Adrenal Insufficiency
&
Addison's disease
Presented by
[Link] Biswas Dipto
FCPS Part-2 Trainee,MU-3
Khulna Medical College Hospital
What is adrenal Insufficiency?
Adrenal Insufficiency results from inadequate Secretion of cortisol and / or
aldesterone
Adrenal hormones:
Causes of adrenal insufficiency
● Primary cause.(ACTH excess)
● Secondary cause.(ACTH deficiency)
The most common cause is secondary Cause (ACTH deficiency)because of-
● Withdrawal of suppressive glucocorticoid therapy
● Hypothalamic or pituitary tumour
Congenital adrenal hyperplasia and Addison's disease are rare causes.
Clinical Features-
Primary vs Secondary adrenocortical insufficiency:
Addison's disease
● It is the primary adrenocortical insufficiency
resulting in glucocorticoid and mineralocorticoid
insufficiency
● there is destruction of adrenal cortex
● There is excess ACTH but Low Cortisol
Causes of Addison's disease
Most common causes are
1. Autoimmune mechanism
2. Tuberculosis of adrenal gland
3. Secondary deposit in adrenals
4. HIV infection
5. Bilateral adrenalectomy
Other causes are (less Common or rare):
● Amyloidosis
● Sarcoidosis
● Hemochromatosis
● Histoplasmosis
● Bilateral Adrenal hemorrhage following meningococcal septicemia
● Lymphoma
Diagnostic Criteria in Addison's disease
Triad of -
● Weakness or emaciation(100% cases)
● Pigmentation(90% cases)
● Hypotension (88% cases)
Other features-
● Gastroenteritis (56% )
● Postural symptoms (12%)
● Salt Cravings(19%)
Fig: Emaciation Fig:Pigmentation of knuckles and creases
Investigation
● CBC:
1. High Eosinophil and lymphocyte
2. High ESR
● Blood Glucose:Low or lower Limit
● Electrolytes:
■ Hyponatremia
■ Hyperkalemia
● Test to Confirm:
■ Serum cortisol & ACTH
■ Short Synacthen Test
■ Long Synacthen test
● Tests to find out causes:
■ x Chest X-ray (to diagnose tuberculosis)
■ Plain X-ray abdomen (to see adrenal calcification in TB)
■ Adrenal auto-antibody
■ USG or CT scan of adrenals (to look for calcification in TB or malignancy).
● Other tests:
■ Screening for pernicious anemia and other autoimmune disorders
■ Thyroid screening
■ Other tests according to suspicion of cause (e.g. sarcoidosis, amyloidosis,
hemochromatosis, HIV, histoplasmosis, metastatic carcinoma, etc)
Disease Association of Addison's disease
● Graves disease
● Hashimoto's thyroiditis
● Pernicious Anemia
● Primary Ovarian failure
● Vitiligo
● Type 1 DM
Treatment of Addison's Disease
1. Replacement of hormones:
❏ Glucocorticoid:
• Hydrocortisone—15 mg in morning (after waking) and 5 mg in afternoon (6pm). According
to some authority, 10 mg after waking, 5 mg at 12 noon and 5 mg at 6 pm .
• Or, if hydrocortisone is not available, prednisolone 5 mg on waking in morning and 2.5 mg
at 6 pm in afternoon.
❏ Mineralocorticoid—Fludrocortisone 0.05 to 0.1 mg (50 to 300 µg) daily.
❏ Androgen—Dehydroepiandrosterone (DHEA) 50 mg/day may be given in female. It increases libido and
sense of well-being, but complications like acne and hirsutism may occur.
2. Treatment of the cause, as for example, antitubercular therapy in tuberculosis.
General Advice to the Patient
1. The patient should always carry a bracelet and steroid card, which should contain
informations
regarding the diagnosis, dose of steroid and doctor’s contact address.
2. Good nutrition, regular meal, high carbohydrate and sufficient salt.
3. The patient should keep ampules of hydrocortisone at home. If oral therapy is
impossible, the patient
should take injection by himself, family members or GP.
4. The patient should know how to increase steroid replacement dose for intercurrent
illness. During
intercurrent stress (fever, cold and trauma), the dose should be doubled
Monitoring Of the Patient & what to do in Stress period
Monitoring of the patient:
● Proper history regarding overall well-being
● Measurement of BP and weight
● Serum electrolyte
Remember the following points during stress -
● Intercurrent stress (fever, cold and trauma)—double dose of steroid.
● During surgery:
● Minor surgery—hydrocortisone 100 mg IM or IV premedication.
● Major surgery—hydrocortisone 100 mg IM or IV 6 hourly for 24 hours, then 50 mg 6
hourly. It should be continued until the patient is capable of taking by mouth.
● If gastroenteritis—IV or IM hydrocortisone should replace oral therapy
What Is Addisonian Crisis
● It is an acute severe adrenocortical insufficiency, characterized by circulatory shock
with severe hypotension.
● It is often precipitated by intercurrent disease, surgery or infection
● Clinical Presentation:
○ Acute abdomen
○ Nausea & Vomiting
○ Diarrhea
○ Unconsciousness
○ Collapse
○ Unexplained Fever
● Lab Findings:
○ Hyponatremia
○ Hyperkalemia
○ Hypoglycemia
○ Hypercalcemia
Causes of addisonian Crisis
● Sudden withdrawal of steroid (common cause, if the patient on steroid
for long time)
● Stress (severe infection and operation)
● Bilateral adrenal hemorrhage (meningococcal septicemia, injury and
anticoagulant)
● Thyroxine therapy in a patient with hypopituitarism without steroid
therapy.
Treatment of Addisonian crisis
● Blood is taken to measure cortisol, glucose and electrolytes
● Three problems are present—shortage of salt, sugar and steroid (3S)
● IV fluid: normal saline rapidly (1 L in 30 to 60 minutes). Subsequently, several liters of normal saline
may be required in 24 hours
● IV 10% glucose
● IV hydrocortisone 100 mg stat. Then hydrocortisone 100 mg IV or IM 6 hourly, which is continued until the patient is
stable and can take by mouth. Then oral steroid is started. Initially, hydrocortisone 20 mg 8 hourly, reducing to 20 to
30 mg in divided doses over a few days (then original replacement therapy should be given)
● Treatment of underlying cause (e.g. infection, adrenal or pituitary pathology, etc)
● In severe hyponatremia (<125 mmol/L), hypertonic saline is unnecessary, plasma Na should not be
increased >10 mmol/L/day. This may cause central pontine myelinolysis (osmotic demyelination
syndrome).
● During crisis or acute illness, mineralocorticoid such as fludrocortisone is unnecessary, as high dose
of steroid provides sufficient mineralocorticoid activity. It can be started later on.
● For hyperkalemia, volume replacement is sufficient. No extra treatment is usually necessary, but
occasionally requires specific therapy
● Morphine is avoided in in abdominal pain of addisonian crisis as patient is sensitive to this drug
