3. ADRENAL GLAND
• FUNCTION :
The adrenal glands are composed of two functionally
distinct endocrine units, the adrenal cortex and medulla,
contained within a single capsule.
4. ADRENAL GLAND
• Adrenal cortex function
three functional zones
• Zona glomerulosa
secretes
mineralocorticoids
(aldosterone) which
regulate sodium and
potassium homeostasis.
• Zona fasciculata secretes
glucocorticoids (most
importantly, cortisol).
• Zona reticularis secretes
sex steroids (primarily
androgens).
5. ADRENAL GLAND
• Adrenal medulla function :
The adrenal medulla synthesizes and secretes
catecholamines, which modulate the body's
sympathetic response to stress.
11. PRIMARY ADRENAL INSUFFICIENCY
AUTOIMMUNE ADRENALITIS
1. AUTOIMMUNE ADRENALITIS
• Autoimmune process that destroys the adrenal
cortex .
• Associated with autoimmune destruction of other
endocrine glands.
• Antibodies that react with several steroidogenic
enzymes (most often 21-hydroxylase).
12. PRIMARY ADRENAL INSUFFICIENCY
AUTOIMMUNE ADRENALITIS
• Early findings The first evidence is increase in
plasma renin activity .
• Normal or low serum aldosterone? If low suggesting
zona glomerulosa is involved.
• Months to years later, zona fasciculata dysfunction
becomes evident, by:
1. Decreasing serum cortisol in response to (ACTH).
2. Increased basal serum ACTH concentrations.
3. Finally by decreasing basal serum cortisol
concentrations and symptoms .
13. PRIMARY ADRENAL INSUFFICIENCY
AUTOIMMUNE ADRENALITIS
• Approximately 65 % of patients with autoimmune
adrenal insufficiency have one or more other
autoimmune endocrine disorders such as type 1 DM,
chronic autoimmune thyroiditis ,Graves' disease.
14. PRIMARY ADRENAL INSUFFICIENCY
AUTOIMMUNE ADRENALITIS
1. Polyglandular autoimmune syndrome type 1.
Polyendocrinopathy-candidiasis-ectodermal
dystrophy (APECED) syndrome.
Hypoparathyroidism or chronic mucocutaneous
candidiasis is first manifestation (candidiasis
mouth).
Adrenal insufficiency usually develops later, at
age 10 to 15 years.
16. PRIMARY ADRENAL INSUFFICIENCY
2. Polyglandular autoimmune syndrome type 2
Much more common than type 1 and primary
adrenal insufficiency is its principal
manifestation .
Chronic autoimmune thyroiditis & type 1 DM
is common
with most cases occurring between age 20
and 40 years
18. PRIMARY ADRENAL INSUFFICIENCY
INFECTIOUS ADRENALITIS
2. INFECTIOUS ADRENALITIS
Tuberculosis :
• Tuberculous adrenalitis results from
hematogenous spread from active infection.
• No Antiadrenal autoantibodies
• Adrenal calcifications 50 % .
• Recovery of normal adrenal function ??
Usually not
24. RECOMMENDATIONS
1. Acutely ill patients with S & S suggestive of PAI
volume depletion, , BP, NA, k ,
fever, abdominal pain, hyperpigmentation.
2. (Recommend) confirmatory testing with the
corticotropin stimulation test in patients with clinical S
& S of PAI when patient’s circumstance allow.
25. RECOMMENDATIONS
3. Patients with severe adrenal insufficiency or adrenal
crisis, we recommend immediate therapy with iv
hydrocortisone with stress dose prior results of diagnostic
tests.
27. RECOMMENDATIONS
1. (Recommend) standard dose 250ug for
adults iv corticotropin stimulation (30 or
60min), Peak cortisol levels below 500 nmol/L
(18 g/dL) at 30 or 60 minutes indicate
adrenal insufficiency.
2. If a corticotropin stimulation test is not
available we suggest using a morning cortisol,
140 nmol/L (5 g/dL) in combination with
ACTH as a initial test suggestive of adrenal
insufficiency .
28. RECOMMENDATIONS
3. (Recommend) measurement of plasma ACTH to
establish PAI. In patients with confirmed cortisol
deficiency.
Plasma ACTH 2 fold the upper limit of the reference
range is consistent with PAI.
4. (Recommend) Measurement of plasma renin and
aldosterone in PAI to determine the presence of
mineralocorticoid deficiency.
30. RECOMMENDATIONS
1. Glucocorticoid replacement regimen
• (Recommend) glucocorticoid therapy in all
patients with confirmed PAI.
• (Suggest) using hydrocortisone (15–25 mg) or
cortisone acetate (20 –35 mg) in two or three
divided oral
• Alternative to hydrocortisone, we (suggest)
using prednisolone (3–5 mg/d)
31. RECOMMENDATIONS
• (Suggest) against using dexamethasone for the
treatment of PAI because of risk of Cushingoid side
effects & due to difficulties in dose titration
• (Suggest) against hormonal monitoring of
glucocorticoid replacement and to adjust treatment
only based on clinical response
32. RECOMMENDATIONS
2. Mineralocorticoid replacement in PAI
• (Recommend) that all patients with confirmed
aldosterone deficiency receive mineralocorticoid
replacement fludrocortisone (starting dose, 50 –100
ug in adults).
• Monitoring mineralocorticoid replacement primarily
based on clinical assessment ( postural hypotension,
or edema), & blood electrolyte measurements.
33. RECOMMENDATIONS
3. Dehydroepiandrosterone replacement.
• (Suggest) a trial of Dehydroepiandrosterone (DHEA)
replacement in women with PAI and low libido,
depressive symptoms or low energy levels despite
otherwise optimized glucocorticoid and
mineralocorticoid replacement.
35. RECOMMENDATION
1. (Recommend) that patient with adrenal crisis should
be treated with an immediate parenteral injection of
100 mg hydrocortisone followed by appropriate
fluid resuscitation & 200 mg of hydrocortisone /24
hours (continuous iv or every 6 hours injection)
2. If hydrocortisone is unavailable, we (suggest) pred-
nisolone as an alternative. Dexamethasone is the
least- preferred alternative and should only be given
if no other glucocorticoid is available
36. RECOMMENDATION
3. For the prevention of adrenal crisis, we (suggest) ad-
justing glucocorticoid dose according to severity of
illness or magnitude of the stressor.
4. Home management of illness with fever if 38 > double the
dose , if 39> triple the dose.
5. Minor to moderate surgical stress we will give
hydrocortisone 25-75mg/24h (1-2 days).
6. Major surgery like adrenal crisis.
7. We (recommend) that every patient should be equipped
with a glucocorticoid injection kit for emergency use and
be educated on how to use it.
37. CASE SCENARIO
• A 32 – years old man comes to the office after a year
of weakness, fatigue , and weight loss. He has
experienced reduced appetite and intermittent
diarrhea. The patient hand no improvement after
several sessions of clinical psychologist, who
suggested evaluation for a physiological cause of his
symptoms . His medical history unremarkable and he
takes no medications , the patient does not use
tobacco , alcohol or illicit drugs . Family history is
notable for hypothyroidism (his sister).
• Temp is 37.2 ,blood pressure is 106/66 mm hg , pulse
94/min , & respirations are 14/min .
38. CASE SCENARIO
On physical examination the patient does not appear to
be in acute distress . His neck show no thyromegaly or
lymphadenopathy. Cardiopulmonary examination is
normal , abdomen is soft with normal bowel sound .
Motor strength and deep tendon reflexes are normal
and symmetrical
• LABS :
- Hemoglobin 12.3 g/dl
- WBC 3000
- serum sodium 130 mEq/L
-serum potassium 5.5 mEq/l
- 8 AM cortisol 7.2 ug/dl(normal 5-23)
- TSH 2.5 mLU/L
39. CASE SCENARIO
Q - Which of the following is the most appropriate next step
in management of this patient ?
A- 24-hours urine free cortisol.
B-ACTH stimulation test.
C- Insulin induced hypoglycemia test .
D-Intravenous hydrocortisone .
E – Low-dose overnight dexamethasone suppression test.
40. CASE SCENARIO
• Answer is
• B- ACTH stimulation test.
Explanation :
The initial test evaluation should include
1- 8 AM cortisol level
2- Plasma ACTH level
However , the ACTH assay can take several days so
ACTH stimulation test is usually preformed to rapidly
confirm the dignosis
41. SPOT DIAGNOSIS
• 44 years old female from India comes with
fever , hemoptysis, weight loss 10 kg , for 3
weeks. Recently she started to complain of
increasing weakness, fatigue.
42.
43. SPOT DIGNOSIS
• Bilateral adrenal enlargement & calcification.
• INFECTIOUS ADRENALITIS most likely due active
TB infection.
