cushing's syndrome
DESCRIPTION
this presentation contains the detailed radiological invesstigations and treatment of Cushing's syndrome.TRANSCRIPT
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The Glucocorticoid axisReem Alyahya
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Introduction• A 46 y/o man present with fever, red tender
area on his right leg, consistent with erysipelas.
• His recent medical history reveals fatigue, easy bruising and wt. gain in the past 6 months.
• His family history is negative for DM and HT.
• physical examination shows central obesity (BMI 32.5 kg/m2, waist circumference 115 cm)
• BP 160/104
• Moon face appearance, a dorsal fat pad in the neck and abdominal purple striae.
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Learning objectives:
• Which additional radiological investigations do you recommend?
• Which treatment would you propose?
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What is Cushing’s syndrome?
• Cushing syndrome is caused by prolonged exposure to elevated levels of either endogenous glucocorticoids or exogenous glucocorticoids.
endogenous
• glucocorticoid overproduction or hypercortisolism
• Due to primary adrenocortical neoplasm
exogenous
• Following the therapeutic adminstrition of synthetic steroids of ACTH.
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Cushing’s syndrome VS Cushing’s disease ?
• Cushing’s syndrome :
- clinical state of increased free circulating glucocorticoid
- Spontaneous Cushing’s syndrome rare
• Cushing’s Disease:
- ACTH dependent hypercorticism , pituitary dependant.
- it is the most common cause of Cushing's.
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Radiological investigations
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Radiological investigations
It should be performed after the biochemical laboratory evaluation has been done.
It’s used to determine the cause or complications.
Adrenal CT or MRI Pituitary MRI Chest X-rayRadiolabelled
octreotide(Scintigraphy)
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Adrenal CT or MRI:
• Adrenal adenomas and carcinomas are relatively large and can be detected by CT scan imaging.
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Pituitary MRI
• A pituitary adenoma can be seen but it is often small and not visible in a significant proportion of cases.
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Chest X-ray
• It is used in patients with suspected ectopic ACTH production.
• The ACTH-secreting tumors are often oat-cell carcinomas of the lung (bronchus carcinoma).
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Radiolabelled octreotide(Scintigraphy)
• involves injection of a radioactive substance followed by an imaging scan.
• Occasionally used in locating ectopic ACTH tumors.
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Treatment
• Successful treatment of Cushing’s syndrome should lead to reversal of the presenting clinical features.
• However, untreated Cushing’s syndrome has a very bad prognosis, with death from: hypertension, MI, infection and heart failure.
• Whatever the underlying cause, cortisol hypersecretion should be controlled prior to surgery or radiotherapy.
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pharmacotherapy
Surgery Radiotherapy
Depending on the cause.
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Pharmacotherapy (Cushing’s syndrome)• The goal of pharmacotherapy is to reduce morbidity and prevent
complications.
Somatostatin Analogs
• bind and activate human somatostatin receptors resulting in inhibition of ACTH secretion, which leads to decreased cortisol secretion.
• pasireotide 0.6-0.9 twice daily
Adrenal steroid inhibitors
• These agents either inhibit the synthesis of mineralocorticoids and glucocorticoids, or competitively bind glucocorticoid receptors.
• Metyrapone750 mg – 4 g daily , in 3-4 divided doses.
• Ketoconazole :200 mg three times daily
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Surgery and radiotherapy (Cushing’s disease)
Trans-sphenoidal removal of the
tumor.
Bilateral adrenalectomy
Pituitary irradiation
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Trans-sphenoidal removal of the tumor
• Is the treatment of choice.
• Result in remission of 75-80% of the cases.
• But the results vary considerably.
• Experienced surgeon is essential.
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Bilateral adrenalectomy
• It’s an effective last resort if other measures fail to control the disease.
• The patient will need hydrocortisone (cortisol) replacement therapy after surgery, and possibly continued throughout life.
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Radiotherapy (pituitary irrdiation).
• Alone is slow acting.
• Only effective in 50-60% even after prolonged follow up
• Used mainly after failed pituitary surgery.
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Cushing’s syndrome due to other causes.
Adrenal adenomas: - should be resected after achievement of clinical remission with metyrapone.
Adrenal carcinoma:- are highly aggressive and has poor prognosis.- if there’s no widespread metastases, tumor bulk should be removed surgically.- adrenolytic drug mitotane may inhibit the growth of the tumor and prolonged survival.- radiotherapy can be used.
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conclusion
• A 46 y/o man present with fatigue, easy bruising and wt. gain
• The patient had moon face appearance, a dorsal fat pad in the neck and abdominal purple striae.
• The diagnostic tests reveals that the patient has Cushing's syndrome.
• The patient will initially manage the cortisol levels by taking metyrapone 4 gm/ 3 daily, Ketoconazole, 200 mg /3 daily
• Further treatment decided based on the specific etiology.
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References
• Kumar & Clarks, Clinical Medicine , eighth edition (2012).
• Up to date : Cushing's syndrome.
• Up to date : Cushing's syndrome treatment (Beyond the Basics)
• Emedicine : medscape: Cushing’s syndrome.
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Thank you for listening.Any question?