Addison’s disease, also known as primary adrenal insufficiency, is a condition that affects the adrenal glands of the endocrine system involved in the production of hormones.^1,3 In Addison’s disease, the adrenal glands do not produce any, or enough, cortisol and aldosterone hormones that are important for regulating body processes such as metabolism and blood pressure.⁴ This is most commonly due to an autoimmune condition, in which the body is attacking its own adrenal glands; however, there can be other causes resulting in the adrenal glands not functioning properly. Addison’s disease differs from secondary adrenal insufficiency (where it is the pituitary gland that is directly affected, causing the adrenal gland not to release cortisol).¹

Symptoms of Addison’s disease may include, but are not limited to, fatigue, weakness, nausea, loss of appetite, abdominal pain, salt cravings and darkening of the skin or mucous membranes.^1,3 Symptoms often develop slowly over time³ and are often mistaken for more common conditions.⁵ A life-threatening complication of Addison’s disease is acute adrenal failure, known as adrenal crisis or Addisonian crisis, and requires immediate medical treatment.^1,2 Adrenal crisis can be brought upon by factors (such as stress, dehydration, trauma or infection) that affects cortisol levels. It can also occur if an individual with adrenal insufficiency does not receive appropriate treatment or misses a dose of their medication.^1,2

Synonyms: Addison disease, autoimmune Addison disease; autoimmune adrenalitis; classic Addison disease; primary Addison disease^3,5

Universal rare disease classifications provide a common language for recording, reporting and monitoring diseases. Please visit the Rare Disease Classifications page for more information about these internationally recognised classifications.

ORPHA:85138 Addison disease

ICD-11: 5A74.0 Acquired adrenocortical insufficiency

Symptoms of Addison’s disease may include:^1,2

• loss of appetite, nausea, vomiting, diarrhoea, weight loss, abdominal pain
• fatigue, weakness, headache, dizziness (especially on standing)
• difficulty concentrating, mood swings, depression
• salt cravings and darkening of skin and mucous membranes
• irregular menstruation in women
• low blood pressure

Adrenal crisis can be a life-threatening complication of Addison’s disease and is a medical emergency (requires immediate medical attention).^1-2 Symptoms may include, but are not limited to, altered level of consciousness and confusion, seizures, low blood pressure (hypotension), as well as symptoms indicative of low blood sugar (hypoglycaemia), low blood sodium levels (hyponatraemia) or high potassium levels in the blood (hyperkalaemia), in addition to the other symptoms of Addison’s disease.²

Untreated chronic Addison’s disease can lead to symptoms such as memory impairment, confusion, apathy, depression, psychosis and even death.^1,2

Please speak to your medical team to learn more about the symptoms and complications of Addison’s disease.

Rare diseases are often serious and progressive, exhibiting a high degree of symptom complexity, leading to significant disability. Majority of the estimated two million Australians living with a rare disease meet the Australian Government’s definition for disability (in accordance to the Australian Public Service Commission and Australian Bureau of Statistics), and many experience severe and permanent disability impacts. If you or someone you care for is experiencing disability-related impacts from a rare condition, please speak with a health or disability professional for advice. Information about relevant disability support can be found at the RARE Portal’s Disability Support Information page.

Addison’s disease occurs when the adrenal glands stop producing the hormones, cortisol and aldosterone (primary adrenal insufficiency).³ This is most commonly due to an autoimmune condition, however there may be other causes in which the adrenal gland is affected, such as cancer, injury, infection or surgery. Addison’s disease could also be a feature of genetic conditions, such as congenital adrenal hyperplasia and adrenoleukodystrophy.¹

Diagnosis of Addison’s disease may be made based on medical history, physical examination and a blood test.² The blood test is usually performed in the early morning as cortisol levels decrease over the day. Individuals with Addison’s disease often have low cortisol, low aldosterone, high adrenocorticotropic hormone (ACTH), low blood sugar, low sodium, high potassium and high renin levels.^1,2

A corticotropin stimulation test (or Synacthen test), to identify low cortisol response to administration of synthetic ACTH, may also be used as a diagnostic tool for adrenal insufficiency.^2,6 It should only be performed under controlled circumstances in a hospital setting.²

Imaging of the adrenal glands may also be performed.³

A differential diagnosis can rule out other conditions that have similar symptoms, such as secondary adrenal insufficiency.⁵

Please speak to your medical team to learn more about the available diagnostic pathways for Addison’s disease.

Treatment of Addison’s disease involves long-term steroid replacement therapy, which is given orally as a tablet.^1,2  It is important for individuals with Addison’s disease to adhere to their therapy to reduce the possibility of adrenal crisis. Regular blood tests and specialist reviews are essential to optimise the required dose of steroids.^1,2  The required dose may change over time, particularly with growing children.²

In the case of adrenal crisis, medical treatment, such as an administration of intravenous (IV; injection into veins) hydrocortisone, should be given immediately as it is a medical emergency.^1,2

It is important for doctors to ensure that their patients have an up-to-date emergency plan and know how to recognise the signs of an adrenal crisis as well as what to do in an emergency.² It is recommended that individuals living with Addison’s disease should always have a ready-to-use and non-expired vial of emergency hydrocortisone. It is also advisable to wear a MedicAlert bracelet or pendant and have ambulance cover.³

Please speak to your medical team to learn more about the possible treatment or management options for your condition. Treatment will depend on an individual’s specific condition and symptoms. It is also important to stay connected to your medical team so that you can be made aware of any upcoming clinical trial opportunities.

Clinical care for rare diseases often involves a multidisciplinary team of medical, care and support professionals. Please note that the information provided here is as a guide and that RVA does not necessarily monitor or endorse specific clinics or health experts.

Healthcare professionals involved in the treatment of Addison’s disease may include general practitioners (GP), physicians, endocrinologists, and endocrine nurses. The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.³

The Endocrine Society of Australia – Search For An Endocrinologist has clinical endocrinologists (that are members of the Endocrine Society of Australia) listed by state, suburb and areas of interest.

Clinical Practice Guideline: Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline  was developed in 2015 by a task force which included an Australian-based specialist.

Addison disease – diagnosis and initial management² is a 2010 focus article in the Australian Family Physician, a peer-reviewed journal of The Royal Australian College of General Practitioners (RACGP).

Individuals living with rare diseases may have complex medical issues and disabilities, which are not always visible. It is often useful to refer to their medical history as well as personal information such as a medical card, doctor’s letter, or if available, a rare disease passport, for relevant information.

It may be important to consider the following when managing individuals living with Addison’s disease at emergency departments/services:

Individuals with Addison’s disease may experience an adrenal crisis, which is a medical emergency. It requires immediate medical attention and administration of hydrocortisone injection and potentially other treatment.^1,2 An endocrinologist should be consulted as soon as possible and admission to an intensive care unit may be required.² Symptoms may include, but are not limited to, sudden weakness, abdominal pain, vomiting, dizziness, confusion, low blood sugar and low blood pressure.¹ In addition, patients often have comorbidities that should be considered during emergency treatment of an adrenal crisis.

Information about ambulance protocols for adrenal insufficiency, provided by individual state and territory ambulance services, are listed at Australian Addison’s Disease Association Inc.: Ambulance Protocols.

For individuals with Addison’s disease, it is important to have an up-to-date emergency plan and knowledge of administering emergency medication.² It is recommended that individuals wear a MedicAlert bracelet or pendant and have ambulance cover. Below are resources that may be useful in preparing for, and to be used in, emergency situations:

• Endocrine Society of Australia: Adrenal Insufficiency Resources includes a sick day management plan and a steroid card for use in emergency situations.
• Australian Addison’s Disease Association Inc. also has resources to help individuals with Addison’s disease deal with the onset of an adrenal crisis.

There are specific considerations around participating in rare disease research, including clinical trials. It is important to be mindful of issues such as data privacy, research ethics, consent and differences in research regulations between Australia and other countries.

If you are interested in finding clinical trials for your condition, please visit the following websites; however, there may not be any clinical trials available:

• Australian Clinical Trials
• ClinicalTrials.gov

It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.

Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance and activities.

Australian Organisation:

Australian Addison’s Disease Association Inc.
Website: https://addisons.org.au

The goal of Australian Addison’s Disease Association Inc. is to provide valuable information and resources for those with Addison’s disease and adrenal insufficiency as well as their carers, medical practitioners and the wider community. The Association also offers a network of support for those medically diagnosed with Addison’s disease/adrenal insufficiency.

Please note that RVA does not monitor or endorse each group/organisation’s operational governance and activities. When engaging with a group, please consider the information on the RARE Portal’s Finding Helpful Peer and Community Supports page.

Addison’s disease varies between individuals, and each person’s experience is unique.   

Personal Story shared with Rare Voices Australia: April’s story.

If you would like to share your personal story with RVA, please visit the Rare Voices Australia: Share Your Story page. RVA will consider your story for publishing on our website and inclusion on the RARE Portal.

The Australian Addison’s Disease Association, Inc.: Fact Sheets has useful resources for people living with Addison’s disease.

For information on available government and social services that provide support for individuals with a rare disease, please visit the National and State Services pages.

People living with a rare disease, including families and carers, often face unique challenges such as diagnostic delays, misdiagnoses, limited treatment options, and limited access to rare disease specialists and support. These challenges may impact people’s emotional wellbeing and quality of life. Many people find it helpful to seek mental health and wellbeing support to cope with ongoing stress and uncertainty. Connecting with people who have shared experiences through a support group may also be helpful. Information about relevant mental health and wellbeing support can be found at:

• Mental Health and Wellbeing Support for Australians Living with a Rare Disease
• The National and State Services pages underneath the ‘Mental Health’ sections listed

Further information on Addison’s disease can be found at:

• Hormones Australia (run by Endocrine Society of Australia): Adrenal Insufficiency
• healthdirect: Addison’s disease
• Genetic and Rare Diseases (GARD) Information Center: Addison Disease
• National Organization for Rare Disorders (NORD): Addison’s Disease

• Australian Addison’s Disease Association Inc.: For Medical Professionals
• The Royal Australian College of General Practitioners (RACGP) – Australian Family Physician (AFP): Addison disease – diagnosis and initial management
• The Royal College of Pathologists of Australasia (RCPA): Adrenocortical insufficiency
• Orphanet: Addison disease
• Human Phenotype Ontology: Addison Disease

This page has been co-developed by Rare Voices Australia (RVA)’s RARE Portal team in consultation with Australian Addison’s Disease Association Inc.

If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.