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

Date: 09-Aug-2023

• Source: Basics of Adrenal insufficiency - By Dr. Om J Lakhani

• Q. Which is the most common cause of Primary adrenal insufficiency currently?

  • Autoimmune

• Q. How common is tuberculosis as a cause of adrenal insufficiency?

  • Tuberculosis cause AI in 7-20% of cases

• **AUTOIMMUNE ADRENALITIS **

• Q. What are the sex differences in autoimmune adrenal insufficiency?

• Polyglandular syndrome occurs mainly female

• Isolated PAI

  • 1st 2 decades- M > F
  • 20-30 years- M = F
  • More than 30 year- F  > M

• Q. How common are 21-hydroxylase antibodies in autoimmune adrenalitis?

  • 86% of cases

• Q. What is the sequence of events that occur in autoimmune adrenalitis?

  • 1st increase in PRA and reduction in aldosterone  - Zona fasciculata damage
  • 2nd – reduction in cortisol response to ACTH
  • 3rd – Increase of ACTH
  • 4th – reduction of Cortisol and symptoms

• Q. Which test is the best predictor of adrenal insufficiency developing in patients having positive 21 hydroxylase antibodies?

  • ACTH is the best predictor of the development of AI

• Q. Which is the most common autoimmune disease associated with autoimmune adrenalitis ?

  • Ovarian failure, according to one study

• Q. Should all type 1 diabetic be screened for Adrenal insufficiency?

  • No
  • However, the risk of adrenal insufficiency is 1% in patients with type 1 diabetes

• POLYGLANDULAR AUTOIMMUNE SYNDROME

• Q. What are the features of APS I?

  • Adrenal insufficiency
  • Ectodermal dysplasia
  • Hypoparathyroidism
  • Mucocutaneous candidiasis

• Q. What is the gene defect in APS I?

  • AIRE gene
  • This is autoimmune recessive

• Q. Which is the 1st manifestation in APS-1?

  • Mucocutenous candidiasis

• Q. Which is the most common manifestation in APS-1?

  • Hypoparathyroidism

• Q.  Adrenal insufficiency occurs at what age?

  • 10-15 years of age

• Q. Which antibodies against adrenal steroidogenesis enzymes are present in APS-1?

  • 21 hydroxylase
  • 17 alpha-hydroxylase
  • Sidechain cleavage enzyme

• Q. Can hypogonadism occur in APS-1?

  • Yes
  • It is seen in 60% of the patients

• Q. What is the cause of malabsorption in APS-1?

  • Antibodies against tryptophan hydroxylase

• Q. Do type 1 diabetes and hypothyroidism occur in these conditions?

  • No
  • It is uncommon

• Q. What occurs 1st disease that occurs in APS 2- Adrenal insufficiency or Hypothyroidism/Diabetes mellitus?

  • 50% of cases PAI occurs first
  • Simultaneous detection – 30% of cases
  • Hypothyroidism /   Type 1 diabetes – 20% cases

• Q. Can hypoparathyroidism occur in APS-2?

  • No
  • It is not seen in APS-2

• Q. What about hypogonadism?

  • Hypogonadism can occur

• Q. What will you suspect if an APS-2 patient has low ACTH and other features of hypopituitarism?

  • Think of autoimmune hypophysitis

• Q. What antibodies cause vitiligo?

  • Antibody against tyrosinase enzyme and melanocyte-specific protein Pmel17

• **INFECTIOUS ADRENALITIS **

• Q. What kind of spread produces Tuberculous adrenalitis?

  • Hematogenous spread produces tuberculous adrenalitis

• Q. Which part of the adrenal is more damaged in tuberculous adrenalitis?

  • The medulla is destroyed more than the cortex

• Q. What happens to the size of adrenal glands in TB adrenalitis?

  • Initially increase in size
  • Then gradually reduces in size due to fibrosis

• Q. Can the adrenal function improve after AKT?

  • Usually no

• Q. How common is calcification seen in Tuberculous adrenalitis?

  • In 50% of cases

• Q. Can the adrenal size be enlarged in patients with tuberculosis even without have AI?

  • Yes
  • It increases in size before treatment and normalizes after treatment even without having adrenal insufficiency

• Q. Why do patients with tuberculosis of the adrenal gland worsen on treatment with AKT?

  • This is because of Rifampicin
  • This is a CYP3A4 inducer that increases the metabolism of Cortisol

• #Clinicalpearl

  • Patients with TB adrenalitis have active tuberculosis somewhere

• Q. Which fungal infections cause adrenalitis?

  • Histoplasmosis or Paracoccidomycosis (South American Blastomycosis)
  • It is rare in cryptococcosis, North American Blastomycosis, and coccidomycosis

• **ADRENAL HEMORRHAGE AND INFARCTION **

• Q. Which organisms are responsible for Waterhouse-Fredrickson syndrome?

  • Neisseria meningitiditis
  • Pseudomonas aeruginosa – most common organisms in one study
  • Neisseria gonorrhea
  • Streptococcus pneumonia
  • Staphylococcus aeruginosa

• Q. What is the cause of adrenal hemorrhage?

  • Anticoagulant therapy
  • Thromboembolic disease
  • APLA syndrome
  • Physical trauma
  • Sepsis
  • Postoperative state

• **METASTATIC DISEASE **

• Q. Why is metastasis to the adrenal gland common?

  • This is because it has a rich sinusoidal blood supply

• Q. Which primary malignancy of the adrenal gives a similar picture at metastasis?

  • Lymphoma

• Q. Which cancers are associated with adrenal metastasis?

  • Lung
  • Breast
  • Melanoma
  • Colon cancer
  • Stomach cancer

• Q. Are all metastasis to adrenals symptomatic?

  • No
  • Symptoms may not occur in all cases of adrenal metastasis
  • This is because the adrenal cortex needs to be destroyed significantly before symptoms occur

• **DRUG INDUCED PAI **

• Q. Which drugs block the steroidogenesis pathway leading to AI?

  • Etomidate
  • Ketoconazole
  • Fluconazole
  • Suramin
  • Metyrapone

• Q. Do all patients who receive these medications develop AI?

  • No
  • The inhibition of enzymes is partial
  • The Increase in ACTH override this partial enzyme inhibition
  • Hence patients with reduced pituitary or adrenal axis reserve develop AI

• Q. Which drugs cause AI by increasing the metabolism of Cortisol?

  • Baribiturtes
  • carbamazapine
  • Rifampicin
  • anticonvulsants
  • Phenytoin
  • Again they cause AI in patients who have limited reserves rather than in patients with adequate reserves

• Q. Which drug destroys the adrenal cortex leading to AI?

  • Mitotane

• Q. Can heparin cause AI?

  • Not directly
  • However, heparin-induced thrombocytopenia is associated with bilateral adrenal hemorrhage

#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)

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