Allergic bronchopulmonary aspergillosis
diseaseOn this page
Also known as ABPAallergic aspergillosisallergic bronchopulmonary mycosisaspergillosis, allergic bronchopulmonaryHinson-Pepys disease
Summary
Allergic bronchopulmonary aspergillosis (MONDO:0015243) is a disease and 37 clinical trials. Top therapeutic interventions include itraconazole, omalizumab, and voriconazole. A subtype of allergic respiratory disease — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: Unknown (Worldwide) [Orphanet-validated]
- Phenotypes (HPO): 14
- Clinical trials: 37
Clinical features
Signs & symptoms
Clinical features (HPO)
14 HPO clinical features (Orphanet curated; top 14 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0001879 | Abnormality of eosinophils | Very frequent (80-99%) |
| HP:0002099 | Asthma | Very frequent (80-99%) |
| HP:0002715 | Abnormality of the immune system | Very frequent (80-99%) |
| HP:0012735 | Cough | Very frequent (80-99%) |
| HP:0001824 | Weight loss | Frequent (30-79%) |
| HP:0002110 | Bronchiectasis | Frequent (30-79%) |
| HP:0002120 | Cerebral cortical atrophy | Frequent (30-79%) |
| HP:0011134 | Low-grade fever | Frequent (30-79%) |
| HP:0001231 | Abnormal fingernail morphology | Occasional (5-29%) |
| HP:0002092 | Pulmonary arterial hypertension | Occasional (5-29%) |
| HP:0002093 | Respiratory insufficiency | Occasional (5-29%) |
| HP:0002097 | Emphysema | Occasional (5-29%) |
| HP:0002105 | Hemoptysis | Occasional (5-29%) |
| HP:0025426 | Abnormal bronchus morphology | Occasional (5-29%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | allergic bronchopulmonary aspergillosis |
| Mondo ID | MONDO:0015243 |
| EFO | EFO:0007140 |
| MeSH | D001229 |
| OMIM | 103920 |
| Orphanet | 1164 |
| DOID | DOID:13166 |
| ICD-10-CM | B44.81 |
| ICD-11 | 1591607082 |
| NCIT | C84547 |
| SNOMED CT | 37981002 |
| UMLS | C3278302 |
| MedGen | 479932 |
| GARD | 0000602 |
| MedDRA | 10006474 |
| Anatomy (UBERON) | UBERON:0001004 |
| Is cancer (heuristic) | no |
Also known as: ABPA · allergic aspergillosis · allergic bronchopulmonary mycosis · aspergillosis, allergic bronchopulmonary · Hinson-Pepys disease
Disease family
This is a subtype of allergic respiratory disease. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › respiratory system disorder › allergic respiratory disease › allergic bronchopulmonary aspergillosis
Related subtypes (3): allergic asthma, allergic rhinitis, hypersensitivity pneumonitis
Subtypes (1): malt worker’s lung
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
Drugs indicated for this disease
0 approved, 3 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.
| Drug | Development status |
|---|---|
| Ibrexafungerp | Phase 3 (in late-stage trials) |
| Itraconazole | Phase 3 (in late-stage trials) |
| Prednisolone | Phase 3 (in late-stage trials) |
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Amphotericin B, Budesonide, Deflazacort, Dupilumab, Voriconazole.
Clinical trials & evidence
Clinical trials
Clinical trials: 37.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 20 |
| PHASE2/PHASE3 | 6 |
| PHASE2 | 5 |
| PHASE3 | 3 |
| PHASE4 | 2 |
| PHASE1 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT00787917 | PHASE4 | TERMINATED | An Exploratory Study to Assess Multiple Doses of Omalizumab in Patients With Cystic Fibrosis Complicated by Acute Bronchopulmonary Aspergillosis (ABPA) |
| NCT04108962 | PHASE4 | WITHDRAWN | Benralizumab in the Treatment of Patients With Severe Asthma With ABPA |
| NCT04227483 | PHASE2/PHASE3 | ACTIVE_NOT_RECRUITING | Deflazacort Vs. Prednisolone in Acute-stage ABPA |
| NCT06174922 | PHASE3 | RECRUITING | A Randomized Trial of Prednisolone, Itraconazole, or Their Combination in Allergic Bronchopulmonary Aspergillosis |
| NCT00974766 | PHASE2/PHASE3 | COMPLETED | Trial on the Efficacy and Safety of Two Different Glucocorticoid Dose Regimens in Allergic Bronchopulmonary Aspergillosis |
| NCT01321827 | PHASE2/PHASE3 | COMPLETED | Monotherapy of Itraconazole Versus Prednisolone in Allergic Bronchopulmonary Aspergillosis |
| NCT01621321 | PHASE2/PHASE3 | COMPLETED | Voriconazole Versus Oral Steroids in Allergic Bronchopulmonary Aspergillosis |
| NCT01857479 | PHASE2/PHASE3 | COMPLETED | A Randomized Controlled Trial of Inhaled Amphotericin B for Maintaining Remission in Allergic Bronchopulmonary Aspergillosis |
| NCT02440009 | PHASE2/PHASE3 | COMPLETED | A Randomized Trial of Itraconazole in Acute Stages of Allergic Bronchopulmonary Aspergillosis |
| NCT02879305 | PHASE3 | COMPLETED | Anemia Studies in Chronic Kidney Disease: Erythropoiesis Via a Novel Prolyl Hydroxylase Inhibitor Daprodustat-Dialysis (ASCEND-D) |
| NCT03059992 | PHASE3 | COMPLETED | Study to Evaluate the Efficacy and Safety of Ibrexafungerp in Patients With Fungal Diseases That Are Refractory to or Intolerant of Standard Antifungal Treatment |
| NCT06244979 | PHASE2 | RECRUITING | iMagIng pulmonaRy Aspergillosis Using Gallium-68-dEferoxamine |
| NCT02273661 | PHASE2 | COMPLETED | Evaluation of a Therapeutic Strategy Including Nebulised Liposomal Amphotericin B (Ambisome®) in Maintenance Treatment of Allergic Bronchopulmonary Aspergillosis (Cystic Fibrosis Excluded). |
| NCT03960606 | PHASE2 | TERMINATED | Study in Adult Asthmatic Patients With Allergic Bronchopulmonary Aspergillosis |
| NCT04442269 | PHASE2 | COMPLETED | Investigating Treatment With Dupilumab in Patients With Allergic Bronchopulmonary Aspergillosis (ABPA) (LIBERTY ABPA AIRED) |
| NCT05667662 | PHASE2 | TERMINATED | Study to Evaluate Itraconazole Administered as Inhaled Dry Powder in Adults With Asthma and ABPA |
| NCT04229303 | PHASE1 | COMPLETED | Phase 1 Three Part SAD, MAD & Cross-Over Study of ZP-059 in Healthy and Asthmatic Subjects |
| NCT05444946 | Not specified | RECRUITING | Oral Itraconazole Versus Combination of Systemic Glucorticoids and Oral Itraconazole in CPA-ABPA Overlap Syndrome |
| NCT06645210 | Not specified | NOT_YET_RECRUITING | Multicenter Registration Study on Allergic Bronchopulmonary Aspergillosis |
| NCT07040332 | Not specified | RECRUITING | Pathogenesis and Inflammatory Phenotype of Allergic Bronchopulmonary Aspergillosis |
| NCT07313904 | Not specified | RECRUITING | Effects of Environmental Fungal Exposure on Bronchial Asthma, ABPA and Bronchiectasis |
| NCT07362667 | Not specified | RECRUITING | A Study of the Efficacy and Safety of Bronchoscopic Airway Clearance and Amphotericin B Spraying in Patients With ABPA |
| NCT07362693 | Not specified | RECRUITING | A Cohort Study of the Efficacy and Safety of Biologics in Patients With ABPA |
| NCT07493629 | Not specified | RECRUITING | Immune Status and Disease Control of Inflammatory Airway Diseases |
| NCT07530770 | Not specified | RECRUITING | Real-World Effectiveness of Benralizumab in Allergic Bronchopulmonary Aspergillosis |
| NCT07611838 | Not specified | RECRUITING | Multi-Omics Inflammatory Phenotype for ABPA Recurrence Risk Prediction |
| NCT00585364 | Not specified | COMPLETED | Mechanisms of Immune Tolerance and Inflammation in Patients With Cystic Fibrosis With ABPA |
| NCT01222273 | Not specified | COMPLETED | Open-label Vitamin D Trial for Patients With Cystic Fibrosis and Allergic Bronchopulmonary Aspergillosis |
| NCT02569879 | Not specified | COMPLETED | Impact of Boostrix™ Maternal Vaccination on Morbidity and Mortality of Pertussis Disease in Infants ≤6 Weeks of Age, in Bogota, Colombia. |
| NCT02853175 | Not specified | COMPLETED | Lung MRI and Allergic Broncho-pulmonary Aspergillosis in Cystic Fibrosis |
| NCT03643185 | Not specified | UNKNOWN | Cytokine Profile of Allergic Bronchopulmonary Aspergillosis |
| NCT04476758 | Not specified | COMPLETED | Immune Profiles in CF Fungal Infection |
| NCT05129033 | Not specified | UNKNOWN | A Prospective Study on Optimizing Treatment for ABPA |
| NCT05616338 | Not specified | COMPLETED | Modeling Bronchial Epithelium in Severe Asthma With Human Induced Pluripotent Stem Cells (iPSC) |
| NCT05897294 | Not specified | NO_LONGER_AVAILABLE | Voriconazole Inhalation Powder for the Treatment of Pulmonary Aspergillosis |
| NCT05903612 | Not specified | WITHDRAWN | Allergic Bronchopulmonary Aspergillosis Prescreening Study |
| NCT07055230 | Not specified | COMPLETED | Interest of IgE Directed Against Recombinant Antigens of Aspergillus Fumigatus in the Diagnosis of Allergic Bronchopulmonary Aspergillosis (ABPA) |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| ITRACONAZOLE | 4 | 5 |
| OMALIZUMAB | 4 | 2 |
| VORICONAZOLE | 4 | 2 |
| AMPHOTERICIN B | 4 | 1 |
| BENRALIZUMAB | 4 | 1 |
| DAPRODUSTAT | 4 | 1 |
| DEFLAZACORT | 4 | 1 |
| IBREXAFUNGERP | 4 | 1 |
| CHEMBL4785395 | 0 | 1 |
| CHEMBL4163812 | 0 | 1 |
| CHEMBL4764856 | 0 | 1 |
| HYOSCYAMINE | -1 | 5 |