Addison's Disease Flashcards
Adrenal Insufficiency Types
- Primary insufficiency (Addison’s)- inability of the adrenal glands to produce sufficient steroid hormones, most common cause is autoimmune
- Secondary insufficiency- inadequate pituitary or hypothalamic stimulation of the adrenal glands
Adrenal Insufficiency Epidemiology
Primary insufficiency is relatively rare
Secondary insufficiency is more common as there are lots of factors that can lead to the suppression of the hypothalamic-pituitary axis (most common is exogenous steroid use)
Adrenal Insufficiency Aetiology
- Addisons is used to describe primary adrenal insufficiency which can have many causes
- TB in developing world
- Autoimmune adrenal destruction is most common cause in Western Europe
- Autoimmune adrenal destruction is isolated in 40% of cases and part of autoimmune polyendocrinopathy syndrome in 60%
- Exogenous steroids is most common cause of secondary insufficiency
Congenital Adrenal Hyperplasia Pathogenesis
- Genetic mutation leads to impaired production of cortisol
- Body recognises reduced cortisol and tries to stimulate adrenal glands further
- However no cortisol can be produced so androgens and sometime aldosterone are produced in large quantities but still no cortisol
- Virilisation occurs
- Most common cause of childhood adrenal insufficiency
Adrenal Insufficiency In Critically Ill Patients
- Increasingly more reported
- CIRCI (critical illness related corticosteroid insufficiency)
- Conditions where adrenal insufficiency may occur include
- Sepsis, severe pneumonia, ARD…
- Should suspect if hypotension does not improve
Adrenal Insufficiency Presentation
- Symptoms are often mild and nonspecific
- Presentation depends on rapidity of loss of adrenal function
Acute Presentation
- May be as a crisis precipitated by infection, surgery or trauma
- Presents with hypotension, hypovolaemic shock, acute abdominal pain, low-grade fever and vomiting
- Sudden onset e.g. WF, presents with collapse and shock
Chronic Presentation
- Symptoms develop insidiously and may be mild
- Fatigue
- Anorexia
- Nausea
- Vomiting
- Weight loss, cravings for salt, dizziness, hypotension GI upset
- Hyperpigmentation (buccal mucosa, lips, palmar creases, scars, pressure areas e.g. knuckles and knees
- Hypotension, postural hypotension
Giving levothyroxine can use up cortisol and symptoms can get worse in those with coexisting Addisons and hypothyroidism
Adrenal Insufficiency Ix
- In early stages investigations can be normal
- Hyponatraemia, hyperkalaemia, hypercalcaemia (rarer)
- FBC may show mild anaemia, eosinophilia, lymphocytosis
- LFTs
- Cortisol (blood tests should be taken between 8 and 9am when levels are highest
- ACTH
- Renin and aldosterone for indication of mineralocorticoid activity
- Synacthen test confirm diagnosis (administer ACTH monitor cortisol)
Adrenal Insufficiency Associated Diseases
- Most cases are autoimmune in origin, comorbidity with other autoimmune diseases common
- Thyroid, DM, pernicious anaemia, vitiligo, premature ovarian failure
Adrenal Insufficiency Management
- Education
- Glucocorticoid replacement with hydrocortisone is mainstay, highest dose in morning
- Increase glucocorticoid during minor illness
- Do not replace thyroid before glucocorticoid
- Mineralocorticoid replacement with fludrocortisone
- Ongoing monitoring annually to ascertain symptoms
- Screen annually for other autoimmune conditions (TFTs, glucose, HbA1c, FBC, B12, coeliac)
Adrenal Crisis
- Fatigue, nausea malaise
- Progressing to hypotension and hypovolaemia
- Urgent admission
- Immediate hydrocortisone IV
- Rehydration with saline
- Continuous cardiac and electrolyte monitoring
- Treat underlying disorders
- Can be caused when long term steroids are stopped, hypothalamic-pituitary axis has been suppressed
- Can be caused by infection
Adrenal Insufficiency Complications
- Adrenal crisis
- Reduced quality of life
- Osteoporosis (usually not a risk but some people require regular high doses)