Allergic Bronchopulmonary Aspergillosis (ABPA) for Expert Patients and non-Specialist Clinicians

Expert Information for Patients, GPs, and Specialist Nurses

🔎 What Is ABPA?

ABPA is a complex hypersensitivity reaction to Aspergillus fumigatus colonising the airways. It is not a fungal infection in the classic sense, but rather an exaggerated immune response — particularly involving IgE and eosinophils — seen in people with asthma or cystic fibrosis (CF).

It leads to recurrent inflammation, mucus plugging, and bronchial damage (including central bronchiectasis) if untreated.

🧬 Disease Mechanism

  • Type I and III hypersensitivity to A. fumigatus

  • Chronic airway inflammation causes mucus impaction and permanent lung damage

  • Associated with elevated total and specific IgE, eosinophilia, and recurrent flares

👥 Who Is at Risk?

  • Moderate to severe asthma

  • Cystic fibrosis

  • Rarely, patients with bronchiectasis or other chronic airway disease

ABPA is often underdiagnosed, especially in adults with difficult-to-control asthma.

⚠️ Common Symptoms

  • Worsening asthma control

  • Wheeze and chest tightness

  • Cough with thick mucus plugs

  • Shortness of breath

  • Intermittent low-grade fever

  • Haemoptysis (less common, usually mild)

  • Fatigue and poor response to inhaled steroids alone

🧪 Diagnosis

Diagnosis is based on a combination of clinical, radiological, and immunological features.

Core Investigations:

  1. Total IgE ≥1000 IU/mL (or >500 in treated patients)

  2. Aspergillus-specific IgE positive

  3. Aspergillus-specific IgG (or precipitating antibodies)

  4. Blood eosinophilia (>0.5 x10⁹/L typically)

  5. Chest CT: central bronchiectasis, mucus plugging (“finger-in-glove”), fleeting infiltrates

  6. Sputum culture or PCR positive for A. fumigatus

Diagnostic Criteria:

Use updated ISHAM criteria (2024 version preferred) combining major and minor features.

💊 Treatment

First-Line:

  • Oral corticosteroids (e.g. prednisolone) – cornerstone of flare management

    • Typically tapered over 3–6 months

Adjunct:

  • Itraconazole or posaconazole – reduces antigen burden and steroid need

    • 3–6 months or longer; monitor liver function and drug levels

Steroid-Sparing Options:

  • Biologics (increasingly used, especially in steroid-dependent or relapsing patients):

    • Omalizumab (anti-IgE)

    • Mepolizumab, Benralizumab (anti-IL-5)

    • Dupilumab, Tezepelumab (emerging options)

🧾 Monitoring

  • Total IgE every 1–3 months (a 25–50% rise may indicate relapse)

  • Pulmonary function tests (FEV1, peak flow)

  • Repeat CT if clinical deterioration or poor steroid response

  • Sputum cultures in persistent symptoms (to exclude Aspergillus bronchitis)

⚠️ Complications

  • Progression to bronchiectasis

  • CPA (if antifungals are stopped prematurely in chronic cases)

  • Recurrent flares leading to irreversible damage

  • Steroid side effects (weight gain, osteoporosis, adrenal suppression)

📚 More Information

  • Specialist referral: patients should be considered for referral to the National Aspergillosis Centre (NAC) or local respiratory immunology team for persistent/recurrent ABPA.

  • Patient support: aspergillosis.org, CF Trust, Asthma + Lung UK

  • Key guidelines: Guidance

by GAtherton

Aspergillus Tracheobronchitis (ATB) for Expert patients and non-Specialist Clinicians

Expert Information for Patients, GPs, and Specialist Nurses

🔎 What Is Aspergillus Tracheobronchitis?

Aspergillus tracheobronchitis (ATB) is a rare but serious form of airway-invasive aspergillosis that primarily affects the trachea and large bronchi, rather than the lung parenchyma. It occurs predominantly in immunocompromised patients and may present with obstructive airway symptoms or respiratory failure.

ATB can exist on a spectrum from superficial colonisation to ulcerative or pseudomembranous invasion of the bronchial wall.

🧬 Pathophysiology

  • Inhaled Aspergillus spores adhere to and invade damaged airway mucosa.

  • Occurs more commonly when local airway immunity is impaired (e.g. in transplant recipients or critical illness).

  • May co-exist with invasive pulmonary aspergillosis (IPA) or appear in isolation.

👥 Who Is at Risk?

High-risk groups include:

  • Lung transplant recipients

  • Hematopoietic stem cell transplant patients

  • Severe COPD or structural airway disease

  • Patients with prolonged corticosteroid use

  • Critically ill or mechanically ventilated patients

  • COVID-19 or influenza patients (sometimes overlapping with CAPA/IAPA)

⚠️ Clinical Presentation

Symptoms depend on the degree of airway obstruction and depth of invasion:

  • Cough (dry or productive)

  • Worsening breathlessness

  • Stridor or wheeze

  • Hoarseness or vocal changes

  • Fever unresponsive to antibiotics

  • Haemoptysis (may be life-threatening)

  • Airway obstruction or collapse in advanced cases

ATB may be mistaken for tracheobronchial malignancy, infection, or stenosis.

🧪 Diagnosis

Bronchoscopy is essential for diagnosis:

  • Direct visualisation of:

    • Ulceration

    • Pseudomembranes

    • Plaques

    • Necrotic debris

  • Biopsies may reveal fungal hyphae invading mucosa.

Microbiological Investigations:

  • Culture and PCR for Aspergillus from BAL or brushings

  • BAL galactomannan

  • Serum galactomannan or β-D-glucan may be supportive

  • CT chest may be normal or show airway thickening, bronchial wall invasion, or tree-in-bud opacities

💊 Treatment

Systemic Antifungals:

  • Voriconazole is first-line

  • Isavuconazole or liposomal amphotericin B if azole intolerant or resistant

Airway Management:

  • Debridement or bronchoscopic removal of pseudomembranes in severe obstruction

  • Airway stenting in refractory strictures

  • Nebulised antifungals (e.g. amphotericin B) may be used as adjunct in selected cases

Prompt initiation of antifungal therapy is vital. Delays can lead to respiratory failure or death.

🧾 Monitoring

  • Clinical response: breathlessness, cough, fever

  • Repeat bronchoscopy in some cases

  • CT imaging of airways

  • Antifungal drug levels

  • Liver and renal function

📚 More Information

  • ATB is under-recognised, especially in non-neutropenic or critically ill patients.

  • Should be considered in transplant recipients or ICU patients with persistent respiratory symptoms and negative bacterial cultures.

  • Referral to respiratory, infectious diseases, and ICU teams is essential.

  • Resources: aspergillosis.org ; BTS Statement on  aspergillosis

by GAtherton

Aspergillus Bronchitis for Expert Patients and non-Specialist Clinicians

Expert Information for Patients, GPs, and Specialist Nurses

🔎 What Is Aspergillus Bronchitis?

Aspergillus bronchitis is a chronic fungal infection of the airways by Aspergillus fumigatus (or rarely other Aspergillus species), seen in individuals with structural lung disease or impaired mucociliary clearance. Unlike ABPA, it is not allergic in origin and does not involve systemic invasion, but is characterised by persistent fungal colonisation with active infection.

🧬 Pathophysiology

  • Chronic colonisation of the conducting airways by Aspergillus

  • Local immune dysfunction (but not systemic immunosuppression)

  • Low-grade inflammation and increased mucus production

  • Often coexists with bronchiectasis, COPD, or CF

👥 Who Is at Risk?

Most commonly seen in patients with:

  • Bronchiectasis (non-ABPA)

  • Cystic fibrosis

  • COPD or asthma with sputum production

  • Post-viral or structural airway damage

  • Chronic antibiotic or corticosteroid use

Not typically seen in severely immunocompromised hosts (in whom invasive aspergillosis is more likely).

⚠️ Common Symptoms

  • Persistent productive cough

  • Thick sputum often yellow or green

  • Worsening breathlessness or wheeze

  • Chronic sputum positivity for Aspergillus

  • Mild fever or malaise (but often afebrile)

  • Poor response to antibiotics alone

Symptoms may resemble chronic bacterial bronchitis or overlap with infective exacerbations of bronchiectasis.

🧪 Diagnosis

Diagnosis requires a combination of clinical and microbiological evidence, with exclusion of ABPA and CPA.

Diagnostic Features:

  1. Chronic productive cough (>4 weeks)

  2. Repeated isolation of Aspergillus from sputum or BAL

  3. Elevated Aspergillus IgG (typically present)

  4. Normal or mildly elevated total IgE (typically <1000 IU/mL)

  5. Absence of cavitary lesions or ABPA features on CT

  6. Response to antifungal treatment supports diagnosis

🛑 Exclude:

  • ABPA (IgE >1000, eosinophilia, central bronchiectasis)

  • CPA (cavities, weight loss, radiological progression)

💊 Treatment

First-Line:

  • Oral antifungals (usually for 3–6 months)

    • Itraconazole (first choice)

    • Voriconazole or posaconazole (if resistant/intolerant)

  • Monitor drug levels and LFTs

Adjuncts:

  • Physiotherapy and airway clearance techniques

  • Nebulised saline or mucolytics

  • Treat co-infections (e.g. Pseudomonas) where relevant

In patients with CF, consider co-management with a specialist CF team.

🧾 Monitoring

  • Sputum cultures to monitor persistence or clearance

  • Aspergillus IgG levels

  • Symptoms (sputum, breathlessness)

  • Liver function and drug monitoring

  • Periodic CT imaging if symptoms worsen or haemoptysis occurs

📚 More Information

by GAtherton

Aspergillus Sinusitis for Expert Patients and non-Specialist Clinicians

Expert Information for Patients, GPs, and Specialist Nurses

🔎 What Is Aspergillus Sinusitis?

Aspergillus sinusitis refers to fungal involvement of the paranasal sinuses by Aspergillus species, especially A. fumigatus. It spans a spectrum from benign colonisation to destructive invasive disease, depending on the host’s immune status.

There are four main clinical forms, with distinct presentations and treatment approaches.

🧬 Main Forms

Type Description Typical Host
Allergic Fungal Rhinosinusitis (AFRS) A hypersensitivity reaction with nasal polyps and allergic mucin Atopic patients (often young adults)
Fungal Ball (Mycetoma) A dense fungal plug within a sinus cavity, non-invasive Immunocompetent individuals
Chronic Invasive Fungal Sinusitis Slowly progressive mucosal and bony invasion Diabetics, immunosuppressed
Acute Invasive Fungal Sinusitis Rapidly destructive, vascular invasion, necrosis Severely immunocompromised (e.g. neutropenic, transplant recipients)

👥 Who Is at Risk?

Depends on form:

🟩 AFRS:

  • Asthma, eczema, allergic rhinitis

  • Nasal polyps

  • Fungal IgE sensitisation (esp. Aspergillus)

🟨 Fungal Ball:

  • Older adults

  • Dental work (esp. upper molars with root involvement)

  • Chronic sinus blockage or prior surgery

🟧 Chronic Invasive:

  • Long-term corticosteroid or immunosuppressive use

  • Poorly controlled diabetes

🟥 Acute Invasive:

  • Haematological malignancies

  • Bone marrow/stem cell transplant

  • Neutropenia or severe COVID-19

⚠️ Clinical Features

Symptom Common To
Nasal congestion, discharge All forms
Facial pain or pressure All forms
Nasal polyps AFRS
Foul smell or thick mucus Fungal ball
Eye pain, proptosis, visual changes Invasive forms
Fever, systemic illness Invasive forms
Black eschar or necrosis Acute invasive sinusitis (medical emergency)

🧪 Diagnosis

Initial Evaluation:

  • Nasal endoscopy: mucosal thickening, polyps, or black necrosis

  • CT scan: sinus opacification, bone erosion, hyperdense lesions

  • MRI: assesses orbital or intracranial extension in invasive cases

Microbiology & Histopathology:

  • Direct microscopy or fungal stain (e.g. GMS)

  • Culture for Aspergillus spp.

  • Aspergillus-specific IgE/IgG in AFRS

  • Tissue biopsy is essential in invasive disease

💊 Treatment

🟩 AFRS:

  • Functional endoscopic sinus surgery (FESS) to clear sinuses

  • Oral and topical corticosteroids

  • Antifungals (controversial; may reduce recurrence)

  • Allergen immunotherapy in selected cases

🟨 Fungal Ball:

  • Surgical removal only (FESS)

  • No systemic antifungal needed unless complications arise

🟧 Chronic Invasive:

  • Surgical debridement

  • Long-term oral antifungals (e.g. voriconazole, posaconazole)

  • Monitor drug levels and imaging

🟥 Acute Invasive:

  • Urgent surgical debridement

  • High-dose IV antifungals (voriconazole or liposomal amphotericin B)

  • Reversal of immunosuppression

  • High mortality if delayed — requires ICU and ID team coordination

🧾 Monitoring

  • Repeat imaging for resolution (especially invasive forms)

  • Symptom scores for AFRS and post-FESS recovery

  • Antifungal levels and LFTs if systemic therapy used

  • Endoscopic surveillance in high-risk or relapsing patients

📚 More Information

by GAtherton

Chronic Pulmonary Aspergillosis (CPA) for Expert Patients and Non-Specialist Clinicians

Expert Information for Patients, GPs, and Specialist Nurses

🔎 What Is CPA?

Chronic Pulmonary Aspergillosis (CPA) is a long-term fungal lung infection caused by Aspergillus, typically A. fumigatus. It occurs in individuals with underlying lung damage and can progress slowly over months to years. It includes several subtypes ranging from cavitary lesions to fibrosing disease and fungal balls (aspergillomas).

🧬 Subtypes of CPA

Subtype Description
Simple aspergilloma Fungal ball within a pre-existing lung cavity
Chronic cavitary pulmonary aspergillosis (CCPA) Multiple cavities ± fungal balls; progressive
Chronic fibrosing pulmonary aspergillosis Advanced form with fibrosis and volume loss
Subacute invasive aspergillosis (SAIA) Intermediate between CPA and invasive disease; more rapid progression over weeks to months
Aspergillus nodules Discrete nodules without cavitation; may mimic malignancy

👥 Who Is at Risk?

CPA typically affects people with pre-existing lung disease or immune dysfunction, including:

  • Tuberculosis (old or active)

  • COPD and emphysema

  • Bronchiectasis

  • Sarcoidosis

  • Prior pneumothorax

  • Lung cancer or surgery

  • Diabetes mellitus

  • Low-dose or chronic steroid use

⚠️ Common Symptoms

CPA symptoms often evolve insidiously over >3 months:

  • Chronic productive cough

  • Haemoptysis (may be mild or massive)

  • Fatigue and weight loss

  • Breathlessness

  • Chest discomfort

  • Fever (occasional)

🧪 Diagnosis

Diagnosis of CPA requires the combination of:

  1. Symptoms ≥3 months

  2. Imaging:

    • CT chest: cavitary lesions, pleural thickening, aspergilloma, fibrotic changes

  3. Microbiology:

    • Positive sputum culture, PCR, or histopathology for A. fumigatus

  4. Serology:

    • Elevated Aspergillus IgG antibodies (essential for diagnosis)

  5. Exclusion of other diseases:

    • Especially active TB, malignancy, and bacterial infections

💊 Treatment

First-Line:

  • Oral triazole antifungals (minimum 6 months, often longer)

    • Itraconazole

    • Voriconazole

    • Posaconazole

  • Therapeutic drug monitoring is crucial

Additional:

  • Inhaled antifungals (e.g. amphotericin B) in selected cases

  • Surgery for localised disease or life-threatening haemoptysis (if fit)

  • Bronchial artery embolisation for bleeding control

  • Physiotherapy and nutritional support

🧾 Monitoring

  • CT scan every 3–6 months during treatment

  • Aspergillus IgG titres to monitor disease activity

  • Liver function and antifungal levels (monthly at minimum)

  • Symptom tracking (cough, energy, weight, breathlessness)

📚 More Information

by GAtherton

Patient referrals in the NHS - how it works

Here’s why GPs in the NHS usually refer patients first to a local specialist (e.g. a local respiratory team) rather than directly to a national centre like the National Aspergillosis Centre (NAC):

🏥 1. The NHS Referral Pathway Is Tiered (Local → Regional → National)

  • The NHS is designed to escalate care through “levels”:

    • GP → Local Consultant → Tertiary/National Centre

  • This structure ensures efficient use of resources and prioritises local care where appropriate.

  • National centres are not intended to be the first point of contact, except in emergencies or highly specialised pre-agreed pathways.

🧠 Analogy: You don’t go straight to a brain surgeon for a headache — you start with your GP.

📝 2. Referral Criteria for NAC Require Specialist Input

  • The NAC (based in Manchester) is a nationally commissioned tertiary centre, which means:

    • It only accepts referrals from consultants (not GPs directly)

    • It expects that basic tests (CT scan, IgE, Aspergillus-specific IgE/IgG, eosinophils, spirometry) have been done

    • Local teams should attempt initial diagnosis and management, and refer on if the case is complex, resistant, or unusual

📄 The NAC’s referral form specifically asks for consultant details and supporting investigations.

⚖️ 3. Clinical Governance and Local Responsibility

  • Local respiratory consultants are responsible for:

    • Ruling out common conditions first

    • Starting standard ABPA or CPA treatment (e.g. steroids, itraconazole)

    • Monitoring early response

  • This ensures that patients who are referred to the NAC are those who really need advanced care, e.g.:

    • Antifungal resistance

    • Multiple relapses

    • Atypical radiology

    • Drug intolerance or failure

    • Need for biologics, surgery, or MDT input

🧭 4. NHS Resource Planning and Fairness

  • National centres are funded to manage only the most complex or rare cases across the UK.

  • If GPs referred patients directly, national centres would become overwhelmed — and many patients would bypass the local care they actually need.

💡 It's not about gatekeeping — it's about managing capacity and focusing expertise where it’s most needed.

🛠️ What Can Patients Do?

If you suspect ABPA or CPA and your GP doesn’t know about NAC:

  1. Ask to be referred to a local respiratory team — ideally one with fungal disease knowledge.

  2. Share NAC information NAC referral criteria & guidance, Support for professionals

  3. If already under a consultant and you're not improving, ask:

    “Would you consider referring me to the National Aspergillosis Centre for specialist input?”

  4. If you're already diagnosed with ABPA or CPA and not improving, you can request your consultant refer you to NAC, citing lack of progress or drug intolerance.

by GAtherton