Cushing's Syndrome Flashcards

1
Q

Define Cushing’s Syndrome?

A

Syndrome associated with chronic inappropriate elevation of free circulating cortisol

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2
Q

How can Cushing’s Syndrome be divided?

A
ACTH Dependent (80%)
ACTH Independent (20%)
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3
Q

What are some of the ACTH Dependent Cushing’s Syndromes?

A
Excess ACTH froma a pituitary adenoma (Cushing's Disease)
Ectopic ACTH (e.g. lung cancer, pulmonary carcinoid tumours)
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4
Q

What are some of the ACTH Independent Cushing’s Syndromes?

A

Benign Adrenal Adenoma

Adrenal Carcinoma

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5
Q

What is the epidemiology of Cushing’s Syndrome?

A

Incidence: 2-4/1,000,000 per year

Peak incidence: 20-40 yrs

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6
Q

What are the presenting symptoms of Cushing’s Syndrome?

A
Increasing Weight 
Fatigue
Muscle Weakness 
Myalgia 
Thin Skin
Easy Bruising 
Poor Wound Healing 
Fractures 
Hirsuitism 
Acne 
Frontal Balding 
Oligomenorrhoea/amenorrhoea 
Depression or psychosis
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7
Q

What are the signs of Cushing’s Syndrome on physical examination?

A
Moon Face 
Facial Plethora 
Interscapular Fat pad 
Proximal Muscle Weakness 
Central Obesity 
Pink/Purple striae on abdomen/breast/thighs
Kyphosis 
Hypertension
Ankle Oedema
Pigmentation in ACTH dependent cases
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8
Q

What is Kyphosis due to in Cushing’s Syndrome?

A

Vertebral Fracture

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9
Q

What is Ankle Oedema due to in Cushing’s Syndrome?

A

Salt and water retention from the mineralocorticoid effect of excess cortisol

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10
Q

On which patients are investigations performed in Cushing’s Syndrome?

A

On patients with a high pre-test probability

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11
Q

What bloods do you do for Cushing’s Syndrome and what might you expect?

A

U&Es - hypokalaemia due to mineralocorticoid effect

BM - High Glucose

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12
Q

What Initial High-Sensitivity Tests do you do for Cushing’s Syndrome?

A

Urinary free cortisol
Late-night salivary cortisol
Overnight dexamethasone suppression test
Low dose dexamethasone suppression test (LDDST)

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13
Q

How does the Low Dose Dexamethasone Suppression Test work?

A

Give 0.5 mg dexamethasone orally every 6 hrs for 48 hrs
In Cushing’s Syndrome, serum cortisol measured 48 hrs after the first dose of dexamethasone fails to suppress below 50 nmol/L

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14
Q

What test would you do to determine the underlying cause of an ACTH-independent Cushing’s Syndrome (Adrenal Adenoma/Carcinoma)?

A

Low plasma ACTH

CT or MRI of adrenals

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15
Q

What tests would you do to determine the cause of an ACTH-dependent Cushing’s Syndrome (pituitary adenoma)?

A

High plasma ACTH
Pituitary MRI
High-Dose dexamethasone SuppressionTest
Inferior Petrosal Sinus sampling (superior to high-dose dexamethasone suppression test)

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16
Q

What might you see on Inferior petrosal sinus sampling for ACTH-dependent Cushing’s Syndrome (Pituitary Adenoma)?

A

Central : peripheral ratio of venous ACTH > 2:1 (or > 3:1 after CRH administration) in Cushing’s Disease

17
Q

What tests would you do for ACTH-Dependent Cushing’s Syndrome (Ectopic)?

A

If lung cancer suspected: CXR, sputum cytology, bronchoscopy, CT scan
Radiolabelled octreotide scans can detect carcinoid tumours because they express somatostatin receptors

18
Q

What is the management plan for Cushing’s Syndrome if iatrogenic?

A

Discontinue steroids, use lower dose or use a steroid-sparing agent

19
Q

What is the medical management plan for Cushing’s Syndrome?

A

Used pre-operatively or if unfit for surgery
Inhibit cortisol synthesis with metyrapone or ketoconazole
Treat osteoporosis
Physiotherapy for muscle weakness

20
Q

What is the surgical management plan for Cushing’s Syndrome?

A

Pituitary Adenomas: trans-sphenoidal adenoma resection
Adrenal Adenoma/Carcinoma - surgical removal of tumour
Ectopic ACTH - treatment directed at the tumour

21
Q

When would you do Radiotherapy in Cushing’s Syndrome?

A

Performed in those who are not cured and have persistent high cortisol after trans-sphenoidal resection of the tumour

22
Q

When can Bilateral Adrenalectomy be performed?

A

May be performed in refractory Cushing’s Disease

23
Q

What are the possible complications of Cushing’s Syndrome?

A

Diabetes
Osteoporosis
Hypertension
Pre-disposition to infections

24
Q

What are the complications of surgery in Cushing’s Syndrome treatment?

A

CSF leakage
Meningitis
Sphenoid Sinusitis
Hypopituitarism

25
Q

What are the complications of Radiotherapy in Cushing’s Syndrome treatment?

A

Hypopituitarism
Radionecrosis
Increased risk of second intracranial tumours and stroke

26
Q

How can Bilateral Adrenalectomy be complicated?

A

By the development of Nelson’s Syndrome (locally aggressive pituitary tumour causing skin pigmentation due to ACTH secretion)

27
Q

What is the prognosis for patients with Cushing’s Syndrome?

A

Untreated - 5 yr survival = 50%

Depression persists for many years following treatment