
When you make the appointment, ask if there's anything you need to do to prepare for testing.
Write down any symptoms you're having
Include any that seem unrelated to the appointment. For example, if you've had headaches more often or if you've been feeling down or more tired than usual, tell your health care provider. Also talk to your provider about changes in your appearance, such as weight gain, new acne or more body hair.
Write down key personal information
Let your provider know if the people closest to you have noticed that you seem irritable or have more mood swings than in the past. It may help to bring a photo of yourself that shows any changes in your appearance since you've started having symptoms.
Make a list of all medicines, vitamins, creams or supplements
Write down the name, dose and dates of any steroid medicines you've taken before, such as cortisone shots.
Take a family member or friend along, if possible
Sometimes it can be hard to remember all the information you get during an appointment. Someone who comes with you may remember something that you missed or forgot.
Write down questions
To ask your health care provider
References
Sharma ST, Nieman LK, Feelders RA. Cushing’s syndrome: epidemiology and developments in disease management. Clin Epidemiol. 2015;17(7):281-293. doi:10.2147/CLEP.S44336 2. Lonser RR, Nieman L, Oldfield EH. Cushing’s disease: pathobiology, diagnosis, and management. J Neurosurg. 2017;126(2):404-417. doi:10.3171/2016.1.JNS152119 3. Giuffrida G, Crisafulli S, Ferraù F, et al. Global Cushing's disease epidemiology: a systematic review and meta-analysis of observational studies. Endocrinol Invest. 2022;45(6):1235-1246. doi:10.1007/s40618-022-01754-1 4. Nishioka H, Yamada S. Cushing's disease. Clin Med. 2019;8(11):1951. doi:10.3390/jcm8111951 5. Feelders RA, Pulgar SJ. Kempel A, Pereira AM. The burden of Cushing's disease: clinical and health-related quality of life aspects. Eur J Endocrinol. 2012;167(3):311-326. doi:10.1530/EJE-11-1095 6. Pivonello R, De Leo M, Cozzolino A, Colao A. The Treatment of Cushing's Disease. Endocr Rev. 2015 Aug;36(4):385-486. doi:10.1210/er.2013-1048 7. Nieman LK, Biller BMK, Findling JW. et al. Treatment of Cushing's syndrome: an Endocrine Society clinical practice guideline. Endocrine. 2015;100(8):2807-2831. doi:10.1210/jc.2015-1818 8. Fleseriu M, Auchus R, Bancos I, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021;9(12):847-875. doi:10.1016/S2213-8587(21)00235-7 9. Dekkers OM, Biermasz NR, Pereira AM, et al. Mortality in patients treated for Cushing's disease is increased, compared with patients treated for nonfunctioning pituitary macroadenoma. J Clin Endocrinol Metab. 2007;92(3):976-981. doi:10.1210/jc.2006-2112 10. Lacroix A, Feelders RA, Stratakis CA, Nieman LK. Cushing's syndrome. Lancet. 2015;386(9996):913-927. doi:10.1016/S0140-6736(14)61375-1 11. Fleseriu M, Castinetti F. Updates on the role of adrenal steroidogenesis inhibitors in Cushing’s syndrome: a focus on novel therapies. Pituitary. 2016;19(6):643-653. doi:10.1007/s11102-016-0742-1 12. Dekkers OM, Horváth-Puhó E, Jørgensen JOL, et al. Multisystem morbidity and mortality in Cushing’s syndrome: a cohort study. J Clin Endocrinol Metab. 2013;98(6):2277-2284. doi:10.1210/jc.2012-3582 13. van Haalen FM, Broersen LHA, Jørgensen JO, Pereira AM, Dekkers OM. Management of endocrine disease: Mortality remains increased in Cushing’s disease despite biochemical remission: a systematic review and meta-analysis. Eur J Endocrinol. 2015;172(4):R143-R149. doi:10.1530/EJE-14-0556
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