Hyper-IgE syndrome

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

Hyper-IgE syndrome is a rare condition of the immune system. People with it have very high levels of an antibody called Immunoglobulin E (IgE), but their immune system does not work properly at fighting certain infections.

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.

by GAtherton

Sinusitis in Patients with ABPA

When to suspect it, when to investigate, and when to refer

Why this matters

Patients with allergic bronchopulmonary aspergillosis (ABPA) are usually managed as having a lung disease. Diagnosis, monitoring, and treatment focus appropriately on the chest, immunology, and asthma control.

However, ABPA occurs within a single continuous airway, extending from the nose and sinuses to the lungs. Disease in the upper airway can coexist with, exacerbate, or complicate lower airway inflammation — yet sinus disease is not routinely assessed in ABPA care pathways.

This article outlines:

What is known about sinus disease in this context
Which symptoms should raise suspicion
When investigation or ENT referral should be considered
What GPs and non-specialists can reasonably do

The united airway: a brief reminder

The upper and lower airways share:

Type 2 (eosinophilic) inflammation
Immunoglobulin E–mediated immune responses
Common triggers, including allergens and fungi

Chronic rhinosinusitis is common in asthma and severe asthma, and treatment of sinus disease can improve lower airway outcomes in some patients.
ABPA sits within this same inflammatory spectrum, even though its management is lung-centred.

Sinus disease in ABPA: what is (and isn’t) known

What we know

Chronic rhinosinusitis is common in patients with asthma and severe asthma
Sinus disease may be symptomatic or relatively silent
ABPA guidelines do not mandate routine ENT review or sinus imaging
ENT involvement, therefore, varies widely between centres

What we do not know

Whether routine ENT assessment improves ABPA outcomes
Which ABPA patients benefit most from sinus intervention
The optimal timing for ENT referral in ABPA

As a result, clinical judgement remains central.

Symptoms that should prompt consideration of sinus disease

Sinusitis in ABPA patients does not always present with classic “blocked nose and facial pain”.
Key symptoms include:

Common but often overlooked

Persistent post-nasal drip
Foul, bitter, metallic, or “infected” taste in the mouth
Throat clearing, chronic cough
Thick or sticky mucus sensation
Symptoms are worse on waking or lying flat

More typical sinonasal features

Nasal blockage or congestion
Facial pressure or fullness
Reduced or altered sense of smell
Nasal crusting or discharge

Contextual clues

Poor durability of response to steroids or antifungals
Recurrent “flares” without clear chest triggers
Coexisting severe asthma or nasal polyps
Symptoms are worse in damp or mould-affected housing

A persistent foul taste in the mouth is a recognised symptom of chronic sinus disease, usually due to post-nasal drainage of inflamed secretions.

Damp homes and sinus disease

Living in damp or mould-affected environments is associated with:

Higher rates of chronic rhinosinusitis
Upper airway irritation and inflammation
Allergic sensitisation to fungal spores

In most cases, this results in inflammatory or allergic sinusitis, not invasive fungal infection.
Fungal involvement may act as an immune trigger, even when not labelled as “fungal sinusitis”.

Fungal sinusitis: rare vs under-recognised

It is important to distinguish between entities:

Type	Frequency	Key point
Invasive fungal sinusitis	Rare	Usually immunocompromised; dramatic presentation
Fungal ball (mycetoma)	Uncommon	Usually obvious on CT
Allergic fungal rhinosinusitis	Likely under-recognised	Requires active suspicion

Allergic fungal rhinosinusitis overlaps biologically with ABPA:

IgE-mediated
Eosinophilic inflammation
Thick allergic mucin

It is not routinely sought, so it may be under-diagnosed in at-risk groups.

What GPs and non-specialists can reasonably do

1. Take upper airway symptoms seriously

Especially in ABPA or severe asthma patients with:

Persistent post-nasal symptoms
Foul taste
Recurrent unexplained deterioration

2. Examine the nose and throat

Look for polyps, discharge, and crusting
Note mouth breathing or altered voice quality
Check dentition (to exclude dental causes)

3. Consider imaging when symptoms persist

CT sinuses (not plain X-ray) is the imaging of choice
Particularly appropriate if symptoms last >8–12 weeks or recur

4. Refer to ENT when:

Symptoms are persistent or progressive
CT shows significant sinus disease
There is a poor response to standard medical therapy
There is diagnostic uncertainty

Referral does not imply surgery — ENT input may be diagnostic or medical.

What this article is not saying

It does not suggest that all ABPA patients need an ENT referral
It does not claim that sinus treatment improves ABPA outcomes
It does not override existing guidelines

It does suggest that earlier consideration of the upper airway is reasonable in selected patients.

Key take-home points for clinicians

The airway functions as a single inflammatory system
Sinus disease may be subtle, under-reported, or atypical
A foul taste in the mouth is a meaningful symptom
Damp or mould exposure increases sinus disease risk
ENT referral is appropriate when symptoms persist or recur
Evidence gaps remain — but clinical vigilance is justified

In summary

ABPA is managed as a lung disease, but patients live with a whole airway.
Recognising when sinus disease may be contributing can help explain persistent symptoms and guide appropriate referral — without over-investigation or over-treatment.

by GAtherton

ABPA and Work: What a Patient Poll Tells Us About Employment, Health, and Real-World Impact

An article for patients, GPs, and non-specialist healthcare professionals

Allergic bronchopulmonary aspergillosis (ABPA) is often discussed in terms of lung function, immunology, and imaging. Far less often do we talk about its impact on everyday life, particularly on a person’s ability to work.

A poll run within the National Aspergillosis Centre patient community asked a simple but powerful question:

Who is still able to work while living with ABPA – and who has had to stop or retire?

The responses provide an important insight into the functional and socioeconomic burden of ABPA.

Key findings from the poll (patient-reported)

Working full time: 17%
Working part time (days or hours): 18% combined
Not working: 30%
Retirement age: 21%
Retired early for health reasons: 12%
Currently on sick leave / full-time carer / pre-diagnosis: small but notable groups

Even allowing for the informal nature of a social media poll, the overall pattern is clear.

What this tells us

1. Sustained full-time work is uncommon in ABPA

Fewer than one in five respondents were able to work full time. Even among those still working, many described reduced hours, flexible arrangements, or fragile employment dependent on day-to-day health.

ABPA is often incompatible with predictable, high-demand working patterns.

2. ABPA frequently leads to work loss or early retirement

A substantial proportion of respondents were either:

No longer working at all, or
Retired earlier than planned specifically because of health

This is particularly striking given that ABPA often affects people during their working years and may coexist with asthma, bronchiectasis, or long-term steroid use.

3. “Retirement age” can hide health-forced exit

Some respondents selected “retirement age,” but accompanying comments revealed that many:

Left work earlier than expected
Changed careers or reduced responsibilities years before retirement
Worked through ill health until they no longer could

This matters when interpreting employment statistics: health-driven work loss may be underestimated.

4. Unpaid work and instability are often overlooked

The poll also highlighted:

People currently on prolonged sick leave
Full-time unpaid carers
Individuals still awaiting diagnosis but already struggling to work

These groups are frequently invisible in employment data, yet represent significant personal and societal impact.

Why ABPA affects the ability to work

For patients and non-specialists, it is important to understand that work difficulties in ABPA are not simply due to “asthma symptoms.”

Common contributors include:

Chronic breathlessness and cough
Severe fatigue and post-exertional exhaustion
Recurrent chest infections
Steroid side-effects (muscle weakness, bone disease, mood changes, diabetes risk)
Unpredictable flare-ups requiring rest, antibiotics, or hospital care
Cognitive and emotional burden of long-term illness

Together, these make consistent attendance, physical work, and high cognitive load difficult to sustain.

Implications for patients

Difficulty working is not a personal failure
Many others with ABPA face similar challenges
Adjustments, reduced hours, or stopping work altogether may be medically appropriate
Asking for support is reasonable and justified

Implications for GPs and non-specialist clinicians

Employment status should be considered a key outcome of disease control
Fit notes, occupational health input, and benefits documentation are part of holistic care
ABPA is a fluctuating condition – patients may cope for periods and then deteriorate
Statements such as “lung function is stable” do not always reflect real-world functioning

Understanding the work impact helps clinicians better support patients in consultations, reports, and advocacy.

Implications for systems and policy

This poll reinforces that ABPA carries a significant socioeconomic burden, including:

Reduced workforce participation
Early retirement
Increased reliance on health and social support systems

Any assessment of disability, employment capability, or long-term planning must take into account:

Variability over time
Treatment burden
Side-effects of necessary medications

In summary

This patient poll sends a consistent message:

ABPA commonly limits the ability to work, often leading to reduced hours, unstable employment, or early exit from the workforce.

For patients, this experience is shared and valid.
For clinicians, it is a reminder that ABPA is not just a radiological or immunological diagnosis, but a life-limiting condition with real-world consequences.

by GAtherton

Why do some people cough up long, tube-shaped pieces of mucus?

In several chronic lung conditions, the airways can become inflamed and produce thick mucus.
When this mucus sits in the bronchial tubes, it can sometimes harden into a cast shaped exactly like the airway.

People often describe these casts as:

long, ribbon-like or “snakeskin” pieces
rubbery or stretchy
white, yellow, or green
shaped like the inside of a tube

Coughing one up can feel dramatic but is usually a sign that your lungs are finally able to clear a blockage.

**What does it mean if a cast has black flecks or dark spots?**

This can look alarming, but several common, mostly harmless explanations exist.

1. Old or dried blood

Tiny amounts of bleeding from irritated airways can dry and turn:

red → brown → black

This often appears as tiny black dots or threads.

2. Inhaled particles

Dust, soot, pollution, or smoke can get trapped in mucus deeper in the lungs and show up as dark specks.

3. Debris from infection or inflammation

Long-standing inflammation can cause:

darkened mucus fragments
tiny bits of fungal, bacterial or biofilm material
oxidised (darkened) mucus layers

These often look like pepper-like flecks and are not dangerous on their own.

4. Oxidation or ageing of thick mucus

When mucus sits for a long time before it is coughed out, it can become darker in spots.

**When this is usually not worrying**

Black flecks are often harmless when:

the amount is small
the colour change is occasional
you feel better after coughing the cast out
there is no new increase in blood, fever, or breathlessness
this fits your usual pattern of mucus plugging

Most people with chronic airway disease experience occasional colour changes in mucus.

When to mention it to your doctor

You should let your team know if:

black flecks keep appearing repeatedly
you cough up more blood than usual
your breathing worsens suddenly
your sputum smells different
you have fever or chest pain
casts become bigger, more frequent, or harder to clear

These changes do not always mean something serious, but they are worth checking.

Why do casts form in the first place?

Conditions that can cause airway casts include:

Bronchiectasis
ABPA (Allergic Bronchopulmonary Aspergillosis)
Severe or eosinophilic asthma
Chronic infections, including fungal or bacterial
COPD with mucus hypersecretion

Inflammation makes mucus thicker, and narrowed airways make it harder to clear.
Over time, mucus can mould itself into the shape of the airway — becoming a cast.

What to do if you cough one up

Stay calm — this often brings relief.
Take note of its colour and size.
Hydrate well to thin mucus.
Continue your usual airway-clearance technique (physio, nebulisers, saline, etc.)
Let your team know if it is unusual for you.

Final reassurance

Coughing up a long, tube-like piece of mucus can feel shocking, but in most cases it simply means your lungs are clearing a blocked area.
Black flecks are usually:

old blood
trapped dust or soot
dried mucus debris

Most of the time, these findings are not dangerous, but they can give useful clues about airway inflammation.

by GAtherton

⭐ Severe Asthma with Fungal Sensitisation (SAFS): The Hidden Burden Behind Difficult Asthma

Estimated prevalence: 15–30% of severe asthma patients show fungal sensitisation.

Severe Asthma with Fungal Sensitisation (SAFS) describes a group of patients with severe asthma who show sensitisation (allergy) to Aspergillus or other environmental moulds but do not meet criteria for ABPA. These patients often experience persistent inflammation, breathlessness, mucus production, and exacerbations that are not adequately controlled by standard asthma therapies.

Although SAFS is common in severe asthma clinics, it remains poorly recognised, frequently mislabelled, and rarely discussed in routine practice. Yet identifying SAFS is crucial because it opens the door to specific interventions — including antifungals or targeted biologics — that can improve symptoms and reduce hospital admissions.

⭐ How Common Is SAFS?

SAFS is more common than ABPA and CPA combined in asthma services.

Population	Estimated prevalence
Moderate asthma	~5%
Severe asthma	15–30%
Patients with frequent exacerbations	up to 40%
ABPA-negative patients with mucus plugging	high likelihood

Across the UK, this represents tens of thousands of people.

⭐ Why SAFS Is Missed

1. The diagnosis is not widely understood

Unlike ABPA or CPA, SAFS lacks:

universally agreed diagnostic criteria
clear imaging features
a single confirmatory test

This leads to variability in thinking and detection.

2. Symptoms mimic uncontrolled asthma

SAFS patients typically experience:

severe breathlessness
wheeze
mucus production
airway plugging
poor response to inhalers
frequent steroid courses

These appear indistinguishable from “difficult” or “type 2–high” asthma.

3. IgE and eosinophils may be normal

Unlike ABPA:

total IgE may be modest
Aspergillus IgE may be borderline
eosinophils may fluctuate, especially with steroids or biologics

Clinicians are often looking for very high IgE levels — but SAFS patients usually don’t show them.

4. Sputum and CT scans appear non-specific

Typical imaging:

mucus plugging
small-airway thickening
variable, patchy inflammation
bronchiectasis may or may not be present

Radiologists often report these changes as:

“consistent with asthma”
“post-infective”
“non-specific inflammatory pattern”

5. The fungal link is overlooked

Many clinicians are unfamiliar with:

the role of mould exposure
sensitisation thresholds
the overlap between environmental allergy and airway disease
when antifungals are appropriate

This leads to delays in recognising fungal-driven asthma.

⭐ Who Is at Highest Risk?

1. Severe asthma patients unresponsive to maximal inhaled treatment

Particularly those with:

frequent exacerbations
nocturnal symptoms
long-term steroid use
persistently low lung function
mucus plugging events

**2. Patients sensitised to Aspergillus or multiple moulds**

Positive skin tests or specific IgE indicate airway allergy that can drive symptoms.

3. Patients with damp or mould exposure at home or work

An important environmental factor often overlooked.

4. ABPA-negative asthma patients with mucus plugging

A large proportion of these patients fit the SAFS profile.

5. Those with co-existing bronchiectasis

Bronchiectasis amplifies the inflammatory response to fungal exposure.

⭐ Specialties That Need Greater Awareness

Severe asthma services & biologics clinics
(primary diagnostic opportunity)
General respiratory clinics
Primary care & urgent care
(patients seen frequently with “persistent asthma symptoms”)
Radiology
(important for identifying mucus plugging)
Allergy/Immunology
(mould sensitisation is central to diagnosis)
Environmental health teams
(exposure to mould and dampness often perpetuates symptoms)

The National Aspergillosis Centre can provide specialist input when diagnosis is unclear or response to treatment is suboptimal.

⭐ Red Flags Suggesting SAFS

1. Severe asthma poorly controlled despite maximal inhalers

Including biologics (omalizumab, mepolizumab, benralizumab, dupilumab, tezepelumab).

**2. Sensitisation to Aspergillus fumigatus or multiple moulds**

3. Repeated mucus plugging episodes

(or “sticky mucus” symptoms)

4. More than 2–3 steroid-treated exacerbations per year

5. Asthma + bronchiectasis

Even mild bronchiectasis increases fungal risk.

6. Symptoms triggered by damp/mould exposure

7. Persistent airway inflammation despite correct inhaler technique

⭐ Misdiagnoses That Delay Recognition

“Difficult asthma”
“Brittle asthma”
“Post-viral inflammation”
“Poor adherence to inhalers”
“Asthma–COPD overlap”
“Psychogenic dyspnoea”
“Recurrent chest infections”

SAFS is a diagnosis hiding in these labels.

⭐ The Cost of Missed SAFS Diagnosis

For patients:

persistent symptoms
steroid dependence
increased risk of ABPA
progressive airway damage
hospital admissions
poor quality of life
possible career and lifestyle impact

For healthcare systems:

repeated A&E visits
asthma admissions
high biologic usage without adequate response
unnecessary antibiotics
escalating steroid toxicity
missed environmental interventions

⭐ Conclusion

SAFS is one of the most common — yet least recognised — fungal-related lung conditions. Although it lacks the dramatic imaging changes of ABPA or CPA, its impact on patients is profound.

Recognising mould sensitisation in severe asthma, understanding the role of fungal allergens, and considering targeted therapies can transform disease control. For complex cases or when the diagnosis is uncertain, referral to the National Aspergillosis Centre is recommended.

Early identification and appropriate treatment reduce steroid use, exacerbations, and long-term airway damage.

by GAtherton

⭐ Aspergillus Bronchitis: The Overlooked Condition Hiding in Plain Sight

Estimated prevalence 1–2% in bronchiectasis and chronic airway disease clinics.

Aspergillus Bronchitis (AB) is a chronic, symptomatic infection of the airways caused by Aspergillus species in people with underlying lung disease. It sits between simple colonisation and chronic pulmonary aspergillosis (CPA), and is frequently overlooked or mislabelled as “recurrent infection,” “post-viral symptoms,” or uncontrolled bronchiectasis.

Unlike CPA, Aspergillus Bronchitis does not require cavities or major structural destruction — which makes it both easier to miss and surprisingly common among people with chronic airway disease.

When recognised and treated (usually with antifungal therapy for several months), symptoms often improve significantly. But because awareness remains low, most patients cycle through unnecessary antibiotics, repeated exacerbations, and worsening airway disease before the real cause is identified.

⭐ What Exactly Is Aspergillus Bronchitis?

Aspergillus Bronchitis is defined by:

chronic productive cough
sputum growing Aspergillus species repeatedly
airway inflammation
symptoms lasting over 3 months
underlying airway disease (bronchiectasis, CF, COPD, prior TB, ABPA)
response to antifungal therapy

Unlike ABPA:

there is no allergic response,
IgE is usually normal,
eosinophils are normal or mildly elevated.

Unlike CPA:

there are no cavities on imaging,
IgG may be normal or only slightly elevated,
disease is confined to the airways, not lung tissue.

This places AB in a “grey zone” — often invisible unless specifically looked for.

⭐ Why Aspergillus Bronchitis Is Missed

1. Symptoms mimic common chronic airway disease

Typical AB symptoms include:

daily productive cough
worsening sputum thickness
breathlessness
fatigue
repeated “chest infections”
slow-to-clear mucus
crackles or wheeze

These resemble:

bronchiectasis exacerbations
COPD flare-ups
chronic infection with Pseudomonas or NTM
post-viral cough
uncontrolled asthma

Without fungal awareness, clinicians default to bacterial explanations.

2. Sputum grows multiple organisms — Aspergillus is dismissed

In bronchiectasis, sputum frequently grows:

Haemophilus
Pseudomonas
Staphylococcus
Streptococcus
NTM

When Aspergillus appears, it’s often labelled:

“colonisation”
“contaminant”
“not clinically relevant”

But repeated isolation with persistent symptoms is highly suggestive of AB.

3. IgE/IgG results may be normal

Many clinicians expect high IgE or IgG to “confirm Aspergillus disease.”
But in Aspergillus Bronchitis:

IgE is usually normal
IgG can be normal or borderline

This leads to false reassurance.

4. Radiology rarely shows overt features

CT scans in AB may show:

mucus plugging
mild bronchial wall thickening
small nodules
progression of bronchiectasis

But they do not show the cavities of CPA or classic features of ABPA.

Therefore radiologists often report scans as “no significant change” or “stable bronchiectasis.”

5. Antibiotics appear to help — temporarily

Patients often improve slightly with:

amoxicillin
doxycycline
macrolides
ciprofloxacin

This gives clinicians the impression of bacterial disease, but symptoms soon return.

6. Lack of awareness

Many specialists (even in respiratory clinics) are unaware that Aspergillus Bronchitis:

exists as a distinct clinical entity
can be disabling
responds to antifungals
predicts progression to CPA if untreated

This leads to significant diagnostic delay.

⭐ Who Is at Highest Risk?

1. Bronchiectasis

The largest risk group.
Aspergillus Bronchitis may account for 1–2% of all bronchiectasis patients, and up to 5–10% in severe or frequent exacerbator groups.

2. Cystic Fibrosis (CF)

These patients frequently grow Aspergillus but not all have ABPA — some have Aspergillus Bronchitis.

3. COPD and chronic productive cough

Especially those with:

frequent mucus plugging
repeated “infective exacerbations”
progressive sputum production

4. Post-TB airway damage

Chronic airway deformity, scarring, and bronchiectasis from old TB predispose to fungal infection.

5. Post-COVID structural disease

A new and growing risk group, especially after prolonged ICU ventilation.

6. ABPA patients

Some patients develop Aspergillus Bronchitis during steroid-dominated treatment or after stopping antifungals.

⭐ Which Specialities Need Greater Awareness?

Respiratory medicine
(especially bronchiectasis clinicians and severe asthma teams)
Infectious Diseases
(frequent respiratory presentations with chronic airway infection)
Radiology
(to recognise subtle but progressive airway changes)
Primary care
(“recurrent chest infection” or “persistent cough” patients)
Physiotherapy & airway clearance teams
(excessive sputum with fungal elements)
Cystic Fibrosis services

The National Aspergillosis Centre is the ideal referral destination when diagnosis is uncertain or symptoms persist despite typical management.

⭐ Red Flags Suggesting Aspergillus Bronchitis

1. Chronic (>3 months) productive cough + repeated Aspergillus in sputum

Even 2 positive sputums in the right clinical context should raise suspicion.

2. Bronchiectasis patient not improving on repeated antibiotics

3. Thick, tenacious mucus with black, grey, or brown plugs

4. Worsening CT bronchiectasis or mucus plugging

5. Absence of features typical for ABPA (normal IgE, no fleeting infiltrates)

6. Asthma or COPD patient with new persistent sputum

7. Partial response to antibiotics but rapid relapse

8. Unexplained fatigue and breathlessness in someone with airway disease

⭐ The Cost of Missed Aspergillus Bronchitis

If AB is not recognised early, consequences include:

repeated exacerbations
accelerating bronchiectasis
long-term airway damage
chronic inflammation
steroid overuse
unnecessary antibiotics
repeated hospitalisations
progression to CPA in some patients

For health systems, missed diagnosis leads to:

higher admission rates
inappropriate long-term antibiotic use
avoidable CT scans and investigations
greater long-term burden of CPA

But antifungal therapy — when appropriately used — can offer marked symptom improvement and reduce exacerbation frequency.

⭐ Conclusion

Aspergillus Bronchitis is a distinct, treatable form of chronic airway disease seen in people with bronchiectasis, asthma, COPD, CF, and post-TB lung damage. Yet lack of awareness means many patients are repeatedly misdiagnosed with bacterial infections or unexplained chronic cough.

Recognising red flags, reviewing sputum results carefully, and considering antifungal therapy can dramatically improve outcomes. Early referral to specialist centres such as the National Aspergillosis Centre is recommended for complex cases or uncertain diagnosis.

Early identification prevents airway deterioration — and reduces the likelihood of progression to CPA.

by GAtherton

⭐ Allergic Bronchopulmonary Aspergillosis (ABPA): Why Diagnosis Is Missed and Who Needs to Be More Aware

With estimated prevalence of 1–2% in asthma clinics and up to 10% in severe asthma services.

Allergic Bronchopulmonary Aspergillosis (ABPA) is a chronic immune reaction to Aspergillus that affects people with asthma or cystic fibrosis. It causes airway inflammation, mucus plugging, recurrent exacerbations, and bronchiectasis if untreated.

Despite being treatable, ABPA remains heavily underdiagnosed, even in countries with advanced respiratory services. Many people are told for years that they have “difficult asthma” or “recurrent chest infections,” only for ABPA to be diagnosed much later, often with significant lung damage already present.

The UK National Aspergillosis Centre (NAC) provides specialist expertise, yet only a small proportion of expected ABPA cases reach specialist review.

This article explains why ABPA is missed, which patients are at risk, which specialities need to be more alert, and the red flags that should prompt testing or referral.

⭐ How Common Is ABPA?

ABPA is more common than most clinicians realise:

Population	Estimated prevalence
General asthma	1–2%
Severe asthma clinics	3–10%
Cystic fibrosis	5–15%
Bronchiectasis (non-CF)	1–4%

Across the UK, this equates to an estimated 15,000–25,000 people living with ABPA — but only a small minority ever receive the correct diagnosis.

⭐ Why ABPA Is Often Missed

1. ABPA looks like “difficult asthma”

Typical symptoms — wheeze, cough, mucus, breathlessness — mimic:

severe asthma
eosinophilic asthma
uncontrolled asthma despite treatment

Patients may be repeatedly stepped up through inhalers, oral steroids, and biologics before ABPA is even considered.

2. Exacerbations are mistaken for infections

Many ABPA flare-ups are treated as:

pneumonia
viral infection
“chest infection”
post-viral asthma worsening

Without fungal-specific thinking, the diagnosis is rarely made.

3. IgE and eosinophils fluctuate

IgE is a cornerstone of ABPA diagnosis, but:

systemic steroids suppress IgE
biologics (benralizumab, mepolizumab, dupilumab) reduce eosinophils
flare-ups produce temporary spikes
baseline ranges vary between labs

Clinicians often overlook ABPA in patients on biologics because eosinophils are normal — despite the underlying fungal allergy still being active.

4. Radiology findings get mislabelled

ABPA causes:

mucus plugging
“tram lines” and bronchial thickening
fleeting infiltrates
upper lobe bronchiectasis

These are often:

labelled “infection”
attributed to asthma airway remodelling
not compared across time
missed on CT unless specifically looked for

5. Inconsistent awareness across specialities

Some clinicians are unfamiliar with:

ISHAM diagnostic criteria
interpreting IgE/IgG results
the relationship between asthma and fungal allergy
the overlap between ABPA and bronchiectasis

This leads to diagnostic delay or misdiagnosis.

6. ABPA evolves into chronic disease if untreated

Repeated inflammation → mucus plugging → bronchiectasis → fibrosis.
By the time a diagnosis is made, airway damage can be permanent.

⭐ Who Is at Highest Risk?

1. Asthma patients with repeated exacerbations

Especially those who:

fail maximal inhaler therapy
require multiple steroid courses
have sudden, dramatic mucus plugging events
experience episodic “flares” with no clear cause

2. Severe asthma clinic patients

Prevalence is up to 10%, especially those with:

high IgE
eosinophilia
sensitisation to multiple allergens
steroid dependence

3. Bronchiectasis patients

Bronchiectasis often coexists with ABPA and can worsen flares.

4. Patients with mucus plugging (“finger-in-glove” signs)

These striking CT appearances strongly suggest ABPA but are often misattributed to infection.

5. People with CF (Cystic Fibrosis)

5–15% develop ABPA at some stage.

⭐ Which Specialities Need Greater Awareness?

Severe asthma services & biologics clinics
(highest yield group for ABPA detection)
Respiratory medicine
(diagnosis often falls here but is highly variable)
General practice
(sees frequent “exacerbations”)
Emergency departments & acute medical units
(manage acute mucus plugging, chest tightness)
Paediatric respiratory medicine
(early recognition prevents chronic damage)
Cystic Fibrosis services
Radiology
(fleeting infiltrates and mucus plugging often give the earliest clues)

The National Aspergillosis Centre should be the referral point for complex or uncertain cases.

⭐ Red Flags Suggesting ABPA

1. Asthma with repeated, unexplained exacerbations

Especially if poorly responsive to normal treatment.

2. High total IgE (>500–1000 IU/mL)

Or rising IgE over time.

3. Eosinophilia (unless suppressed by treatment)

4. Positive Aspergillus sensitisation

(Skin prick test or specific IgE)

5. Bronchiectasis, particularly central or upper lobe

6. Fleeting pulmonary infiltrates

7. Mucus plugging on CT (“finger-in-glove” appearance)

8. ABPA flare triggered by stopping antifungals

9. Asthma + Aspergillus in sputum

⭐ The Cost of Missed ABPA Diagnosis

Failure to diagnose ABPA leads to:

progressive airway damage
permanent bronchiectasis
steroid dependence
hospital admissions
repeated infections
respiratory failure in advanced stages
reduced quality of life
avoidable healthcare expenditure

Delayed diagnosis increases the risk of progression to CPA, a far more serious chronic fungal infection requiring long-term antifungal therapy.

Early recognition, correct treatment, and referral to specialist centres like the National Aspergillosis Centre dramatically improve long-term outcomes.

⭐ Conclusion

ABPA is not rare — especially within severe asthma clinics, bronchiectasis services, and CF units. Yet it remains significantly underdiagnosed because its symptoms mirror those of common respiratory conditions, and because key investigations like IgE, IgG, and CT interpretation are inconsistently used.

A structured approach — recognising red flags, performing appropriate testing, and referring complex cases to the National Aspergillosis Centre — can reduce the burden of avoidable airway damage and improve the lives of thousands of patients.

by GAtherton

⭐ Chronic Pulmonary Aspergillosis: Why Diagnosis Is Missed and Who Needs to Be More Aware

With estimated prevalence of 3–4 cases per 100,000 population, and far higher rates in high-risk groups.

Chronic Pulmonary Aspergillosis (CPA) is a slowly progressive fungal lung disease affecting an estimated 3–4 per 100,000 people in the UK, with higher estimates in global settings with greater TB prevalence. Despite this, many clinicians will go through entire careers without confidently recognising it — not because it is extremely rare, but because it almost always hides inside other long-term lung diseases.

The UK is unusual in having a nationally commissioned specialist service — the National Aspergillosis Centre (NAC), based at Wythenshawe Hospital, Manchester — offering funded diagnostics, multidisciplinary review, and long-term antifungal management. But only a fraction of expected CPA cases are ever referred. Most are simply never diagnosed.

This article explains why diagnoses are missed, who is at highest risk, which specialities need to be more alert, and the red flags that should trigger testing or referral.

⭐ How Common Is CPA? The Numbers Behind the Problem

The UK prevalence is estimated at 3–4 per 100,000 people — approximately 2,000–2,500 people with CPA at any given time.

But the risk is far higher in specific groups:

Risk Group	Estimated CPA prevalence
Post-TB lung disease	6–10% in those with residual cavities
Severe COPD (GOLD III–IV)	1–3%
Bronchiectasis	1–3%
NTM disease	3–10%
Sarcoidosis with fibrosis	1–2%
Immunosuppression (steroids/biologics)	Unknown, but rising

Using these figures, the true UK caseload could exceed 4,000–6,000 individuals, yet NAC receives ~500–1,000 referrals, highlighting a large diagnostic gap.

⭐ Why CPA Is So Often Missed

1. Symptoms mimic common chronic lung diseases

CPA presents with:

Persistent cough
Breathlessness
Fatigue
Weight loss
Recurrent “chest infections”
Haemoptysis

These overlap almost perfectly with:

COPD
bronchiectasis
post-TB changes
long COVID
NTM infection
repeatedly “slow to clear” pneumonia

Because symptoms are non-specific, clinicians rarely think fungal.

2. Interpretation of imaging is inconsistent

CPA shows:

one or more cavities
pleural thickening
nodules
progressive changes over months
fungal balls

Common reporting pitfalls:

labelled “post-infective scarring”
misinterpreted as malignancy
seen but not compared longitudinally
incidental CT findings not acted upon

Radiology is one of the biggest missed opportunities for early detection.

3. IgG testing is not routinely requested

Aspergillus IgG is the key diagnostic biomarker — but it is:

often confused with IgE
not available in some hospitals
omitted from workups for recurrent infection
unfamiliar to non-respiratory clinicians

Without IgG, CPA is rarely diagnosed.

4. Short-term improvement with antibiotics is misleading

Patients with CPA may temporarily feel better after:

broad-spectrum antibiotics
steroids
physiotherapy

This transient improvement creates false reassurance.

5. CPA spans multiple specialisms — and no one owns it

Diagnosis requires combined expertise across:

respiratory medicine
infectious diseases
radiology
microbiology
immunology

When no one speciality takes responsibility, patients get lost.

⭐ Which Patients Are at High Risk?

CPA almost always develops on a background of existing lung damage.

1. Post-TB lung disease (PTLD)

Globally the largest CPA population.
Residual cavities are the strongest predictor.

Specialities needing awareness:

TB teams
ID physicians
Radiologists
Community TB nurses
Public health TB programmes

2. COPD (especially severe / emphysema)

Millions of people are potentially at risk.
Recurrent infections + bullae/cavities = fertile ground for CPA.

Specialities:

COPD clinics
Pulmonary rehab
Acute medicine (frequent admissions)

3. Bronchiectasis

Damaged airways enable persistent Aspergillus colonisation and inflammation.

Specialities:

Bronchiectasis MDTs
Severe asthma & NTM clinics
Respiratory physiotherapy

4. Sarcoidosis and ILD

Fibrosis and traction bronchiectasis develop cavities over time.

5. Post-COVID or post-influenza structural disease

Emerging risk group, especially in patients with:

ventilatory lung injury
persistent CT abnormalities
chronic steroid exposure

6. Chronic steroid or immunomodulator use

While invasive aspergillosis is linked to profound immunosuppression, CPA often affects those with milder, chronic immune dysfunction:

systemic steroids
high-dose inhaled steroids
biologics affecting eosinophils
poorly controlled diabetes
chronic kidney disease
malnutrition

⭐ Which Specialities Need to Be More Alert?

Respiratory Medicine – primary detection, but awareness varies greatly
Infectious Diseases – especially post-TB and persistent infection clinics
Radiology – key to spotting early changes
Primary Care – sees patients repeatedly with “ongoing chest infections”
Emergency & acute medicine – haemoptysis presentations
Bronchiectasis and NTM services – strong overlap
Severe asthma and biologics teams – ABPA → CPA evolution
TB clinics – highest prevalence globally, often least recognised

The National Aspergillosis Centre should be the referral point for any complex or uncertain case.

⭐ Red Flags: When to Suspect CPA

1. Cavities on CT (thin-, thick-walled, evolving, or multiple)

Especially with pleural thickening.

2. Haemoptysis

CPA is one of the most common causes of haemoptysis in people with cavities.

3. Symptoms lasting >3 months

Chronic cough, fatigue, weight loss, breathlessness.

4. “Recurrent infections” that never fully resolve

5. Post-TB patient with any new or worsening symptoms

6. Bronchiectasis patient with new cavity or Aspergillus culture

7. High or rising Aspergillus IgG

8. ABPA patient who deteriorates off antifungals

⭐ The Cost of Missed Diagnoses

When CPA is not recognised early, the consequences are severe:

irreversible lung damage
repeated hospitalisations
emergency haemoptysis events
prolonged antifungal therapy with more toxicity
reduced quality of life
avoidable deaths

For systems like the NHS, late diagnosis increases costs:

unplanned admissions
repeated CT imaging
prolonged antibiotics
intensive care during haemoptysis
complex surgery (lobectomy/pneumonectomy)

Early referral to specialist centres like the National Aspergillosis Centre prevents many of these harms.

⭐ Conclusion

CPA is not rare within the populations most likely to develop it.
Missed diagnoses are common, predictable, and preventable.

By increasing awareness across Respiratory, Infectious Diseases, Radiology, Primary Care, TB services, and severe asthma pathways — and by using simple tools such as Aspergillus IgG and careful CT interpretation — clinicians can dramatically reduce the diagnostic delay that damages lungs, quality of life, and survival.

The UK is fortunate to have the National Aspergillosis Centre as a nationally commissioned referral service. Recognising CPA early and referring appropriately has the power to save lives, reduce system costs, and improve long-term outcomes.

by GAtherton

🌿 Biologics when ABPA and CPA overlap: What Patients Need to Know

Understanding how they work, when they’re helpful, and when extra care is needed

Biologic medicines (such as omalizumab, mepolizumab, benralizumab, dupilumab and newer options like tezepelumab) are increasingly used to treat Allergic Bronchopulmonary Aspergillosis (ABPA) and severe asthma. They can be life-changing for some people.

However, their place in Chronic Pulmonary Aspergillosis (CPA) — especially in people who have both ABPA and CPA together — is more complicated and needs careful specialist supervision.

This article explains what we know so far.

🌟 1. ABPA and CPA are different conditions — but some people have both

ABPA is mainly an allergic reaction to Aspergillus in the airways.
CPA is a chronic fungal infection that causes cavities, scarring, and long-term lung damage.
Some people start with ABPA and later develop CPA, or the two conditions overlap.
The 2024 international ABPA guidelines now recognise this overlap as real and important.

Because biologics target allergy pathways rather than fungal infection, treatment decisions must look at both sides of the disease.

🌿 2. Biologics in ABPA: the evidence is strong and growing

Biologics can help patients with ABPA or severe asthma by:

reducing steroid use
improving breathing
decreasing mucus plugging
lowering flare-ups
improving quality of life

Biologics most commonly used in ABPA include:

Biologic	Target	Notes
Omalizumab	IgE	Well established, helps many ABPA patients
Mepolizumab	IL-5	Helps eosinophilic inflammation
Benralizumab	IL-5Rα	Similar to mepolizumab; long-acting
Dupilumab	IL-4Rα	Very promising for allergic disease; growing evidence for ABPA
Tezepelumab	TSLP	Very new; limited ABPA data so far

For many people with ABPA, biologics are safe and effective when monitored.

⚠️ 3. Biologics and CPA: much less evidence

CPA is caused by persistent fungal infection and structural lung damage.
Biologics do not treat fungal infection, and they do not prevent cavities.
In CPA, the mainstay of treatment is still:
- antifungal medication (usually itraconazole, voriconazole or posaconazole)
- careful imaging (CT scans)
- airway clearance
- sometimes surgery or bronchoscopy

There is no strong evidence that biologics help CPA itself.

🔄 **4. What about patients who have both ABPA and CPA?**

This is where things become more complex.

Biologics may help the allergic part (ABPA), but:

they do not treat fungal infection
they do not stop fungal cavities from progressing
they may reduce inflammation that normally helps the body contain infection

If antifungal treatment is interrupted or not strong enough, fungal activity may increase while the allergic symptoms improve — so regular monitoring is essential.

Specialist centres (like the NAC) now emphasise:

✔️ Continue antifungals if CPA is active
✔️ Watch cavities with regular CT scans
✔️ Monitor Aspergillus IgG/IgE and fungal cultures
✔️ Check whether symptoms are from allergy, infection, or both
✔️ Make joint plans between asthma/airway doctors and mycology specialists

❓ 5. Are some biologics better than others for ABPA/CPA overlap?

There is no official guidance yet, but early observations suggest:

⭐ Most promising for ABPA:

Dupilumab seems particularly effective for allergic disease (IgE, mucus, airflow), though still off-label for ABPA.

⭐ Increasing interest:

Tezepelumab works outside the eosinophil pathway and may be useful in some asthma types, but research in ABPA is only just starting.

⭐ Useful in selected cases:

Anti-IL-5 biologics (mepolizumab, benralizumab) help airway eosinophils but may not help every ABPA patient.

⚠️ Uncertain in CPA:

None of the biologics treat fungal infection or cavities directly.
Their role in active CPA remains unclear and requires careful oversight.

🧭 6. What this means for patients

If you have ABPA only, biologics may be an excellent option — especially if:

steroids cause side-effects
your asthma is uncontrolled
you have frequent flare-ups
your IgE levels are very high
mucus plugging or wheezing continues despite treatment

If you have CPA or cavities, treatment needs to be more cautious:

antifungal medication usually needs to continue
biologics may still help if the allergic component is significant
CT scans must be repeated to make sure cavities are not progressing
specialists must weigh benefits vs. risk for each patient individually

💬 7. Summary

Biologics can be extremely helpful for ABPA.
They do not treat CPA, and cannot replace antifungal medicines.
In patients with both ABPA and CPA, the approach must be personalised.
Dupilumab and (possibly) tezepelumab are emerging biologics with promise, but evidence is still developing.
Decisions should always be made with a specialist centre such as the National Aspergillosis Centre (NAC).

by GAtherton

Understanding Mucous Casts in Allergic Bronchopulmonary Aspergillosis (ABPA)

People living with Allergic Bronchopulmonary Aspergillosis (ABPA) often notice thick, unusual mucus coming up during a flare. Some of this mucus can look very different from “normal” sputum and may be described as mucous casts. This leaflet explains what they are, why they happen, and what they mean for your ABPA.

⭐ What are mucous casts?

A mucous cast is a thick, sticky plug of mucus that forms inside your airways.
It takes on the exact shape of the airway or branch it was sitting in – a bit like a soft mould of the inside of your lungs.

When coughed up, casts may look:

long and tube-shaped
soft and rubbery
curled or C-shaped
occasionally branching, like a twig
pale yellow/cream with darker specks

These darker flecks can include dead inflammatory cells, airway debris, and sometimes tiny amounts of fungal material trapped inside.

⭐ Why do they happen in ABPA?

ABPA is not an infection, but an allergic over-reaction to the Aspergillus fungus.
This allergic inflammation causes:

1. Excess mucus production

Your airways create far more mucus than usual.

2. Thicker, stickier mucus

Inflammation changes the chemistry of the mucus, making it harder to clear.

3. Swollen, narrowed airways

This makes it easy for mucus to get stuck and form plugs.

4. Trapped material

Casts can contain:

fungal spores
inflammatory cells
dust or other inhaled particles
old blood or tissue debris

All of this can glue together into a cast.

⭐ Are mucous casts harmful?

They are not dangerous on their own, but they can cause problems:

Airway blockage → breathlessness, wheeze, sudden tightness
Chest infections → trapped mucus is an ideal place for bacteria
ABPA flare-ups → casts often appear during periods of high inflammation
Reduced airflow on CT scans → seen as “bronchial impaction”

Telling your clinical team when you notice casts helps them judge how active your ABPA is.

⭐ What do mucous casts look like in ABPA?

Patients often describe:

“noodles”
“worms”
“rubbery plugs”
“little branches”
“specks of brown/black” within pale mucus

These appearances are normal in ABPA and do not mean your lungs are permanently worsening.

⭐ How are mucous casts managed?

1. Airway clearance

This is the most important step. Techniques include:

huff-coughing
active cycle of breathing
nebulised saline (hypertonic or isotonic)
flutter/PEP devices (Acapella, Aerobika)
chest physiotherapy

These help loosen and move mucus from deeper airways.

2. Medication

Depending on your treatment plan:

inhalers (bronchodilator + inhaled steroids)
biologics (e.g., mepolizumab, dupilumab, omalizumab)
antifungal medication if prescribed as part of your ABPA care
oral steroids if medically appropriate

Biologics can reduce the inflammation that causes casts, so many patients notice fewer plugs over time.

3. Monitoring

Your team may keep an eye on:

sputum samples
IgE levels
CT scan changes
symptom patterns

⭐ When should I tell my team?

Contact your clinical team if you notice:

more frequent mucous casts
sudden breathlessness or chest tightness
a drop in your usual oxygen saturation
fever or signs of infection
coughing up blood
a change in colour or smell of mucus

⭐ Reassurance

Mucous casts are very common in ABPA.
They can look alarming, but they are simply a sign that your airways are inflamed and producing thick mucus.

Coughing them out is helpful, not harmful.
It allows the affected airway to reopen and can rapidly improve breathing.

✅ Further Reading

For more patient-oriented information, you can visit the AFIT website where the term “casts” is discussed in the context of aspergillosis: Aspergillus.org.uk – search “casts”.

by GAtherton