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Hypercortisolism (Cushing's disease/syndrome) (Clinical presentation…
Hypercortisolism
(Cushing's disease/syndrome)
Prognosis
Good if promptly treated
Main complications are CVD
Definition
Endocrine disorder of
excess cortisol production
Epidemiology
Cushing syndrome uncommon
Cushing disease very rare
Pathophysiology
Primary Cushings (Cushing syndrome)
Dysfunction of adrenal cortex, excess cortisol released
Loss of circadian rhythm of cortisol release (usually high AM)
Secondary Cushings (Cushing's disease)
Pituitary or ectopic tumour, releasing excess ACTH (or CRH, but rare)
Causes bilateral adrenal hyperplasia
Normal function
Hypothalamus secretes CRH; pituitary secretes ACTH to adrenals, which produce glucocoticoids (cortisol)
Cortisol responsible for metabolic functions
Adrenals also produce mineralocorticoids (aldosterone) and sex steroids (androgens, DHEA), independent of HPA
Aetiology
Primary
Neoplastic: adrenal tumour, adrenal hyperplasia
Drugs: excess exogenous steroids
Congenital/genetic: McCune Albright syndrome
Secondary
Neoplastic: pituitary tumour, ectopic tumour
(e.g. lung, thyroid, prostate)
Clinical
presentation
Buffalo hump)
Thin skin
Round (moon) face
Striae
Central obesity
Bruising
Proximal weakness
Acne
Hirsuitism
Amenorrhoea/oligomenorrhoea
Depression
Diagnosis
Examination
General exam
Moon faces, acne, hirsuitism, supraclavicular fat pads, buffalo hump, central obesity, striae, bruising, proximal muscle wasting,
if secondary to ACTH tumour, skin pigmentation seen
Neuro exam
Proximal muscle weakness
Investigations
Bloods
FBC, CRP, U+E, LFT, TFT, glucose (DM),
lipids (hyperlipidemia), bone profile (high ALP, Ca, phos),
Hormones (high cortisol with loss of diurnal rhythm,
high ACTH if pituitary/ectopic, low if adrenal)
Imaging
MRI head (pituitary tumour)
CT abdo: (adrenal hyperplasia/tumour)
CXR/CT chest (lung tumours)
MRI/CT contrast (locate ectopic tumours
DEXA (osteoporosis)
Bedside
Obs (HTN), ECG
Measurements (weight gain)
Special tests
Overnight dex suppression test
(dex at midnight, measure cortisol AM, normally suppressed but stays high in Cushings)
48h dex suppression test
(dex for 2d, compare BL and 48h cortisol, failure to suppress is Cushings; adrenal/ectopic is only partly suppressed, pituitary is suppressed)
Plasma ACTH
(if low, then adrenal, if high, then pituitary/ectopic)
CRH test
(cortisol rise if pituitary, not with ectopic)
Serum K
(low in ectopic, normal in pituitary)
Sphenoid sinus blood
(high if pituitary disease vs ectopic)
History
DH
Meds steroids), allergies
FH
Cushings disease, endocrine diseases,
cancers
PMH
Known medical conditions, DM, cancers
Previous surgery
SH
Living arrangements, occupation,
smoking, alcohol, diet
PC/HPC
Appearance changes, weight gain,
bruising and stretch marks,
acne, excess hair, low mood
Management
Medical
Adrenolytics
Indication: adjunct to surgery for adenoma, carcinome
E.g. mitotane
MOA: destroy adrenal tissue
Radiotherapy
Indication: adjuct to surgery in adenoma/ carcinoma
Steroidogenesis inhibitors
Indication: ectopic disease unsuitable for surgery,
pre-surgery to manage symptoms
E.g. ketoconazole, fluconazole
MOA: steroidogenesis inhibitors, prevent cortisol synthesis
ACTH antagonists
Indication: pituitary diease awaiting surgery
Surgical
Transphenoidal surgery
Indication: pituitary tumour
MOA: removal of tumour via nose
Adrenolectomy
Indication: adrenal adenoma or carcinoma
MOA: removal of adrenal gland laparoscopically;
if bilateral, may need to remove both
SEs: Nelson's syndrome if bilateral adrenalectomy (enlarging invasive pituitary tumour with HIGH ACTH and skin pigmentation)
Ectopic tumour surgery
Indication: ectopic e.g. lung
MOA: surgical removal of tumour
Conservative
Information, advice, support
Investigate and manage cause