Igg4 Disease

Overview

IgG4-related disease (IgG4-RD) is a rare condition where the immune system causes inflammation and scarring in one or more organs. IgG4 is a type of antibody made by the body. In this disease, immune cells and antibodies build up in tissues, which can make organs swell or stop working properly. It is not an infection and cannot be passed from person to person.

It is rare. Many doctors may see only a few cases in their whole career.

It can affect anyone. It is most often found in middle-aged and older adults, and slightly more often in men. Children can get it, but this is very rare.

Symptoms

Common symptoms

• Painless swelling of glands near the jaw, cheeks, or eyes
• Enlarged lymph nodes
• Dry eyes or dry mouth
• Tummy pain, especially from inflammation of the pancreas
• Yellowing of the skin or eyes if the liver or bile ducts are involved
• Feeling very tired
• Unexplained weight loss
• Fever
• Kidney problems found on blood tests
• Swelling or fullness around the eyes

Symptoms in children

• IgG4-related disease is very rare in children.
• When it happens, symptoms depend on which organs are affected and may include swelling, tiredness, or tummy pain.
• Diagnosis can be delayed because the disease is uncommon in children, so specialist care is important.

Symptoms in older adults

• It is more common in older adults.
• It can be mistaken for other conditions, so tests and specialist review may take time.
• Older adults may have other health conditions, so treatment and monitoring need to be carefully tailored.

Causes

Main causes

• The exact cause is not known.
• It is an immune-mediated condition: the immune system, which normally fights infection, mistakenly attacks the body's own tissues.
• This leads to inflammation and scarring in organs.
• It is not an infection and is not spread from person to person.
• Genetic and environmental factors may play a role, but no single trigger has been proven.

Risk factors

• Being middle-aged or older
• Being male
• Asian ancestry has been reported more often in some studies, but it can affect anyone
• A family history of other immune-related conditions may sometimes be present, but there is no clear inherited pattern

When to see a doctor

See a doctor urgently if:

• You develop yellowing of the skin or eyes
• You have severe tummy pain, fever with chills, or cannot keep fluids down
• You have a sudden change in vision or severe eye pain
• You notice new confusion, severe weakness, or trouble breathing

Book a routine appointment if:

• A painless lump or swelling lasts more than a few weeks
• Dry eyes or dry mouth persists
• You have unexplained tiredness, weight loss, or fever
• Blood tests show liver, kidney, or immune changes that need checking
• You have repeated swelling in the glands near your jaw, cheeks, or eyes

Diagnosis

There is no single test for IgG4-related disease. A doctor combines your symptoms, a physical exam, blood tests, scans, and often a biopsy. They also rule out infections, cancers, and other immune conditions.

Tests that may be done

• Blood tests, including IgG4 level and markers of inflammation
• Liver and kidney blood tests
• Urine tests
• Imaging such as ultrasound, CT, or MRI scans
• A biopsy, where a small sample of tissue is checked under a microscope
• Eye exams if the eyes or glands around the eyes are affected
• Heart or lung tests if those organs may be involved

What to expect at your appointment

Diagnosis can take time. You may be referred to a specialist, such as a rheumatologist, gastroenterologist, or immunologist. Biopsy results can take days to weeks. Doctors need to look at the whole picture before confirming the diagnosis.

Treatment

Treatment aims to reduce inflammation, protect organs, and prevent scarring. It is tailored to which organs are involved and how active the disease is.

Self-care at home

• Keep a symptom diary so you can tell your care team what is changing
• Attend monitoring appointments and blood tests
• Rest when you feel tired, and plan your day around your energy
• Use gentle eye and mouth care if you have dryness
• Do not smoke, and keep alcohol low
• Ask your pharmacist or care team before starting any new medicine, including over-the-counter products

Medical treatments

Doctors usually start with medicines that calm the immune system. Steroid medicines are a common first treatment. Other immune-suppressing medicines or biologic medicines may be used if the disease is severe, keeps coming back, or steroid treatment causes problems. Treatment is adjusted over time. Antibiotics do not help because this is not an infection. Your care team will monitor you with blood tests and scans.

When is surgery considered?

Surgery is not a usual cure. It may be needed to take a biopsy, relieve a blockage, or repair a complication such as a weakened, bulging blood vessel. Ask your specialist whether surgery is relevant to your case.

Living with this condition

Many people manage with regular follow-up. Tiredness and appointments can affect work, school, and family life. Planning rest, asking for help, and keeping good records can make daily life easier.

Lifestyle tips

• Stop smoking and avoid secondhand smoke
• Keep alcohol low
• Get enough sleep and rest when needed
• Stay physically active in a way that suits your energy and health
• Manage stress with simple routines, breathing exercises, or talking to someone you trust
• Keep up with eye and mouth care if you have dryness

Diet and exercise

No special diet has been proven to cure IgG4-related disease. Eat balanced meals, stay hydrated, and limit very salty and highly processed foods. Gentle activity, such as walking, can help your general health. Ask your care team if you need a special plan because of kidney, pancreas, heart, or blood vessel involvement.

Mental health and emotional wellbeing

Living with a long-term condition can cause worry, low mood, or feeling isolated. These feelings are common and treatable. Talking to your care team, a counsellor, or trusted people can help. If you ever have thoughts of harming yourself or ending your life, contact your local emergency number or a local crisis support service right away.

Prevention

There is no known way to prevent IgG4-related disease. Because the cause is not fully understood, the focus is on early recognition, treatment, and regular follow-up.

Vaccines

No vaccine prevents IgG4-related disease. If you take immune-suppressing medicines, ask your care team which vaccines are safe for you and when to have them.

Screening programmes

There is no routine screening test for the general public. If you have the disease, doctors monitor the organs involved with blood tests and scans.

Complications

If left untreated

• Organ damage from ongoing inflammation and scarring
• Blockages in ducts or organs
• Diabetes if the pancreas is damaged
• Kidney problems
• Eye or vision problems
• A weakened, bulging blood vessel called an aneurysm
• Return of symptoms after treatment

Long-term outlook

Most people improve with treatment. The disease can come back, so follow-up matters. Many people live full lives with monitoring and care. IgG4-related disease is not cancer. With early care, serious damage is often preventable or manageable.

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