Adrenal insufficiency pathophysiology

Adrenal insufficiency Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Xyz from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Interventions
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Adrenal insufficiency pathophysiology On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Adrenal insufficiency pathophysiology All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Adrenal insufficiency pathophysiology
CDC on Adrenal insufficiency pathophysiology
Adrenal insufficiency pathophysiology in the news
Blogs on Adrenal insufficiency pathophysiology
Directions to Hospitals Treating Psoriasis
Risk calculators and risk factors for Adrenal insufficiency pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

The exact pathogenesis of [disease name] is not fully understood.

OR

It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].

OR

[Pathogen name] is usually transmitted via the [transmission route] route to the human host.

OR

Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.

OR

[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].

OR

The progression to [disease name] usually involves the [molecular pathway].

OR

The pathophysiology of [disease/malignancy] depends on the histological subtype.

Pathophysiology

Physiology

Adrenal insufficiency arises due to the deficiency of adrenocortical hormones.The normal physiology of adrenocortical hormone production can be understood as follows: The adrenal cortex is divided into three zones from outside to inside as - Zona glomerulosa, zona fasciculata and zona reticularis which are responsible for the production of streroidogenic hormones like mineralocorticoid, glucocorticoid and androgens respectively.

Pathogenesis

The pathophysiology of adrenal insufficiency depends on the etiological subtype.

Autoimmune adrenalitis: Both humoral and cell-mediated immune mechanisms attack enzymes involved in adrenocortical hormone synthesis. Autoantibodies against 21-hydroxylase, an enzyme required in the biosynthesis of adrenocortical hormones can be found. The patients remain asymptomatic until 90% of the cortex is destroyed. It can present as isolated autoimmune adrenalitis in 30-40% of cases or as an autoimmune polyglandular syndrome in 60-70% of cases. The autoimmune polyglandular syndrome in subclassified as autoimmune polyglandular syndrome type 1(APS type 1) and autoimmune polyglandular syndrome type 2 (APS type 2)

Genetics

Autoimmune adrenalitis: Strong genetic association has been seen with HLA DR3/DQ2 and DR4/DQ8.

• The autoimmune polyglandular syndrome type 1 is transmitted in an autosomal recessive pattern. Gene involved in the pathogenesis is the autoimmune regulator gene (AIRE).
• The autoimmune polyglandular syndrome type 2 shows a polygenic inheritance. A strong association has been shown with HLA DR3 of MHC.

[Disease name] is transmited in [mode of genetic transmission] pattern.

OR

Genes involved in the pathogenesis of [disease name] include:

• [Gene1]
• [Gene2]
• [Gene3]

OR

The development of [disease name] is the result of multiple genetic mutations such as:

• [Mutation 1]
• [Mutation 2]
• [Mutation 3]

Associated Conditions

Autoimmune adrenalitis:

• Autoimmune polyglandular syndrome type 1 is also known as Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED). It is associated with chronic mucocutaneous candidiasis, hyperparathyroidism, total alopecia.

• Autoimmune polyglandular syndrome type 2 is associated with autoimmune thyroiditis, vitiligo, premature ovarian failure, type 1 diabetes mellitus, pernicious anemia.

Gross Pathology

On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Microscopic Pathology

On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].