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• Credits

  • Section Writer: Dr. Om J Lakhani
  • Section Editor: Dr. Om J Lakhani

• Q. What are the features of glucocorticoid deficiency?

  • Gastrointestinal symptoms- nausea and vomiting
  • Fasting hypoglycemia
  • Fatigue and weakness
  • Failure to thrive in an infant
  • Morning headache
  • Reduce free water clearance

• Q. What are the causes of Mineralocorticoid deficiency?

  • Hypotension, syncope, and dizziness
  • Dehydration
  • Salt craving
  • Muscle weakness
  • Weight loss
  • Gastrointestinal (GI) symptoms- nausea and vomiting
  • Electrolyte imbalance

• #Clinicalpearl

  • GI symptoms and weight loss (or failure to thrive in infant) are because of both Glucocorticoid and Mineralocorticoid

• Q. What are the features of Adrenal androgen deficiency?

  • Males and prepubertal children- No symptoms
  • Post pubertal females- absence of axillary and pubic hair, no libido

• Q. Hypoglycemia is a prominent feature of primary or secondary adrenal insufficiency?

  • It is a prominent feature of secondary adrenal insufficiency

• Q. Which hormone is responsible for the acute adrenal crisis- Mineralocorticoid or Glucocorticoid?

  • Mineralocorticoid are primarily responsible for the acute adrenal crisis
  • Hence acute adrenal problem can also occur in patients who are on glucocorticoid replacement due to mineralocorticoid insufficiency

• Q. Can patients with secondary adrenal insufficiency present with adrenal crisis ?

  • Generally is is less common because of normal mineralocorticoid
  • However, Glucocorticoid deficiency alone can lead to hypotension

• Q. How does isolated glucocorticoid deficiency produce hypotension?

  • Reduced action of PNMT enzyme- which converts norepinephrine to epinephrine
  • Reduce vascular response to angiotensin II and Norepinephrine
  • Reduced synthesis of angiotensinogen
  • Increase prostacyclin production

• Q. What is the feature of abdominal pain in PAI, and what is the cause?

  • It is generalized abdominal pain
  • The exact cause is not known
  • Possibly since it is because of autoimmune disease- it may be due to Serositis

• Q. What is the relation between fever and PAI?

  • Fever is exacerbated by hypocortisolemia

• Q. What are the features of Chronic Adrenal insufficiency?

  • Fatigue, Malaise and weakness
    • Fatigue that is worsened by exertion and improved with bed rest
    • Weakness that is generalized, not limited to particular muscle groups
  • Anorexia
  • Weight loss

• Q. How much weight loss is seen?

  • Weight loss is about 2-5 kg

• Q. What are the GI symptoms of Adrenal insufficiency ?

  • Nausea
  • Diarrhea alternating with constipation
  • Vomiting
  • Abdominal pain
    • Abdominal pain and vomiting may herald an adrenal crisis

• Q. What is the cause of the GI symptoms?

  • It is not known

• Q. Is postural hypotension seen in these patients?

  • Yes

• Q. Can a hypertensive patient have Primary adrenal insufficiency?

  • Unlikely
  • The presence of hypertension is a strong evidence against PAI

• Q. How common is hyponatremia, and how does it manifest?

  • Hyponatremia is very common, it is seen in 90% of cases
  • Salt craving is also expected and also increase the thirst for iced liquids

• Q. How common are hyperkalemia and metabolic acidosis in PAI?

  • Present in 60-65% of cases

• Q. Is hypercalcemia common ?

  • It is present but rare

• Q. Why is insulin sensitivity increased in PAI?

  • Loss of gluconeogenic effect of Cortisol
  • Loss of effect of epinephrine to increase blood sugar

• Q. Which patients tend to have hypoglycemia in Adrenal insufficiency?

  • It is mainly fasting Hypoglycemia
  • It is more common in secondary adrenal insufficiency
  • Also more common in infants and children
  • Also seen in patients with Type 1 diabetes

• Q. Which POMC product is a stronger stimulant of melanogenesis than MSH?

  • ACTH 1-17

• Q. Is the POMC system present in the skin?

  • Yes, it is present in keratinocytes
  • It produces MSH locally

• Q. What are the areas of hyperpigmentation in primary adrenal insufficiency?

  • Areas of sun exposure
    • Face
    • Neck
    • Back of hands
  • Areas exposed to friction
    • Straps for socks
    • Elbow
    • Knee
    • Knuckles
    • Waist (belt)
    • Bra straps
  • Normally pigmentated area
    • Areola
    • Axilla
    • Perineum
    • Umbilicus
    • Others
    • Palmar crease
    • Existing freckles may become darker, new freckles may appear
  • Oral
    • The inner surface of the tongue
    • Buccal mucosa- along line of dental occlusion
  • Hairs may become darker
  • Nails may show dark bands
  • Pigmentation often is patchy

• Q. What happens to the scars?

  • Scars acquired during PAI – remain hyperpigmented
  • Scars acquired before PAI- normal pigmentation
  • Scars acquired on PAI treatment- normal

• Q. Does the hyperpigmentation disappear with treatment?

  • Yes it does
  • In hairs and nails, it disappears slowly
  • Also, it doesn't disappear from scars are melanocytes are trapped into the fibrous connective tissue

• Q. Is sexual dysfunction seen in PAI?

  • Only In women
  • This is because of the loss of adrenal androgens
  • It does not occur in men who have androgens produced by testis as well

• Q. How common is amenorrhea?

  • Seen in 25% of cases
  • Note possibility of Premature ovarian insufficiency (POI) in these cases

• Q. What are the musculoskeletal symptoms?

  • Myalgia and arthralgia are common
  • So much so that they even develop contractures

• Q. What happens to the 'ear' in PAI?

  • Calcification of the auricular cartilage is seen in long-standing primary and secondary Adrenal insufficiency
  • It is mainly seen in men
  • It does not improve with treatment with Glucocorticoid

• Q. What are the psychiatric symptoms of Adrenal insufficiency?

  • Impairment of memory that can progress to confusion, delirium, and stupor
  • Depression manifested by apathy, poverty of thought, and lack of initiative
  • Psychosis, manifested by social withdrawal, irritability, negativism, poor judgment, agitation, hallucinations, paranoid delusions, and bizarre or catatonic posturing

• Q. Can PAI lead to vitiligo?

  • Vitiligo may be often associated with PAI
  • In autoimmune Adrenal insufficiency – vitiligo may be one of the autoimmune disease associated
  • Autoimmune destruction of dermal melanocytes may lead to de novo vitiligo in patients with PAI

• Q. Which other findings would you look for?

  • Splenomegaly is present
  • Enlargement of tonsils – often seen

• Q. What is the importance of eosinophilia in PAI?

  • Thorn et al described relative eosinophilia as a feature of PAI
  • However, eosinophils  > 500 /um3 were seen only in < 20% of patients with PAI

• Q. Why is hypoglycemia more common in secondary and tertiary adrenal insufficiency?

  • Probably because of lack of dehydration and Mineralocorticoid deficiency, the patient can tolerate the disease longer
  • hence they come with hypoglycemia, whereas patients with PAI come with Mineralocorticoid deficiency features
  • It was earlier believed it is because of Growth hormone deficiency associated- however it is also seen in isolated ACTH deficiencies also

• Q. Are GI symptoms common in secondary/tertiary adrenal insufficiency?

  • No
  • They are uncommon

• Q. Which other conditions lead to hyperpigmentation similar to that seen in PAI?

  • Antimalarial use
  • Antineoplastic agents
  • Zidovudine
  • Hemochromatosis
  • Tetracycline
  • Phenothiazine

• Q. How will you differentiate PAI from hemochromatosis based on pigmentation?

  • PAI has the pigmentation of oral mucosa while hemochromatosis does not

• #Clinicalpearl

  • Bilateral Adrenal enlargement incidentally detected may be a clue to Adrenal insufficiency

• Q. Which CYP metabolizes Cortisol?

  • CYP3A4

• Q. Which addictive substances produce Adrenal insufficiency?

  • Opioids

#Updates

• Date: Sunday, 18 April 2021
• Source: YouTube video: Adrenal Medicine: Addison's Disease (Screening, Diagnosis & Management Dr. Sharvil Gadve followed by Panel discussion with experts)

• Date: 09-Aug-2023
• Source: Basics of Adrenal insufficiency - By Dr. Om J Lakhani

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