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The Glucocorticoid axis

Reem Alyahya
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.
Learning objectives:
• Which additional radiological investigations do you recommend?

• Which treatment would you propose?


What is Cushing’s syndrome?
• Cushing syndrome is caused by prolonged exposure to elevated levels of
either endogenous glucocorticoids or exogenous glucocorticoids.

endogenous exogenous

• glucocorticoid • Following the


overproduction therapeutic
or adminstrition of
hypercortisolism synthetic
• Due to primary steroids of
adrenocortical ACTH.
neoplasm
Cushing’s syndrome VS Cushing’s disease ?
• Cushing’s syndrome : • Cushing’s Disease:

- clinical state of increased free - ACTH dependent


circulating glucocorticoid hypercorticism , pituitary
dependant.
- Spontaneous Cushing’s
syndrome rare - it is the most common cause of
Cushing's.
Radiological investigations
Radiological investigations
 It should be performed after the biochemical laboratory evaluation has
been done.
 It’s used to determine the cause or complications.

Radiolabelled
Adrenal CT or
Pituitary MRI Chest X-ray octreotide
MRI
(Scintigraphy)
Adrenal CT or MRI:

• Adrenal adenomas and


carcinomas are relatively large
and can be detected by CT scan
imaging.
Pituitary MRI
• A pituitary adenoma can be seen
but it is often small and not
visible in a significant proportion
of cases.
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).
Radiolabelled octreotide
(Scintigraphy)

• involves injection of a radioactive


substance followed by an imaging
scan.
• Occasionally used in locating
ectopic ACTH tumors.
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.
Depending
pharmacotherapy on the
cause.

Radiotherapy Surgery
Pharmacotherapy (Cushing’s syndrome)
• The goal of pharmacotherapy is to reduce morbidity and prevent
complications.

Somatostatin Adrenal steroid


Analogs inhibitors
• bind and activate human • These agents either
somatostatin receptors inhibit the synthesis of
resulting in inhibition of mineralocorticoids and
ACTH secretion, which glucocorticoids, or
leads to decreased competitively bind
cortisol secretion. glucocorticoid receptors.
• pasireotide • Metyrapone
0.6-0.9 twice daily 750 mg – 4 g daily , in 3-4
divided doses.
• Ketoconazole :200 mg
three times daily
Surgery and radiotherapy (Cushing’s disease)

Trans-sphenoidal
removal of the
tumor.

Pituitary Bilateral
irradiation adrenalectomy
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.


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.
Radiotherapy (pituitary irrdiation).
• Alone is slow acting.

• Only effective in 50-60% even


after prolonged follow up
• Used mainly after failed pituitary
surgery.
Cushing’s syndrome due to other causes.
 Adrenal adenomas:  Adrenal carcinoma:
- should be resected after - are highly aggressive and has
achievement of clinical remission poor prognosis.
with metyrapone. - 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.
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.
• Kumar & Clarks, Clinical Medicine ,
eighth edition (2012).

• Up to date : Cushing's syndrome.

• Up to date : Cushing's syndrome


treatment (Beyond the Basics)
References
• Emedicine : medscape: Cushing’s
syndrome.
Thank you for listening.
Any question?

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