Hyper-IgE syndrome

A patient-friendly guide (and why it matters if you have aspergillosis)

It is not the same as having lots of allergies, even though it can look very similar at first.

What is IgE, and why does it matter?

IgE is usually involved in allergies and asthma.

In Hyper-IgE syndrome:

• IgE levels are extremely high (often many thousands)

• But the immune system is unbalanced

• This makes infections—especially in the lungs and skin—harder to control

So IgE is high, but protection is weak.

How might Hyper-IgE syndrome affect everyday life?

Not everyone has the same symptoms, but common features include:

Lung and chest problems

• Repeated chest infections (often from a young age)

• Ongoing cough, breathlessness and mucus

• Lung damage such as bronchiectasis

• Lung cavities that can later become infected by moulds such as Aspergillus

Skin and infection problems

• Long-standing eczema or very sensitive skin

• Recurrent skin infections or boils

• Infections that keep coming back or take a long time to clear

Other clues (in some people)

• Frequent infections in childhood

• Bone or joint problems

• Dental issues (for example baby teeth not falling out on time)

Why is this important for people with aspergillosis?

For many people, Aspergillus causes allergy or irritation.

In Hyper-IgE syndrome:

• The immune system struggles to control moulds

• Aspergillus can behave more like a true infection, not just an allergy

• Lung damage can happen more easily and progress faster

This means doctors may need to:

• Monitor lungs more closely

• Treat fungal disease earlier and for longer

• Be cautious with repeated or long-term steroid use

Specialist centres such as the National Aspergillosis Centre are often involved when aspergillosis and immune problems overlap.

Isn’t this just severe allergy or ABPA?

Hyper-IgE syndrome can look similar to:

• Severe allergic asthma

• Allergic Bronchopulmonary Aspergillosis (ABPA)

The key difference is that in Hyper-IgE syndrome:

• The immune system itself is faulty

• High IgE is part of a wider immune problem

• Treating allergy alone may not be enough

Some people are treated for asthma or ABPA for years before this possibility is considered.

How is Hyper-IgE syndrome treated?

There is no single cure, but good treatment can make a big difference. The aim is to prevent infections, protect the lungs, and reduce symptoms.

1. Preventing infections (most important)

Because the immune system does not fight germs normally:

• Some people take regular low-dose antibiotics

• Others use antibiotics early and promptly when infections start

For people with aspergillosis:

• Antifungal medicines may be needed

• Monitoring is usually closer and longer-term

2. Protecting the lungs

Many people develop bronchiectasis or lung damage, so care often includes:

• Airway clearance physiotherapy

• Saline nebulisers to help clear mucus

• Regular sputum tests

• Early treatment of flare-ups

The goal is to stop the cycle of:

infection → inflammation → permanent lung damage

3. Managing inflammation and allergy (carefully)

People may also have asthma-like symptoms, eczema and multiple allergies.

• Steroids can help symptoms, but long-term or frequent use can increase infection risk

• Doctors usually try to keep steroid doses as low as possible

Biologic treatments (such as anti-IgE medicines):

• May help some people

• Do not fix the immune problem

• Are considered on an individual basis, usually in specialist centres

4. Skin care

• Regular moisturising

• Prompt treatment of infected eczema

• Good skin care helps reduce infection risk

How is Hyper-IgE syndrome diagnosed?

Diagnosis usually involves:

• A detailed review of your medical history (often including childhood infections)

• Blood tests of immune function

• Referral to an immunology specialist

• Sometimes genetic testing

Does having high IgE mean I definitely have this?

No.
Hyper-IgE syndrome is rare.

But it may be worth asking about if:

• Your IgE has always been extremely high

• You’ve had repeated infections for many years

• You have bronchiectasis without a clear cause

• Aspergillosis seems unusually persistent or severe

• Standard asthma or allergy treatments don’t fully explain your symptoms

Key message

Very high IgE does not always mean “just allergy.”
In a small number of people, it reflects a deeper immune problem that changes how aspergillosis behaves and how it should be treated.

If your illness doesn’t quite fit the usual labels, it is reasonable to ask whether an immunology review would help.