Presentation
A widely used antiacne drug, retinoic acid, can change the clinical presentation of CS and delay the diagnosis. [journals.aace.com]
All patients in whom the ectopic ACTH-secreting tumour was obvious at presentation died of their primary tumour within 8 months, whereas seven of the 10 patients with occult tumours at presentation are alive 1.5-16.5 years later, and appear cured. [ncbi.nlm.nih.gov]
Conference: 9th Annual ENETS Conference (2012) Category: Clinical Presenting Author: Drs. Kimberly Kamp [enets.org]
Meningioma presenting with Cushing's syndrome: An unusual clinical presentation. Ann Neurol 2003;53:138-42. [ijem.in]
We present a case of ACTH-dependent CS where treatment and diagnosis was complicated by a patient deferring key testing. Mr. R is a 52 year-old male who presented with decreased libido, depression, and lower back pain. [endocrine.org]
Entire Body System
- Fatigue
Symptoms also include fatigue, weakness, depression, mood swings, increased thirst and urination, and lack of menstrual periods in women. [nadf.us]
[…] gain/central obesity (97%); dorsocervical fat pad (buffalo hump); supraclavicular fat pads; facial fullness (moon face) Reproductive system- menstrual irregularity; infertility Psychiatric effects- depression (62%), psychosis, irritability, insomnia, fatigue [dundeemedstudentnotes.wordpress.com]
Symptoms Weight Gain Fatty tissue deposits Excessive hair growth High blood pressure Fatigue Flushing Acne Stretch marks Loss of muscle Anxiety Easy bruising Headache Round face Diagnosis Clinical presentation Urine test Blood test Saliva test – tests [raredr.com]
[…] growth on the face, neck, chest, abdomen, and thighs Periods that become irregular or stop Men may have: Decreased or no desire for sex Impotence Other symptoms that may occur include: Mental changes, such as depression, anxiety, or changes in behavior Fatigue [medlineplus.gov]
- Virilization
Cushing syndrome patients can manifest with virilization due to the excess cortisol and adrenal androgens. [themedicalbiochemistrypage.org]
Women may experience symptoms of virilization secondary to elevated androgen levels. These signs include facial hair, acne and oligomenorrhea. Elevated cortisol causes vasoconstriction, hypertension, sodium retention and hypokalemia. [wikilectures.eu]
[…] metabolic Insulin resistance → hyperglycemia (see diabetes mellitus ) → mild polyuria in the case of severe hyperglycemia Dyslipidemia Weight gain characterized by central obesity, moon facies, and a buffalo hump ♂ : decreased libido ♀ : decreased libido, virilization [amboss.com]
A 55-yearold woman presented with a history of severe proximal muscle weakness, polyuria, progressive virilization, anxiety, dyspnea on exercise, difficult to treat hypertension, and type 2 diabetes mellitus since 4 months. [file.scirp.org]
Androgen levels may also be increased if it is an ACTH-dependent form: women experience virilization with hirsutism and cycle disorders. [lecturio.com]
- Recurrent Infection
infections Female patients with Cushing syndrome may also have signs of androgen excess (e.g. hirsutism, acne, changes in libido, virilisation) and menstrual changes 6. [radiopaedia.org]
Cardiovascular
- Hypotension
The test is well tolerated, with the most common side effects being transient facial flushing occurring in 20% of subjects, and rare dyspnea and hypotension. [pituitary.mgh.harvard.edu]
Physical findings that occur in a patient in adrenal crisis include hypotension, abdominal pain, vomiting, and mental confusion (secondary to low serum sodium or hypotension). [aippg.net]
Skin
- Hyperpigmentation
Furthermore, certain patient groups may demonstrate additional or atypical clinical features depending on the etiology of Cushing syndrome 6 : patients with Cushing disease: hyperpigmentation, because melanocyte-stimulating hormone (MSH) and adrenocorticotropic [radiopaedia.org]
Hyperpigmentation is not a feature of primary hypercortisolism. [amboss.com]
Paraneoplastic cortisol production (rare) Signs and Symptoms of Cushing's Syndrome Truncal obesity, moon face Arterial hypertension Diabetes mellitus Muscle weakness, muscle atrophy Menstrual disorders Striae rubrae, acne, edema Mental changes, headache Hyperpigmentation [urology-textbook.com]
[…] levels seen in ectopic ACTH syndrome that overwhelm the 11-beta-hydroxysteroid dehydrogenase enzyme, resulting in hypertension, edema, and hypokalemia as a result of the mineralocorticoid effects of cortisol. 3 Atypically, this patient did not have hyperpigmentation [mdmag.com]
Other effects include hyperpigmentation secondary to high serum levels of ACTH, resulting in a characteristic brown or bronze coloring of the mucous membranes, hair and skin. [wikilectures.eu]
- Ulcer
[…] disease Cataracts “CUSHINGOID” is the acroynm for side effects of corticosteroids : C = Cataracts, U = Ulcers, S = Striae /Skin thinning, H = Hypertension / Hirsutism / Hyperglycemia, I = Infections, N = Necrosis (of femoral head), G = Glucose elevation [amboss.com]
The pharmacist should educate the patient on the prevention of peptic ulcer disease, diabetes, and weight gain. The nurse should educate the patient on the risk of infections and when to follow up with the healthcare provider. [statpearls.com]
The skin shows bad tissue repair with a predisposition for hemorrhages (ecchymosis, hematoma), acne, striae rubrae, atrophy, ulcers, and furuncles. [lecturio.com]
- Gastroenterologic: Peptic ulceration (rare in endogenous hypercortisolism). - Endocrine: Hypothyroidism may occur from anterior pituitary tumors, which can interfere with proper thyroid-releasing hormone (TRH) and thyroid-stimulating hormone (TSH) [aippg.net]
- Alopecia
[…] immune system Adenoid hypertrophy Adipose tissue Adrenal gland Adrenal insufficiency African trypanosomiasis Age-related macular degeneration Aging changes Airway management and ventilation Airways and lungs Alcohol use disorder Alcoholic liver disease Alopecia [amboss.com]
Hirsutism and alopecia, nephrolithiasis (often subclinical), osteoporosis with pathologic fracture, gonadal dysfunction, neurologic difficulties including impairment in cognition and memory, and psychiatric symptoms ranging from anxiety or depression [path.upmc.edu]
Musculoskeletal
- Muscle Weakness
Results: Three months after the discontinuation of retinoic acid treatment (at a dose of 40 mg daily for 6 months, with a 4 month break and then for an additional 6 months), a 17.5-year-old male presented with short stature (-3.0 SD), muscle weakness, [journals.aace.com]
A 69-year-old woman presented to the endocrine teaching clinic for evaluation of progressive weakness over 1 1/2 years. She had proximal muscle weakness with inability to stand from a sitting position and she required assistance to ambulate. [mayoclinic.org]
A 43-year-old gentleman presented with complaints of progressively increasing proximal muscle weakness, low back pain, breathlessness, weight gain, stria and generalized edema for 6 weeks. [ijem.in]
Case: A 26-year-old male patient presented with moon face, purplish striae, supraclavicular fat pads and proximal muscle weakness. [endocrine-abstracts.org]
Typical clinical features include central obesity, thin, easily bruisable skin, abdominal striae, secondary hypertension, hyperglycemia, and proximal muscle weakness. [amboss.com]
- Thin Extremities
There’s increased breakdown of most types of tissue leads to muscle wasting and thin extremities, skin thinning, easy bruising and abdominal striae or lines, bone thinning, or osteoporosis, which can lead to fractures. [osmosis.org]
In addition to the unique patterns of fat deposition, Cushing syndrome patients have thin extremities (spider appearance) that are due to the effects of cortisol on protein disposition in skeletal muscle. [themedicalbiochemistrypage.org]
extremities and central obesity, buffalo hump. [aippg.net]
extremities, decreased bone density (especially spine), kyphosis and back pain (secondary to bone loss), easy bruising and poor wound healing due to thin and atrophic skin [4], acne, psychiatric or emotional disturbances, impaired reproductive function [physio-pedia.com]
Classic clinical features include central obesity and rounded face, 'buffalo hump' caused by fat deposit on the posterior neck, purple skin striae, and thinned extremities due to muscle atrophy. [path.upmc.edu]
Face, Head & Neck
- Round Face
face), and less commonly, acne due to hyperandrogenization. [journals.aace.com]
Symptoms Weight Gain Fatty tissue deposits Excessive hair growth High blood pressure Fatigue Flushing Acne Stretch marks Loss of muscle Anxiety Easy bruising Headache Round face Diagnosis Clinical presentation Urine test Blood test Saliva test – tests [raredr.com]
Typically, patients have the following clinical features 6 : rounded face, sometimes described as 'moon-shaped' progressive centripetal obesity and weight gain prominent supraclavicular fat pad, sometimes known as a 'buffalo hump' purple skin striae easy [radiopaedia.org]
She had a round face but no plethora. Her skin was very frail with multiple bruises noted in all four extremities but no striation in her abdominal area. There was supraclavicular fullness noted. [mayoclinic.org]
Urogenital
- Oligomenorrhea
Results: Only 6 women (17.6%) had a normal cycle, 9- (26.5%) oligomenorrhea, 12-(35.3) amenorrhea and 7- (20.6%) polymenorrhea. [endocrine-abstracts.org]
Hyperandrogenism may exist because of the high ACTH drive, and in women this may be confusing as acne, hirsutism, oligomenorrhea and amenorrhea, are often seen in Cushing’s syndrome. [slideshare.net]
These signs include facial hair, acne and oligomenorrhea. Elevated cortisol causes vasoconstriction, hypertension, sodium retention and hypokalemia. [wikilectures.eu]
Neurologic
- Lethargy
Aminoglutethimide: commonly produces nausea, marked lethargy and rash. Ketoconazole 400mg to 800mg daily. [slideshare.net]
Neuropsychological : lethargy, depression, sleep disturbance, psychosis Musculoskeletal Osteopenia ;, osteoporosis ;, pathological fractures ;, avascular necrosis of the femoral head Muscle atrophy / weakness Endocrine and metabolic Insulin resistance [amboss.com]
Side effects include gastrointestinal symptoms, lethargy, ataxia, hypothyroidism, head ache, bone marrow supression, and skin rash. [clinicaladvisor.com]
Mitotane is a difficult drug to administer: it is given in multiple divided daily doses, and dosage must be carefully titrated to maximize effect and minimize side effects such as diarrhea, vomiting, lethargy, and somnolence. [path.upmc.edu]
- Agitation
Neuropsychiatric changes Include labile mood, agitated depression, anxiety, panic attacks, mild paranoia, impaired short-term memory and cognition, and insomnia. SeeTable II for signs and symptoms in patients with Cushing’s syndrome. Table II. [clinicaladvisor.com]
- Asthenia
Her main complaint was a severe asthenia associated with impaired night rest, weight gain, nocturia, and decreased of libido. [elsevier.es]
Main side effects are digestive (nausea, vomiting, diarrhea), neurologic (sleepiness, asthenia) and metabolic (hypercholesterolemia). [ojrd.biomedcentral.com]
Workup
[…] ovarian tumors adrenocorticotropin (ACTH)-independent macronodular adrenocortical hyperplasia (AIMAH) : rare cause of ACTH-independent Cushing syndrome 4 corticotropin-releasing hormone-secreting tumor: very rare hypothalamic tumors ectopic production The workup [radiopaedia.org]
A traditional workup of a patient with ACTH-dependent Cushing's syndrome might include a clinical history, biochemical testing, neuroimaging, and an inferior petrosal sinus sampling (IPSS). [pituitary.org]
Because acute illness activates the HPA axis, resulting in increases in ACTH and cortisol, the laboratory workup for Cushing syndrome should not be performed when subjects are acutely ill. [aippg.net]
[…] hw71687.html ↑ 7.0 7.1 7.2 Gail K Adler, MD, PhD, FAHA, Assistant Professor, Department of Medicine, Division of Endocrinology, Diabetes and Hypertension, Brigham and Women's Hospital, Harvard Medical School Cushing Syndrome: Differential Diagnoses & Workup [physio-pedia.com]
The endocrinologist needs to be aware of the pitfalls of the tests in use, and that these may need repeating over time if the source of ACTH is not found at initial diagnostic workup. REFERENCES 1. Newell-Price J, Bertagna X, Grossman AB, Nieman LK. [scielo.br]
Treatment
We present a 45-year-old woman with CD who started treatment with cabergoline as the first and only treatment for her disease. [elsevier.es]
As more targeted-approaches are now available, mitotane remains but a remote option for medical treatment in benign CS. [thieme-connect.com]
The primary study hypothesis is that FKBP5 levels are elevated in patients with Cushing's syndrome, and these levels decrease after successful surgical treatment. [clinicaltrials.gov]
A collaboration between MRC Technology and Prof Adrian Clark and Dr Li Chan at the William Harvey Research Institute, Queen Mary University of London has been launched to develop melanocortin receptor type 2 (MC2R) antagonists for the treatment of ACTH-dependent [lifearc.org]
Conclusion: Retinoic acid treatment may alter the clinical presentation of ACTH-dependent CS and consequently delay the diagnosis. [journals.aace.com]
Prognosis
The prognosis is worsened in the setting of an unresectable hormone-secreting tumor. [statpearls.com]
[…] features, treatment and survival of ECS-NET patients Materials and methods: Retrospective analysis of data on ECS-NET from 17 centers, obtained by a questionnaire Conference: 13th Annual ENETS conference (2016) Category: Epidemiology/Natural history/Prognosis [enets.org]
Adrenal carcinoma These are very aggressive and the prognosis is poor. Generally, if there are not widespread metastasis, then you should treat the primary tumour surgically. [almostadoctor.co.uk]
Prognosis is favorable if surgery is curative. The rare adrenocortical carcinomas are associated with a 5-year survival rate of 30% or less. [emedicine.medscape.com]
Treatment and prognosis Management depends on the specific etiology 6. [radiopaedia.org]
Etiology
Treatment and prognosis Management depends on the specific etiology 6. [radiopaedia.org]
If the ACTH level is low (20 pg/mL), then the etiology is ACTH-dependent (pituitary adenoma, ectopic source, or CRH secretion). High-dose dexamethasone suppression testing and CRH stimulation test will confirm the etiology. [usmleforum.com]
Published: July 11, 2013 By Sherry Boschert, Family Practice News Digital Network SAN FRANCISCO – The size of a pituitary tumor on magnetic resonance imaging in a patient with ACTH-dependent Cushing's syndrome can't differentiate between etiologies, but [pituitary.org]
License: CC BY 3.0 Definition and Etiology of Hypercortisolism The etiology differentiates between an exogenous hypercortisolism, due to long-term treatment with glucocorticosteroids, and an endogenous increased production and secretion of cortisol or [lecturio.com]
Furthermore, if the lung nodule is a benign lung hemangioma, we recommend that physicians not exclude it as the etiology of Cushing syndrome. [kjim.org]
Epidemiology
[…] multicenter study on clinicopathologic features, treatment and survival of ECS-NET patients Materials and methods: Retrospective analysis of data on ECS-NET from 17 centers, obtained by a questionnaire Conference: 13th Annual ENETS conference (2016) Category: Epidemiology [enets.org]
Background/Epidemiology/Aetiology Clinical syndrome due to chronic exposure to excess glucocorticoid hormones irrespective of the underlying cause. [dundeemedstudentnotes.wordpress.com]
Cushing's syndrome: epidemiology and developments in disease management. Clin Epidemiol 2015; 7:281–293 [Google Scholar] 8. Steffensen C, Bak AM, Rubeck KZ, Jorgensen JO. Epidemiology of Cushing's syndrome. [ajronline.org]
Morbidity and mortality in Cushing's disease: an epidemiological approach. Clin Endocrinol (Oxf). 1994;40(4):479-84. [hopkinsguides.com]
Epidemiology Prevalence 5–6/100.000 Most often women in the 3rd and 4th decade of life Causes (Etiology) of Cushing's Syndrome ACTH-dependent Cushing's Syndrome (Overproduction of ACTH): Pituitary adenoma (Cushing's disease) Paraneoplastic ACTH production [urology-textbook.com]
Pathophysiology
Understanding Pathophysiology (3rd Ed.). St. Louis, MO: Mosby. Nieman, L.K. (2012). Causes and pathophysiology of Cushing’s syndrome. In A. Lacroix and K.A. Martin (Eds.), UpToDate. [wikilectures.eu]
Useful For Suggests clinical disorders or settings where the test may be helpful Determining the cause of hypercortisolism and hypocortisolism Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to [mayocliniclabs.com]
Study Group of the Italian Society of Endocrinology on the Pathophysiology of the hypothalamic–pituitary–adrenal axis. [link.springer.com]
Louis: Saunders Elsevier; 2009: 481-483. ↑ 3.0 3.1 3.2 Harold J Bruyere: 100 Case Studies in Pathophysiology. [physio-pedia.com]
Prevention
At the time of discharge, the patient should have a dietary consult and how to prevent osteoporosis. The pharmacist should educate the patient on the prevention of peptic ulcer disease, diabetes, and weight gain. [statpearls.com]
Reagan Authors note: Purdue University Year: 2000 Reference: Veterinaria n.2 Supplemento Ottobre 2000 Pages: 37 - 46 Download attachments: PDF (21682 Downloads) Related items Leishmaniasis prevention: what should be known before recommending a topical [veterinaria.scivac.org]
To prevent weight gain, aerobic exercise is good to increase your heart rate. Examples of aerobic exercise include fast walking, jogging, cycling, and swimming. [6] Education on avoiding falls and removing loose rugs and other hazards in the home. [physio-pedia.com]
A false-positive test can be caused by drugs which increase the metabolic breakdown of dexamethasone, preventing its ability to suppress cortisol levels (phenytoin, carbamazepine, and rifampin). [usmleforum.com]
Because glucocorticoids suppress the immune system, they reduce inflammation and are also frequently given to people who have had organ transplants to prevent the immune cells from attacking the transplant. [endocrinediseases.org]