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ABPA — Allergic Bronchopulmonary Aspergillosis (Diagnosis and Treatment)

Hypersensitivity reaction to Aspergillus fumigatus colonization in airways of asthma and cystic fibrosis patients producing chronic poorly controlled asthma, recurrent fleeting pulmonary infiltrates, central bronchiectasis, eosinophilia, elevated total IgE >1000 IU/mL, and positive Aspergillus-specific IgE/IgG; treatment with systemic corticosteroids and itraconazole/voriconazole antifungals to prevent progressive bronchiectasis and respiratory failure.

Written by: Saygı Hospital Health Guide Editorial Board
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This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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What is ABPA — Allergic Bronchopulmonary Aspergillosis (Diagnosis and Treatment)?

Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity pulmonary disease caused by chronic colonization and exaggerated immune response to inhaled Aspergillus fumigatus (>90 percent; rarely other Aspergillus species — A. flavus, A. niger, A. terreus) within airway mucus of susceptible patients with asthma (1–2 percent of asthmatics, 7–14 percent of severe asthma) or cystic fibrosis (7–9 percent of CF patients). First described by Hinson and colleagues in 1952. Worldwide prevalence estimated 4–5 million ABPA cases globally complicating asthma and CF.

Pathophysiology involves multifactorial hypersensitivity to Aspergillus antigens with mixed Gell-Coombs reactions: (1) Type I IgE-mediated immediate hypersensitivity producing wheal-flare on skin testing, IgE elevation, mast cell-mediated bronchoconstriction; (2) Type III immune complex deposition with complement activation; (3) Type IVa Th2-mediated cellular immunity with IL-4, IL-5, IL-13 cytokine production driving eosinophilic airway inflammation, mucus hypersecretion, IgE class switching. Genetic predisposition: HLA-DRB1*15:01, *15:02, *15:03 association, surfactant protein A2 polymorphisms, IL-4Rα and IL-10 polymorphisms, CFTR mutations as cofactors. Defective airway clearance and persistent Aspergillus colonization in mucus produces chronic immune activation, eosinophilic and lymphocytic infiltration, mucus impaction, central bronchiectasis (predominantly proximal large airways with distal sparing), and progressive lung damage.

ISHAM (International Society for Human and Animal Mycology) 2013 diagnostic criteria for ABPA: (1) Predisposing condition — asthma or cystic fibrosis (some controversy regarding ABPA in non-asthmatic patients); (2) Obligatory criteria (both required) — (a) type I Aspergillus skin test positivity (immediate hypersensitivity) OR elevated A. fumigatus-specific IgE level (>0.35 kUA/L), (b) elevated total IgE concentration >1000 IU/mL (cutoff may be reduced in CF — >500 IU/mL); (3) Other criteria (at least 2 of 3) — (a) Aspergillus-specific precipitins (precipitating antibodies) or IgG positive in serum, (b) radiographic features compatible with ABPA (transient or fixed pulmonary opacities, central bronchiectasis), (c) total eosinophil count >500 cells/μL in steroid-naive patients (excluded from criteria when patient on systemic corticosteroids).

ABPA staging (Patterson 1982 modified): Stage I — Acute (newly diagnosed with active symptoms, elevated IgE, eosinophilia, infiltrates); Stage II — Remission (asymptomatic for ≥6 months off corticosteroids, normal IgE or stable elevated, no infiltrates); Stage III — Exacerbation (symptoms recurrence, doubling of baseline IgE, new infiltrates after Stage I or II); Stage IV — Corticosteroid-dependent asthma (cannot taper steroids without exacerbation, may have wheezing without infiltrates or rising IgE); Stage V — Fibrotic end-stage (extensive bronchiectasis, fibrosis, dyspnea, often type II respiratory failure, irreversible). Imaging features: chest X-ray — fleeting pulmonary infiltrates, parallel line shadows (bronchial wall thickening), ring shadows, finger-in-glove (mucus plugs in dilated bronchi); high-resolution CT (HRCT) — central upper lobe bronchiectasis (pathognomonic — affects medial 2/3 of lung field, distal airways spared), high-attenuation mucus (HAM — mucus density >70 HU within bronchi, helpful diagnostic feature, may indicate active disease), tree-in-bud, mucus plugs, fleeting infiltrates.

Symptoms

Worsening asthma control with frequent exacerbations despite optimal therapy
Productive cough with brown-grey or black mucus plugs (containing fungal hyphae and eosinophils — hallmark)
Wheezing and shortness of breath
Hemoptysis (sometimes severe)
Pleuritic chest pain
Low-grade fever
Malaise and fatigue
Frequent fleeting pulmonary infiltrates on imaging
Eosinophilia (>500 cells/μL when steroid-naive)
Elevated total IgE (>1000 IU/mL or rising)
Cystic fibrosis patient with new asthma-like symptoms or worsening lung function
Atopic background with allergic rhinitis, eczema, or other atopic comorbidities

Risk Factors

Asthma (especially severe asthma — 7–14 percent prevalence of ABPA)
Cystic fibrosis (7–9 percent prevalence)
Atopic background (increased Aspergillus skin test reactivity)
HLA-DRB1*15:01, *15:02, *15:03 alleles
Surfactant protein A2 polymorphisms
IL-4Rα, IL-10, CFTR variants
Environmental Aspergillus exposure (compost, decaying vegetation, water-damaged buildings, marijuana, basement)
Bronchial damage or anatomic predisposition (asthma airway remodeling, CF mucus stasis)
Inadequate asthma or CF control with mucus retention
Tropical climate with high environmental fungal burden

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Asthma or CF patient with worsening control, productive cough with mucus plugs
  • Hemoptysis in asthma or CF patient (rule out ABPA, pulmonary embolism, infection)
  • Pulmonary infiltrates on imaging in asthma or CF patient
  • Newly elevated total IgE >1000 IU/mL in asthma patient
  • Persistent eosinophilia (>500 cells/μL) in asthma patient
  • CF patient with declining lung function despite optimization
  • Suspected ABPA — for ISHAM criteria evaluation and treatment initiation
  • ABPA exacerbation despite standard therapy (steroid increase or antifungal addition)
  • Steroid-dependent ABPA with side effects (consider antifungal or biologic alternative)
  • Advanced ABPA with respiratory failure or massive hemoptysis — emergent

Treatment Methods

01
Diagnostic workup per ISHAM 2013 criteria: confirm asthma or CF diagnosis; serum IgE total >1000 IU/mL (>500 in CF), Aspergillus fumigatus-specific IgE (>0.35 kUA/L), Aspergillus-specific IgG (precipitating antibodies), Aspergillus skin prick test (immediate type I); complete blood count with differential (peripheral eosinophilia >500 cells/μL when steroid-naive); chest X-ray and high-resolution CT chest (central bronchiectasis, fleeting infiltrates, mucus plugs, high-attenuation mucus); spirometry; sputum cultures and microscopy may show Aspergillus hyphae but is not diagnostic; bronchoscopy not routinely needed but may be performed for atypical presentations or to clear mucus plugs causing lobar collapse
02
Differential diagnosis: cystic fibrosis with bronchiectasis (distal disease vs ABPA central), asthma with persistent eosinophilia and fleeting infiltrates (eosinophilic asthma without ABPA), eosinophilic granulomatosis with polyangiitis (EGPA — vasculitis, neuropathy, asthma triad), chronic eosinophilic pneumonia (peripheral upper lobe infiltrates, no ABPA criteria), parasitic infection (eosinophilia from parasites — strongyloides, ascaris, etc.), allergic fungal sinusitis with extension, semi-invasive aspergillosis (chronic necrotizing — distinct entity in immunocompromised), aspergilloma in pre-existing cavity
03
First-line therapy oral corticosteroids: induction with prednisolone 0.5–0.75 mg/kg/day for 2–4 weeks (some use 0.75–1 mg/kg up to 60 mg max), tapered over 6–12 weeks; alternative regimen — prednisolone 0.75 mg/kg/day for 6 weeks then 0.5 mg/kg/day for 6 weeks then taper by 5 mg every 6 weeks over months; goals — clinical improvement, normalization or substantial reduction of total IgE (target ≥35 percent reduction within 8 weeks indicates response), resolution of pulmonary infiltrates, eosinophil normalization; bone health, glucose, blood pressure monitoring during corticosteroid therapy
04
Antifungal therapy: itraconazole 200 mg twice daily for 4–6 months as primary alternative or adjunct to corticosteroids — particularly useful in steroid-dependent disease (Stage IV) or when steroid sparing desired; reduces fungal antigen load and inflammatory drive; monitor itraconazole levels (trough >1 mcg/mL) and liver function (transaminitis common); voriconazole 200 mg twice daily as alternative (similar evidence; QT prolongation, photosensitivity, periostitis, neurologic side effects, drug interactions); posaconazole, isavuconazole emerging options; antifungal alone less effective than combination with corticosteroids in active disease
05
Combination therapy: most effective approach in moderate-severe disease combines short-course oral corticosteroids with itraconazole 4–6 months; allows lower steroid dose and shorter duration with similar efficacy and reduced steroid side effects
06
Inhaled antifungals: nebulized amphotericin B (deoxycholate or liposomal) for refractory disease — limited evidence but increasing use, especially in CF; potential for direct airway antifungal delivery with reduced systemic absorption; bronchospasm risk requires premedication with bronchodilator
07
Biologic therapy for ABPA (emerging): omalizumab (anti-IgE — case series suggest benefit in some ABPA, particularly with severe asthma component, may reduce steroid requirement; off-label for ABPA but increasingly used), mepolizumab (anti-IL-5 — for eosinophilic ABPA, particularly steroid-dependent — reduces eosinophilia and exacerbations), dupilumab (anti-IL-4Rα — increasing evidence in ABPA particularly in CF, may target Th2 inflammation more comprehensively), benralizumab — case reports of benefit; tezepelumab — emerging interest given upstream TSLP target
08
Treat exacerbations promptly: increase or restart corticosteroids; consider adding antifungal if not on; bronchoscopic mucus plug removal if causing lobar collapse; address comorbidities (sinusitis, GERD)
09
CF-specific considerations: aggressive airway clearance (chest physiotherapy, nebulized hypertonic saline, mucolytics, dornase alfa), CFTR modulator therapy if eligible (modulators may reduce mucus stasis and ABPA risk), antimicrobial coverage of bacterial pathogens, attention to drug interactions (azole antifungals interact with CFTR modulators)
10
Long-term monitoring: clinical assessment quarterly with symptoms, exacerbation frequency, steroid dose; serum total IgE every 1–2 months during active treatment then every 3–6 months (rising IgE may indicate exacerbation); spirometry every 3–6 months; chest CT annually or per clinical change; assessment of corticosteroid side effects (osteoporosis with DXA scan every 1–2 years, glucose, blood pressure, ophthalmologic for cataracts and IOP); adherence to inhaled and systemic therapy; trigger avoidance counseling
11
Patient education: avoidance of high-Aspergillus environments (compost, water-damaged buildings, marijuana smoking, basement work, gardening with damp leaves), ventilation and humidity control at home, action plan for symptom worsening, importance of medication adherence and follow-up, recognition of exacerbation signs, awareness of corticosteroid and antifungal side effects, smoking cessation, vaccinations including annual influenza and pneumococcal
12
Prognosis: with adequate treatment, most patients achieve disease control and avoid progression to fibrotic end-stage; relapse common (especially in CF) requiring chronic management; lung transplantation consideration in fibrotic end-stage with respiratory failure; multidisciplinary care (pulmonologist, allergist-immunologist, infectious diseases, CF specialist if applicable, nutritionist, mental health) essential for optimal outcomes

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.