Addisons disease Flashcards
What causes addisons disease
Lack of aldosterone and coritisol
primary hypoadrenalism
Why does addisons cause bronzed skin and where is the first place its seen
Melanin production from ACTH excess
First place seen is the gums
What treat addisonian crisis with
Hydrocortisone IV + fluids
What is an acute adrenal crisis
hypovolaemic shock as no pressure from adrenergic receptors
Hypoglycaemia
What electrolyte imbalance is seen in addisons
Acidosis - hyperkalmaeia
What need to check before giving levothyroxine
Cortisol levels - levothyrozine increases cortisol metabolism - can send into addisonian crisis
Cortisol function
- Blood sugar regulation
- BP control
- Antinflammatory effects
What is addisons disease
Primary adrenal insuffiency -> lack of aldosterone and cortisol due to destruction of adrenal cortex
Excess ACTH
Causes of addisons
Autoimmune
TB - most common globallu
Adrenal metastasis
Bilateral adrenalectomy
Adrenal haemorrhage
Infections
Genetic conditions
pROLONGED use of glucocorticids or drugs inhibiting steroid production eg ketoconazole supress HPA axis -> adrenal atrophy and insufficiency
Infections that can cause addisons
histoplasmosis, crytpococcosis, CMV, HIV
Genetic conditions causing addisons
Amyloidosis and haemochromatosis
Congenital adrenal hyperplasia
Congenital or neonatal primary adrenal insuffficiency - CYP21A2, AIRE 9APS-1)
Why is there excess of ACTH in addisons disease
Lack of aldosterone and cortisol stimualtes release but cortex cant respond to ACTH due to destruction
Prognosis of addisons
20-30 yr old women more common
If untreated always fatal
Require lifelong treatment, premature risk of death
Clinical features of addisons
Weight loss
Anorexia
Slat cravings
Malaise + easy fatiguabilit
Weakness
Fever
Depression
Impotence/amenorrhea
N+V
Diarrhoea Confusion
Syncope
Abdo pin
Constipation
Myalgia
Joint or back pain
Signs of addisons disease
New scars, palmar crease pigementation
Buccal pigmentation
Postural hypotension
Weight loss
General
Dehydration
Loss of body hair
Vitilifo
WHat causes pigmentation in addisos
POMC -> increased Melanocyte StimHoromne - dark skin
Electrolytes in addisons
Hyponatermia
Hyperkalemia
Metabolic acidosis
How do a short synacthen test
Cortisol at 0 minutes - gie ACTH 250mg
Cortisol level at 30+60 mins
What result from short synacthen test diangoses addisons
<500 at 30 or 60 mins
When should short synacthen test be perfrmed
18-24 hours after last steroid dose
Management of addisons
Gluco and mineralcorticoid replacement
Glucocorticoid replacement - hydrocortisone, prednisolone, dexamethasone
Mineralcorticoid replacement - fludrocortisone
When do yuo double glucocortiocids taken in addisons
unwell - moderate fever, infection with antibiotics, local anaesthetis
What causes an addisonian crisis
Lack of steroids - first presentation of addisons or not enough steroids given, taken, retained etc
Clinical features of addisons
Fatigue, weight loss, no energy
Hypotnesion 0 dizzym collapse, shok
Abdo pain - tender, guarding, N+V
Fever
Confusion - somnolence, delirium, coma
Back and leg cramps
Biochem results in addiosnian crisis
Hyponatermia
hyperkaelmia
Hypoglycaemia
Pre renal failure
Investigations in addisons cirsis
Cortiosl, U+Es, TFTs, ACTH, FBC
BP
Drug hisory #Dont wait to treat if sus
Treatment of addisonian crisis
IV bolus of 100mg IV hydrocortisone
Cont 200mg IV hydro per 24 hours, or 50mg every 6
Rehydration - rapid 1000ml first hour
When give hydrocortisone IV 100mg in addisons
Severe illness eg persistent vomitting, prep for colonosopy, acute trauma or surgery any major stressors
What give in adrenal crisis initially
IM hydrocortisone - 100mg emergency it
Why is it important to treat an addisonian crisis so quickly
Hypovolaemia -> ischamia and end organ damage
Death
What 9amcortiisol level would make addisons more or less liely
> 500 - v unlikely
<100 = v abnormal
100-500 - > ACTH stim tst
What antibpdies can be seen in addisons
anti-21-hydroxylase
What need to monitor in addisons
Osteoporosis due to long term steroid use - DEXA scans and give vit D
Addisons definitive investiation
ACTH stimulation test - short synacthen test
How is ACTH test done
PLasma cortisol measred before and 30 mins after 250ug IM stnacthen
Management addisons crisis
hydrocortisone 100 mg im or iv
1 litre normal saline infused over 30-60 mins or with dextrose if hypoglycaemic
continue hydrocortisone 6 hourly until the patient is stable.
oral replacement may begin after 24 hours and be reduced to maintenance over 3-4 days
