Table of Contents
- What Is Autoimmune Addison’s Disease?
- Understanding the Autoimmune Process
- Signs and Symptoms
- Diagnosis and Testing
- Treatment and Management
- Living with Addison’s Disease
- Adrenal Crisis: What You Need to Know
- Associated Autoimmune Conditions
- Support and Resources in the UK
- Frequently Asked Questions
- Key Takeaways
📊 Addison’s Disease in the UK – Key Statistics
What Is Autoimmune Addison’s Disease?
Autoimmune Addison’s disease is a rare but serious condition where the body’s immune system mistakenly attacks and destroys the outer layer of the adrenal glands (adrenal cortex). This autoimmune process prevents the glands from producing sufficient amounts of vital hormones, particularly cortisol and aldosterone.
Key Facts About Addison’s Disease in the UK
- Prevalence: Affects approximately 1 in 10,000 people in the UK
- Annual diagnoses: Over 300 new cases diagnosed yearly
- Gender distribution: More common in women (ratio 1.8:1)
- Age of onset: Most commonly diagnosed between ages 30-50
- Autoimmune cause: Accounts for 68-94% of cases in developed countries
The condition is named after Dr Thomas Addison, who first described it in 1855. In the UK, autoimmune disease is the predominant cause of Addison’s disease, unlike developing countries where tuberculosis remains the leading cause.
Understanding the Autoimmune Process
How Autoimmunity Develops
In healthy individuals, the immune system protects against infections and foreign substances. However, in autoimmune Addison’s disease, the immune system produces antibodies that attack the cause of the illness. However, if you develop a problem with your immune system, it can start to attack your own healthy tissues and organs.
The 21-Hydroxylase Connection
The primary target of autoimmune attack is an enzyme called 21-hydroxylase (21OH). Presence of 21-hydroxylase autoantibodies (21OHAb) identifies subjects with ongoing clinical or pre-clinical adrenal autoimmunity.
Progression of Adrenal Damage
- Early stages: Minimal symptoms, only apparent during stress
- 90% destruction threshold: When 90% of the adrenal cortex is destroyed, your adrenal glands will not be able to produce enough of the steroid hormones cortisol and aldosterone
- Complete failure: Life-threatening hormone deficiency requiring immediate treatment
Genetic Predisposition
Research has shown that some people with certain genes are more likely to develop autoimmune disorders. Key genetic risk factors include:
- HLA-DRB1*04:04 variant: Most well-known risk factor
- PTPN22, CTLA4, BACH2 genes: Associated with increased autoimmune susceptibility
- Family clustering: Evidence suggests familial predisposition
Signs and Symptoms
Early Warning Signs
Addison’s disease symptoms develop gradually and can be easily mistaken for other conditions. Around one-half of patients with AD are diagnosed after an acute adrenal crisis, which can be rapidly fatal.
Primary Symptoms
Fatigue and Weakness
- Extreme tiredness that doesn’t improve with rest
- Muscle weakness, particularly in the legs
- Reduced exercise tolerance
Gastrointestinal Symptoms
- Nausea and vomiting
- Loss of appetite
- Unexplained weight loss
- Abdominal pain
Cardiovascular Signs
- Low blood pressure (hypotension)
- Dizziness when standing up quickly
- Feeling faint or lightheaded
Characteristic Features
Hyperpigmentation (Bronze Discolouration) One of the most distinctive signs is darkening of the skin, particularly in:
- Areas of friction (elbows, knuckles, knees)
- Palmar creases and knuckles
- Scars and skin folds
- Gums and inside of the mouth
- Nipples and genitals
Salt Cravings Intense craving for salty foods due to aldosterone deficiency
Mood and Cognitive Changes
- Depression and anxiety
- Irritability
- Confusion or brain fog
- Difficulty concentrating
Laboratory Abnormalities
Blood tests may reveal:
- Low sodium (hyponatraemia)
- High potassium (hyperkalaemia)
- Low blood sugar (hypoglycaemia)
- Low cortisol levels
- Elevated ACTH
Diagnosis and Testing
Initial Assessment
If Addison’s disease is suspected, consider measuring urea and electrolytes (U&E) as sometimes, although by no means always, a low sodium and high potassium will be found, and a 9 am serum cortisol level.
Primary Diagnostic Tests
Morning Cortisol Test
- Normal range: >500 nmol/L makes Addison’s disease very unlikely
- Low range: <100 nmol/L indicates definite abnormality requiring investigation
- Indeterminate: 100-500 nmol/L requires further testing
Short Synacthen Test (ACTH Stimulation Test)
The gold standard diagnostic test:
- Measures cortisol response to synthetic ACTH injection
- Normal response: Cortisol rises above 550 nmol/L
- Addison’s disease: Inadequate cortisol rise
Additional Specialist Tests
Additional relevant secondary care tests at the time of diagnosis include plasma adrenocorticotropic hormone and renin, and serum dehydroepiandrosterone sulfate.
Autoantibody Testing
- 21-hydroxylase antibodies: Present in 80-90% of autoimmune cases
- Adrenal cortex antibodies (ACA): Less specific but supportive
When to Suspect Addison’s Disease
Healthcare professionals should consider testing when patients present with:
- Unexplained fatigue lasting months
- Recurrent hypoglycaemia
- Unexplained nausea and vomiting
- Progressive skin pigmentation
- Low blood pressure with electrolyte imbalances
Emergency Diagnosis
If suspected (features of persistent vomiting, muscle weakness, dehydration, hypotension, headache, extreme fatigue, and shock), the patient should be admitted as a medical emergency.
Treatment and Management
Hormone Replacement Therapy
AAD requires life-long substitutive therapy with two-three daily doses of hydrocortisone (HC) (15–25 mg/day) or one daily dose of dual-release HC and with fludrocortisone (0.5–2.0 mg/day).
Glucocorticoid Replacement
Hydrocortisone (Cortisol Replacement)
- Standard dosing: 15-25mg daily in divided doses
- Timing: Larger dose in morning, smaller doses afternoon/evening
- Dual-release formulation: Once-daily option (Plenadren®)
Alternative Options
- Prednisolone: Sometimes used as alternative
- Dexamethasone: Rarely used due to long half-life
Mineralocorticoid Replacement
Fludrocortisone
- Standard dose: 0.05-0.2mg daily (some patients require up to 0.3mg)
- Function: Replaces aldosterone to regulate salt and water balance
- Monitoring: Blood pressure and electrolytes
Medication Timing and Dosing
Optimal Dosing Schedule
Morning dose (on waking)
- 60-70% of total daily hydrocortisone
- Take immediately upon waking
- Mimics natural cortisol rhythm
Afternoon dose
- 20-30% of daily dose
- Usually taken around lunchtime
- Some patients require third dose early evening
Monitoring and Adjustments
The lowest possible HC dose must be identified according to clinical and biochemical parameters to minimize long-term complications that include osteoporosis and cardiovascular and metabolic alterations.
Signs of Under-replacement
- Persistent fatigue
- Weight loss
- Low blood pressure
- Skin pigmentation
- Salt cravings
Signs of Over-replacement
- Weight gain
- High blood pressure
- Thin skin and easy bruising
- Mood changes
- Difficulty sleeping
- Blood sugar problems
Living with Addison’s Disease
Sick Day Rules
The Addison’s Disease Self-Help Group outlines essential Sick Day Rules for individuals with adrenal insufficiency, helping them manage their condition during illness, injury, or stress to prevent adrenal crisis. It emphasises the importance of adjusting steroid medication based on symptoms, such as fever, vomiting, or trauma, and provides specific dosage guidelines for different steroid treatments, including hydrocortisone, prednisolone, and Plenadren. Additionally, it advises on emergency situations, hospital preparation, and the necessity of maintaining a three-month reserve supply of essential medications. See here for details
Stress Dosing Guidelines
- Minor illness: Double usual dose
- Moderate illness: Triple usual dose
- Severe illness/surgery: IV hydrocortisone required
Emergency Preparedness
Emergency Injection Kit
All patients must carry:
- Hydrocortisone injection (typically Solu-Cortef® 100mg)
- Pre-filled syringes or ampoules
- Needles and syringes
- Instructions for use
When to Use Emergency Injection
- Persistent vomiting preventing oral medication
- Severe illness or injury
- Loss of consciousness
- Signs of adrenal crisis
Lifestyle Modifications
Diet and Nutrition
Salt Intake
- Increased salt requirement (especially in hot weather)
- Listen to body’s salt cravings
- Consider salt supplements during exercise
Regular Meals
- Maintain regular eating schedule
- Avoid prolonged fasting
- Carry snacks for emergencies
Exercise and Activity
- Regular moderate exercise beneficial
- Increase hydrocortisone for intensive exercise
- Stay hydrated
- Recognise early fatigue signals
Workplace and Travel Considerations
Employment Rights
- Addison’s disease may qualify as disability under Equality Act 2010
- Reasonable adjustments may include flexible hours
- Regular breaks and access to medication
Travel Preparations
- Carry extra medication (double requirements)
- Medical alert jewellery
- Travel insurance covering pre-existing conditions
- Doctor’s letter explaining condition and medications
- Time zone medication adjustment planning
Adrenal Crisis: What You Need to Know
What Is Adrenal Crisis?
An adrenal crisis (Addisonian crisis) is a life-threatening emergency occurring when cortisol levels drop dangerously low. An adrenal crisis is a medical emergency.
Recognising Adrenal Crisis
Warning Signs
- Severe nausea and vomiting
- Extreme weakness and fatigue
- Low blood pressure
- Severe abdominal, back, or leg pain
- Confusion or loss of consciousness
- High fever
- Severe dehydration
Emergency Action Plan
Immediate Steps
- Give emergency hydrocortisone injection immediately
- Call 999 stating “adrenal crisis” or “Addisonian crisis”
- Lie patient flat and keep warm
- Monitor breathing and consciousness
Emergency Treatment
- IV hydrocortisone: 100-200mg immediately
- IV fluids: Large volumes of saline solution
- Hospital admission: For monitoring and continued treatment
Prevention Strategies
Medication Compliance
- Never miss hydrocortisone doses
- Carry spare medication at all times
- Set medication alarms
- Educate family/friends about emergency procedures
Early Intervention
- Increase medication at first sign of illness
- Seek medical attention promptly for persistent symptoms
- Don’t delay emergency injection if in doubt
Associated Autoimmune Conditions
Autoimmune Polyendocrine Syndromes
Most people with Addison’s disease develop or have a preexisting autoimmune disease.
Type 1 (APS-1)
Characteristics:
- Typically diagnosed in childhood
- Addison’s disease with chronic mucocutaneous candidiasis
- Hypoparathyroidism
Type 2 (APS-2) – Most Common
Associated conditions:
- Autoimmune thyroid disease (40% of patients)
- Type 1 diabetes (11% of patients)
- Premature ovarian failure (up to 16% of patients)
Other Common Associations
Vitiligo (6% of patients)
- Patches of depigmented skin
- Often appears before Addison’s diagnosis
- May be first sign of autoimmune process
Pernicious Anaemia (10% of patients)
- Vitamin B12 deficiency
- Requires regular monitoring and supplementation
Coeliac Disease (2% of patients)
- Gluten intolerance
- May require strict gluten-free diet
Monitoring for Associated Conditions
Annual surveillance for other autoimmune disorders is recommended.
Recommended Annual Screening
- Thyroid function tests (TSH, T4)
- HbA1c or glucose tolerance test
- Vitamin B12 levels
- Calcium and parathyroid hormone
- Coeliac antibodies (if symptomatic)
Support and Resources in the UK
Primary Support Organisation
Addison’s Disease Self-Help Group (ADSHG)
The Addison’s Disease Self-Help Group (ADSHG) is the UK & Ireland charity working to improve the lives of people with Addison’s Disease, adrenal insufficiency and all who support them.
Contact Information:
- Website: www.addisonsdisease.org.uk
- Address: Starling House, 1600 Bristol Parkway North, Bristol, BS34 8YU
- Charity Registration: 1179825
Services Provided:
- Information and educational resources
- Online support forum
- Local support groups
- Emergency medical advice
- Healthcare professional education
- Research funding
ADSHG Online Forum
- 24-hour peer support
- Experienced patient guidance
- Medication and lifestyle advice
- Emergency support sanctuary
NHS Resources
NHS England
- NHS 111: For urgent medical advice
- NHS website: www.nhs.uk/conditions/addisons-disease/
- Local endocrinology services: Hospital-based specialist care
Specialist Centres
Major UK centres with Addison’s expertise:
- Royal Victoria Infirmary, Newcastle
- Birmingham Heartlands Hospital
- St Bartholomew’s Hospital, London
- University Hospital of Wales, Cardiff
Professional Organisations
Society for Endocrinology
- Website: www.endocrinology.org
- Clinical guidelines and professional resources
- Patient information materials
- Healthcare professional training
Royal College of General Practitioners
- Primary care guidance
- Educational resources for GPs
- Addison’s disease awareness initiatives
Mental Health Support
NHS Mental Health Services
- Counselling and psychological support
- Depression and anxiety management
- Chronic illness adaptation support
Charity Support
- Mind: Mental health charity
- Samaritans: 24-hour emotional support
- Citizens Advice: Benefits and employment guidance
Financial Support
Benefits and Allowances
Addison’s disease may qualify for:
- Personal Independence Payment (PIP)
- Employment and Support Allowance
- Blue Badge parking permit
- Medical exemption certificate (prescription charges)
Insurance Considerations
- Travel insurance with pre-existing condition cover
- Life insurance disclosure requirements
- Critical illness insurance eligibility
Frequently Asked Questions
General Questions
Q: Is Addison’s disease hereditary? A: While not directly inherited, there is a genetic predisposition to autoimmune conditions. Since individuals with Addison’s disease (AD) present considerable co-occurrence of additional autoimmune conditions, clustering of autoimmunity was also predicted among their relatives.
Q: Can Addison’s disease be cured? A: Currently, there is no cure for Addison’s disease. However, with proper hormone replacement therapy, most people live normal, active lives. With treatment, symptoms of Addison’s disease can largely be controlled. Most people with the condition have a normal lifespan and are able to live an active life with few limitations.
Q: Will I need to take medication forever? A: Yes, hormone replacement therapy is required for life. The adrenal glands cannot regenerate once destroyed by the autoimmune process.
Medication Questions
Q: What happens if I miss a dose of hydrocortisone? A: Take the missed dose as soon as you remember, unless it’s nearly time for your next dose. Never double up doses. If you feel unwell, contact your healthcare provider.
Q: Can I drink alcohol with Addison’s disease? A: Moderate alcohol consumption is generally safe, but alcohol can affect blood sugar levels. Always take your medication as prescribed and eat regularly when drinking.
Q: Are there any medications I should avoid? A: Some medications can interact with hydrocortisone or affect adrenal function. Always inform healthcare providers about your Addison’s disease before receiving any new medications.
Lifestyle Questions
Q: Can I exercise with Addison’s disease? A: Yes, regular exercise is beneficial. You may need to increase your hydrocortisone dose for intense exercise and ensure adequate hydration and salt intake. See tips on gentle movement
Q: Can women with Addison’s disease have children? A: Yes, pregnancy is possible but requires specialist management. Women with AAD have lower fertility and parity as compared to age-matched healthy controls. Hormone doses need adjustment during pregnancy.
Q: Can I work with Addison’s disease? A: Most people with well-managed Addison’s disease can continue working. You may need workplace adjustments, and your employer has a duty to make reasonable accommodations.
Emergency Questions
Q: When should I use my emergency injection? A: Use immediately if you’re vomiting and can’t keep oral medication down, experiencing signs of crisis, or are unconscious/severely unwell. When in doubt, use it – it’s safer to give than to delay.
Q: What should I tell paramedics in an emergency? A: Clearly state “I have Addison’s disease and this is an adrenal crisis.” Show your medical alert jewellery and emergency card. Inform them of any emergency injection already given.
Key Takeaways
Essential Points to Remember
- Autoimmune Addison’s disease is the leading cause of primary adrenal insufficiency in the UK
- Early diagnosis is crucial but often delayed due to non-specific symptoms
- Lifelong hormone replacement is essential for survival and quality of life
- Emergency preparedness can be life-saving during adrenal crisis
- Regular monitoring helps detect associated autoimmune conditions
- Support networks provide invaluable practical and emotional assistance
Living Successfully with Addison’s Disease
With proper management, education, and support, people with autoimmune Addison’s disease can lead fulfilling, active lives. The key components are:
- Consistent medication adherence
- Emergency preparedness
- Regular medical monitoring
- Lifestyle adaptations
- Strong support networks
- Ongoing education
Getting Support
If you’ve been diagnosed with Addison’s disease, you’re not alone. Connect with the Addison’s Disease Self-Help Group and speak with your healthcare team about developing a comprehensive management plan tailored to your needs.
Remember: Knowledge is power, preparation saves lives, and support makes the journey manageable.
Disclaimer: This guide provides general information about autoimmune Addison’s disease and should not replace professional medical advice. Always consult your healthcare provider for personalised medical guidance and treatment decisions.
Last Updated: June 2025 | Next Review: June 2026
About This Guide
This comprehensive guide has been developed using current NHS guidelines, peer-reviewed medical literature, and guidance from the Addison’s Disease Self-Help Group. All medical information is sourced from reputable organisations including the NHS, Society for Endocrinology, and leading UK endocrinology centres.
