Autoimmune diseases are a broad range of more than 80 related disorders that include rheumatoid arthritis, systemic lupus erythematosus (lupus) and vasculitis disorders (inflammation of blood vessels). They are thought to be inherited in many cases, but factors such as infections and some drugs (medications) may also play a role in triggering autoimmune diseases.

Autoimmune diseases are  usually diagnosed using a combination of clinical history, blood tests and other investigations such as x-rays and a biopsy. In autoimmune diseases, the immune system attacks the body's own cells, tissues and organs, resulting in inflammation and damage, so treatments aim to relieve symptoms, minimise organ and tissue damage and preserve organ function. 

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Fast Facts about Autoimmune Diseases 

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Autoimmune Diseases 

Sjögren’s Disease

Systemic Lupus Erythematosus (SLE) 

Vasculitis Disorders

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Webpage updated April 2025

ASCIA PCC Vasculitis disorders 2019162.14 KB

Vasculitis disorders result from inflamed of blood vessels. They are relatively rare and can affect people of all ages. Granulomatosis with polyangiitis (also known as Wegener’s granulomatosis) is the most common form of vasculitis, and it affects around five in a million people.

Vasculitis disorders cause a wide range of symptoms, that can affect the skin and internal organs. Treatment length varies, and some people need to use medications for long periods of time.

What is vasculitis?

Vasculitis disorders result from inflammation of blood vessels, including arteries, arterioles, veins, venules and capillaries. The inflammation causes a narrowing of blood vessels, which can result in blood flow obstruction (ischaemia).  This may lead to tissue damage (necrosis) and blood clots (thrombosis). 

What causes vasculitis?

There are three main underlying causes of vasculitis disorders:

• Autoimmunity.
• Allergy or hypersensitivity to medications, toxins or other inhaled environmental irritants (where removing the medication, toxin or irritant usually stops symptoms).
• Viral or parasite Infections.

Allergy, hypersensitivity and infections should be ruled out before autoimmunity is considered as the cause of a vasculitis disorder.  

Autoimmune related vasculitis

The main role of the immune system is to defend against infections (such as bacteria, moulds and viruses) and other invaders (such as cancer cells), whilst protecting the body’s own cells. Autoimmunity occurs when the body doesn’t recognise its own cells and attacks them.

Antibodies produced by the immune system in vasculitis disorders cause inflammation in blood vessels that can lead to problems. Complications depend on which blood vessels, organs and other systems are affected.   

Vasculitis disorders may also occur in people with other autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus (SLE) and dermatomyositis. 

Diagnosis 

Due to the wide range of signs, symptoms and body systems involved, an extensive history and physical examination is needed to diagnose the type of vasculitis disorder. Blood tests are taken, and in some cases an x-ray or biopsy may be required. An exact diagnosis is needed to provide the right treatment.

Treatment

When vasculitis is due to an autoimmune disorder, immunosuppressive drugs are usually used.

Plasmapheresis (a procedure that filters the offending autoantibodies out of the blood plasma and returns the filtered blood back to the patient), may be used in serious cases that do not respond to other treatment.

Signs and symptoms

General signs and symptoms of vasculitis disorders include:

• Fever.
• Loss of appetite and weight loss.
• Fatigue, weakness and lethargy.
• General aches and pains.

Specific signs and symptoms of vasculitis disorders include:

• Skin may have purple or red spots or bumps, clusters of small dots, splotches, bruises, urticaria (hives), itch.
• Joints may have pain, arthritis in one or more joints.
• Lungs display a shortness of breath, coughing up blood, signs that suggest pneumonia.
• Gastrointestinal tract as indicated by mouth ulcers (sores), or stomach pain. In severe cases blood flow to the intestines can be blocked.
• Sinuses, nose, throat, ears where chronic (ongoing) sinus or middle ear infections, ulcers in the nose, and hearing loss are evident.
• Eyes may be red, itchy and burning, increased sensitivity to light, blurred vision. 
• Brain may experience headaches, changes in mental function, stroke-like symptoms such as muscle weakness and paralysis.
• Nerves resulting in numbness, tingling, and weakness in various body parts, loss of feeling or strength in hands and feet, shooting pains in arms and legs.

Examples of vasculitis disorders (small blood vessels)

• Granulomatosis with polyangiitis affects sinuses, lungs, kidneys, and skin.
• Eosinophilic granulomatosis with polyangiitis affects the lungs, skin, and nerves.
• Cryoglobulinaemia affects skin, kidneys, and nerves.
• Goodpasture’s syndrome affects lungs and kidneys.
• Henoch-Schonlein purpura affects skin, joints, kidneys, and gut.
• Microscopic polyangitis affects skin, kidneys, and nerves.

Examples of vasculitis disorders (medium blood vessels)

• Behcet’s disease affects mucous membranes, skin, and eyes.
• Central nervous system vasculitis affects the brain.
• Kawasaki syndrome affects skin, mucous membranes, lymph nodes, and blood vessels.
• Polyarteritis nodosa affects arteries, kidneys, gut, nerves, and skin.

Examples of vasculitis disorders (large blood vessels)

• Giant cell (temporal) arteritis affects arteries of the head and neck.
• Takayasu arteritis affects arteries of the head and neck.
• Polymyalgia rheumatic produces inflammation and swelling in joint and muscles.

© ASCIA 2019

ASCIA is the peak professional body of clinical immunology/allergy specialists in Australia and New Zealand.

ASCIA resources are based on published literature and expert review, however, they are not intended to replace medical advice. The content of ASCIA resources is not influenced by any commercial organisations.

For more information go to www.allergy.org.au 

To donate to immunology/allergy research go to www.allergyimmunology.org.au/donate 

Updated May 2019

ASCIA PCC Systemic Lupus Erythematosus (SLE) 2019135.77 KB

Systemic Lupus Erythematosus (SLE), also known as lupus, is a disease of the immune system, which is estimated to affect more than 20,000 people in Australia and New Zealand. Symptoms can be vague and vary between people, and therefore diagnosis can be difficult. However, once diagnosed, a combination of prescribed treatment and lifestyle adjustments enables most people with lupus to enjoy an almost normal life.

What is lupus?

The main role of the immune system is to fight foreign invaders such as bacteria, moulds and viruses.  In autoimmune diseases the immune system produces antibodies that attack the body's own healthy tissue. Lupus is an autoimmune disease, and the antibodies produced by the immune system in lupus cause inflammation, tissue damage and pain.

Who is affected by lupus?

Around 90% of people with lupus are women and the majority develop the condition between 15 and 45 years. When lupus occurs in children it is usually diagnosed during puberty. Lupus is more common and severe in Indigenous Australians, Polynesians and those with descendants from South East Asia. 

There are two main types of lupus

There are two main types of lupus, which differ significantly in the type and severity of symptoms:

• Systemic lupus erythematosus (also known as SLE) is characterised by flare ups and periods of improvement (remissions), and can can affect almost any organ or system of the body.  In most people only the skin and joints are affected.  However, in some people SLE can also affect the kidneys, lungs, heart, blood vessels and/or brain.
• Discoid lupus (also known as chronic cutaneous lupus erythematosus) is generally milder than SLE and usually appears as a red scaly rash on sun exposed areas such as the face, scalp, arms, legs or trunk.  Most people with discoid lupus have symptoms only on their skin.  However, a small number of people with discoid lupus will develop SLE. 

Other milder forms of lupus include:

• Subacute cutaneous lupus - the main symptoms are skin rashes, sun sensitivity and joint aches.
• Drug induced lupus - this is usually a transient form that develops as a reaction to certain medications and clears up when the medications are ceased. 

The cause of lupus is unknown

A combination of genetic and environmental factors contribute to the formation of the antibodies that lead to lupus.  Possible triggers of disease flare ups include:

• Hormones
• Certain medications and chemicals
• Viral and bacterial infections
• Exposure to UV light
• Dietary factors
• Stress
• Pregnancy

Lupus symptoms may be vague, variable and unpredictable

Lupus can cause many symptoms, including:

• Joint pain or swelling - seen in ~50% of people with lupus
• Skin rashes that get worse with sun exposure - seen in ~20% of people with lupus
• Fever
• Loss of appetite and weight loss
• Fatigue, weakness and lethargy - these affect ~10% of people with lupus and may be severe

Most people with lupus will never experience all the symptoms and no two individuals seem to experience identical symptoms.

The course of lupus is usually unpredictable

For some people, symptoms will subside after treatment of the initial acute attack.

For others, periods of improvement (remission) are punctuated by brief flares of disease.

Early diagnosis is important

The diagnosis of lupus is usually suspected on the basis of clinical symptoms and signs and confirmed by laboratory tests.

Blood tests will usually include an Anti Nuclear Antibody (ANA) test, which measures antibodies to self tissues. Whilst this is a good screening test, not all people with SLE have a positive ANA result and many people with a positive ANA do not have SLE. For example, close relatives of SLE patients may have a positive ANA without developing SLE themselves. Additional blood tests are therefore necessary to confirm the diagnosis and to monitor the activity of SLE.

Effective treatments are available for lupus

The aim of lupus treatments is to reduce inflammation in tissues and improve quality of life. Treatment must be individualised, taking account of the severity of the disease.

There are five main groups of drugs that are used to treat lupus:

• Non steroidal anti inflammatory drugs (NSAIDs) such as Aspirin, ibuprofen, naproxen and Cox-2 inhibitors reduce inflammation and can relieve the fevers, muscle aches, and arthritis that accompany lupus, but they do not alter the course of the disease or the underlying immune process.
• Anti malaria drugs (such as hydroxychloroquine and chloroquine) are often used to reduce joint pains, skin rashes and fatigue.
• Corticosteroids (such as Prednisolone) are very effective anti inflammatory medications and are the drugs of choice for treating serious complications of lupus, such as those affecting the heart, lungs and nervous system.
• Immune suppressing drugs (such as methotrexate) suppress the immune system and are generally used when serious disease is present and steroids alone are not enough to control the disease.
• Cytotoxic drugs (such as cyclophosphamide) are potent immunosuppressive agents that are used to treat serious manifestations of SLE, especially kidney inflammation (glomerulonephritis).

Diagnosis, treatment and lifestyle changes are important

The outlook for most people with lupus is good. Early detection, effective treatment and some lifestyle adjustments enable most people with lupus to feel well and live normal lives. It is only a small minority of people who find the condition substantially reduces their quality of life.

Lupus and pregnancy

Women with lupus should talk to their doctor before considering pregnancy. They should be made aware of any potential risk for themselves and the baby. It is preferable for lupus to be in remission, as this reduces the risk of a disease flare occurring during pregnancy. Lupus flares occurring during pregnancy are usually mild and occur in the first three months (trimester). In the first few weeks after birth new mothers may experience lupus flares but this can be controlled with corticosteroids.

It is important to discuss therapy options with your doctor to ensure that any current medication taken will not adversely affect the pregnancy. Women should have no difficulty becoming pregnant as lupus does not usually lower fertility.  However, a small proportion of women with lupus are at increased risk of blood clots and recurrent miscarriages.

Some tips to help people with lupus to lead normal lives

• Take rests before allowing yourself to become fatigued, which may mean restructuring your schedule and avoiding stressful situations.
• Commence a program of regular moderate exercise (without becoming fatigued) as this should improve well being and prevent muscle wasting.
• Sun exposure can trigger flare ups so avoid excessive exposure to the sun by wearing a broad brimmed hat, long sleeves and long trousers, and always use maximum strength sunblock that protects against UVA and UVB rays.
• Avoid contact with people who have known infections.
• Find out as much about lupus as you can to help gain control and remove fear.
• Visit your doctor regularly (particularly if you feel that your symptoms are worsening) and keep a list of your symptoms and any questions you might have for your doctor.
• Always take your medications as you have been instructed and keep a record of your medications.
• Do not smoke.
• Drink alcohol only in moderation (up to two standard drinks daily)

There are currently no cures for lupus, but there are effective medications that will bring the disease under control, and often permanently. As you grow older, it is likely that the disease will improve.

© ASCIA 2019

ASCIA is the peak professional body of clinical immunology/allergy specialists in Australia and New Zealand.

ASCIA resources are based on published literature and expert review, however, they are not intended to replace medical advice.

The content of ASCIA resources is not influenced by any commercial organisations.

For more information go to www.allergy.org.au

To donate to immunology/allergy research go to www.allergyimmunology.org.au/donate