ADDISON’S

DISEASE

GROUP 4
CASE • A 19-year-old student presents with an 18-month
history ofprogressive tiredness, weight loss and
SCENARIO lethargy. One year before presentation she had a
common cold that forced her to stay away from
lectures for2 days. Three months before
presentation, another common cold laid her low for
almost 2 weeks, duringwhich time she lost 3 kg in
weight. The local student health doctors assured her
that the symptoms werethe result of either being
pregnant or taking drugs. On examination, she was
noted to have darker skinthan her mother and
pigmented palmar creases.
Q1: WHAT DO YOU THINK THE LIKELY DIAGNOSIS IS?

• This is fairly classic presentation of Addison’s disease. it is relatively

easy in retrospect to put together the sign and symptoms of Addison’s
disease, but it can be very difficult to recognize, particularly in its
early stages, when adrenal function is only suboptimal under stress.
• Addison's disease, also called adrenal insufficiency, is an uncommon
disorder that occurs when your body doesn't produce enough of
certain hormones. In Addison's disease, your adrenal glands, located
just above your kidneys, produce too little cortisol and, often, too
little aldosterone. Signs and symptoms include extreme fatigue,
weight loss and decreased appetite, darkening of the skin and etc.
which is stated on the scenario.
Q2: WHAT TESTS COULD
YOU DO TO CONFIRM THIS?
• The diagnosis is confirmed by laboratory test results.
Combined measurements of early-morning serum cortisol and
plasma ACTH are performed to differentiate primary adrenal
insufficiency from secondary adrenal insufficiency and from
normal adrenal function. Patients with primary insufficiency
have a greatly increased plasma ACTH level (more than 22.0
pmol/L) and a serum cortisol concentration lower than the
normal range (less than 165 nmol/L) or in the low-normal
range. Other laboratory findings include decreased levels of
blood glucose (hypoglycemia) and sodium (hyponatremia), an
increased serum potassium concentration (hyperkalemia), and
an increased white blood cell count (leukocytosis).
Q2: WHAT • The diagnosis is confirmed by low levels of adrenocortical
hormones in the blood or urine and decreased serum cortisol
TESTS COULD levels. If the adrenal cortex is destroyed, baseline values are
low, and ACTH administration fails to cause the normal
YOU DO TO increase in plasma cortisol and urinary 17-

CONFIRM hydroxycorticosteroids. If the adrenal gland is normal but

not stimulated properly by the pituitary, a normal response
THIS? to repeated doses of exogenous ACTH is seen, but no
response occurs after the administration of metyrapone
(Metopirone), which stimulates endogenous ACTH.
Q3: WHAT In the developed world at least 80% of cases Addison’s disease are caused by
autoimmune destruction of the adrenal glands. Tuberculosis remains a
relatively common Addison’s disease in the absence of autoimmune disease

TESTS and is suggested by the presence of enlarged adrenal glands with necrotic areas
and often dots of calcification visible on computed tomography (CT). In boys
under 16years of age, X linked adrenoleukodystrophy, diagnosed by measuring

WOULD HELP an increase in circulating very-long chain fatty acids, is an important cause of
adrenal failure. In older patients, measurement of anti-cardiolipin antibodies, a
marker of primary antiphospholipid syndrome, is useful if features such as

ESTABLISH recurrent venous thrombosis suggest that the condition is not idiopathic.

THE CAUSE? In primary adrenal failure, a random plasma ACTH will be

high. In secondary adrenal failure, the sign and symptoms
related to the conditions are usually much more subtle and
the biochemical signs do not appear. ACTH will be low or
low-normal and the cortisol response to exogenouse
ACTH will be suboptimal.
Q4: WHAT IS • Glucocorticoid replacement (usuallu with 10-15 mg
hydrocortisone first thing in the morning and
THE INITIAL another 5mg hydrocortisone around 4pm) is
MANAGEMENT required immediately. In primary Addison’s disease,
? mineralocorticoid replacement with fludcortisone
50-100 day is also required. As Addison’s disease is
often disclosed by an episode of acute stress, it is
not uncommon for glucocorticoid treatment to be
given parenterally and at a higher dose at least
initially.
 Patients with autoimmune Addison’s disease are more likely to have other
autoimmune diseases, and a proportion of them to claim to ‘not feel right’ despite
what appears to be adequate replacement therapy. Under conditions of stress, patients
should be asked to step up their glucocorticoid dose two or threefold for a few days,
Q5: WHAT ARE THE LONG- they are asked to wear a medic Alert braceletor necklace and carry an injectible form
TERM of glucocorticoid to use if they find themselves unable to take oral steroids. For some
patients a longer-acting glucocorticoid such as prednisolone seems to be more
COMPLICATIONS/SEQUELAE? comfortable that the short-aching hydrocortisone.

Hypoglycemia

Low levels of cortisol

Complications from adrenal crisis

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