Understanding Addison’s Disease: Symptoms, Diagnosis, Treatment, and More
Explore the causes, symptoms, diagnosis, treatment, and prevention of Addison's disease in this comprehensive patient guide.
Understanding Addison’s Disease (Underactive Adrenal Glands)
Addison’s disease, a form of adrenal insufficiency, occurs when the adrenal glands do not produce sufficient hormones, particularly cortisol and aldosterone. This rare endocrine disorder can cause a range of symptoms and, if untreated, may become life-threatening. Early recognition, diagnosis, and appropriate management are vital to maintaining optimal health and preventing complications.
What is Addison’s Disease?
Addison’s disease, sometimes called primary adrenal insufficiency, is a rare but serious condition. It results from the gradual destruction or malfunction of the adrenal cortex—the outer layer of the adrenal glands—leading to low production of essential steroid hormones:
- Cortisol – a hormone that helps the body respond to stress, maintain blood pressure, regulate blood sugar, and more.
- Aldosterone – helps control blood pressure by regulating the balance of sodium and potassium.
- Adrenal androgens – minor sex hormones, especially important in females.
Without enough of these hormones, key body processes are disrupted. Addison’s disease can affect anyone regardless of age or gender, though it is most common in adults between ages 30 and 50.
How Do Adrenal Glands Work?
The adrenal glands are two small organs situated above each kidney. Each gland has two major parts with different functions:
- Adrenal cortex: Produces corticosteroid hormones (such as cortisol and aldosterone).
- Adrenal medulla: Produces adrenaline and noradrenaline (fight-or-flight hormones).
Cortisol is crucial for maintaining blood sugar, blood pressure, and helping the body respond to stress, while aldosterone helps balance salt and water, affecting blood pressure and heart function.
Causes of Addison’s Disease
Addison’s disease results when the adrenal cortex is damaged or cannot function properly. The main causes include:
1. Autoimmune Destruction
Most cases in developed countries are due to the immune system mistakenly attacking the adrenal cortex. This is called autoimmune adrenalitis. When damage is significant, hormone production drops.
2. Infections
In some parts of the world, infections such as tuberculosis, fungal infections, or HIV/AIDS can damage the adrenal glands leading to adrenal insufficiency.
3. Other Causes
- Cancer and tumors – cancers that metastasize to the adrenals or primary adrenal tumors.
- Adrenal hemorrhage – bleeding into the glands, often due to sepsis or certain medications.
- Genetic defects and rare diseases – such as adrenoleukodystrophy in children.
- Medication-induced – certain blood thinners or drugs that suppress adrenal function.
Types of Adrenal Insufficiency
| Type | Description | Main Cause |
|---|---|---|
| Primary | Damage or disease of the adrenal glands themselves (Addison’s disease) | Autoimmune, infection, cancer, hemorrhage |
| Secondary | Problems with the pituitary gland, which sends ACTH to adrenal glands | Pituitary tumors/injury, sudden stopping corticosteroids |
| Tertiary | Malfunction of the hypothalamus, which signals the pituitary | Sudden steroid withdrawal, brain disorders |
Symptoms of Addison’s Disease
Symptoms often develop slowly and may be vague or nonspecific, making early diagnosis challenging. Common symptoms include:
- Chronic fatigue and muscle weakness
- Unintentional weight loss, decreased appetite
- Nausea, vomiting, diarrhea
- Dizziness or fainting (especially when standing up)
- Low blood pressure (hypotension)
- Salt cravings
- Increased skin pigmentation (darkening) in scars, skin creases, gums
- Low blood sugar (hypoglycemia), especially in children
- Mood changes: irritability, depression
- Menstrual changes in women; decreased libido
If left untreated or undiagnosed, symptoms may worsen and lead to an Addisonian crisis—a life-threatening emergency requiring immediate treatment.
What is an Addisonian Crisis?
An Addisonian crisis (acute adrenal crisis) is a sudden, severe episode caused by extremely low cortisol. This may occur due to illness, injury, stress, or if medications are missed or the condition goes undiagnosed. Symptoms include:
- Severe vomiting and diarrhea
- Sudden, severe pain in abdomen, lower back, or legs
- Profound weakness and confusion
- Very low blood pressure (shock)
- Loss of consciousness
- Severe dehydration
This crisis can be fatal if untreated. Immediate medical attention and hospital care are vital. Treatment includes intravenous steroids, fluids, and managing any underlying triggers.
Diagnosis of Addison’s Disease
Early diagnosis is crucial but can be difficult due to the non-specific nature of the symptoms. Diagnosis is made based on a combination of clinical suspicion, laboratory tests, and imaging.
Key Diagnostic Steps
- Medical history and physical exam – to identify characteristic symptoms and physical findings (such as skin pigmentation).
- Blood tests – to measure levels of cortisol, aldosterone, ACTH (adrenocorticotropic hormone), sodium, potassium, blood sugar, and renin.
- ACTH stimulation test – assesses how the adrenal glands respond to synthetic ACTH. Low or absent cortisol response suggests adrenal insufficiency.
- Imaging – CT or MRI scans of the adrenal and pituitary glands in select cases to identify the cause (e.g., infection, tumor, bleeding).
- In some scenarios, tests for adrenal antibodies or additional autoimmune diseases may be recommended.
Treatment of Addison’s Disease
Treatment aims to replace missing hormones for life. Most people need two types of replacement therapy:
- Glucocorticoid replacement – Hydrocortisone, prednisone, or dexamethasone to replace cortisol.
- Mineralocorticoid replacement – Fludrocortisone to replace aldosterone, helping maintain salt and water balance.
Key Considerations in Treatment
- Medication doses may need adjustment during illnesses, surgeries, or periods of physical or emotional stress.
- Patients may require extra salt in their diet, especially during hot weather, exercise, or gastrointestinal illness.
- Dehydroepiandrosterone (DHEA) may be prescribed in some cases, particularly women, to improve mood and well-being.
Managing an Addisonian Crisis
An Addisonian crisis must be treated immediately as a medical emergency. Treatment includes:
- Intravenous glucocorticoids (such as hydrocortisone)
- Rapid infusion of saline (salt solution) and glucose to restore blood pressure and electrolytes
- Treating the underlying trigger (infection, injury, etc.)
All patients with Addison’s disease should be aware of crisis symptoms and carry emergency identification and injectable steroids.
Living With Addison’s Disease
Most people with Addison’s disease can lead full, active lives with proper treatment, education, and self-care. Essential tips include:
- Take medications exactly as prescribed and do not skip doses.
- Always carry a medical alert bracelet or card indicating adrenal insufficiency and the need for emergency steroids.
- Keep an emergency injection kit of corticosteroid for crises.
- Maintain a supply of extra medication at work or when traveling.
- Consult your healthcare professional about adjusting medication during illness, injury, surgery, or emotional stress.
- Carry a written emergency plan and ensure close family and friends know how to respond to a crisis.
- Have regular follow-up with your healthcare team, including periodic laboratory checks.
Potential Complications and Related Conditions
Patients with Addison’s disease may be at increased risk for other autoimmune conditions, such as:
- Thyroid disorders (hypothyroidism or hyperthyroidism)
- Type 1 diabetes
- Pernicious anemia
- Vitiligo
- Other hormonal imbalances
Regular check-ups and blood tests can help detect these complications early. Prompt management and coordination with specialists may be advised.
Prevention and Prognosis
There is no known way to prevent primary Addison’s disease, but early diagnosis and proper hormone replacement therapy can prevent a crisis and complications. With lifelong, tailored treatment, most individuals have a normal life expectancy and good quality of life.
Patients on long-term steroid therapy for other health conditions should work with their healthcare team to taper medications safely and avoid triggering secondary or tertiary adrenal insufficiency.
Frequently Asked Questions (FAQs)
What triggers an Addisonian crisis?
Triggers include severe emotional or physical stress, infections, injuries, surgery, or missing medications. Always carry an emergency steroid kit and identify yourself as having adrenal insufficiency.
Can Addison’s disease be cured?
Addison’s disease cannot currently be cured, but it is highly manageable with lifelong hormone replacement therapy.
What are the long-term effects of Addison’s disease?
With proper management, most people can expect to live a normal lifespan. Unmanaged disease or repeated crises can cause complications related to electrolyte imbalances or shock.
Do I have to adjust my medications for exercise or illness?
Yes. During physical or emotional stress, illness, or injury, your medication dose may need to be increased. Always consult your healthcare provider for a personalized plan.
Are there dietary considerations for Addison’s disease?
You may need a higher salt intake, especially in hot weather or during heavy exercise. Discuss with your healthcare provider whether dietary changes are needed for your specific case.
How can I help a loved one with Addison’s disease?
- Understand the basics of the disease and its treatment.
- Learn to recognize signs of an Addisonian crisis and how to use an emergency injection kit.
- Remind them to wear a medical alert bracelet and keep medications nearby.
Summary
Addison’s disease is a rare but serious endocrine disorder requiring lifelong hormone therapy and regular medical care. By recognizing symptoms early, adhering to medication, being prepared for emergencies, and fostering a supportive environment, most people with Addison’s disease can live healthy, fulfilling lives. Collaborate closely with your healthcare team for tailored advice and updates on treatment advances.
References
- https://www.healthline.com/health/addisons-disease
- https://www.aafp.org/pubs/afp/issues/2014/0401/p563.html
- https://www.mayoclinic.org/diseases-conditions/addisons-disease/diagnosis-treatment/drc-20350296
- https://www.ncbi.nlm.nih.gov/books/NBK441994/
- https://rarediseases.org/rare-diseases/addisons-disease/
- https://my.clevelandclinic.org/health/diseases/15095-addisons-disease
- https://www.nhs.uk/conditions/addisons-disease/
- https://www.health.harvard.edu/diseases-and-conditions/addisons-disease-overview-of-an-uncommon-but-serious-condition
Similar Articles
Read full bio of Sneha Tete
