Bronchiolitis obliterans syndrome
diseaseOn this page
Also known as BOBOOPbronchiolitis obliteransbronchiolitis obliterans with obstructive pulmonary diseaseconstrictive bronchiolitisorganised pneumoniaorganising pneumoniaorganized pneumoniaorganizing pneumonia
Summary
Bronchiolitis obliterans syndrome (MONDO:0015265) is a disease and 85 clinical trials. Top therapeutic interventions include montelukast, azithromycin, and belumosudil. A subtype of bronchiolitis — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: Unknown (Worldwide)
- Phenotypes (HPO): 16
- Clinical trials: 85
Clinical features
Signs & symptoms
Clinical features (HPO)
16 HPO clinical features (Orphanet curated; top 16 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0002094 | Dyspnea | Very frequent (80-99%) |
| HP:0006536 | Airway obstruction | Very frequent (80-99%) |
| HP:0011946 | Bronchiolitis obliterans | Very frequent (80-99%) |
| HP:0012418 | Hypoxemia | Very frequent (80-99%) |
| HP:0012735 | Cough | Very frequent (80-99%) |
| HP:0030877 | Reduced FEV1/FVC ratio | Very frequent (80-99%) |
| HP:0032341 | Reduced forced vital capacity | Very frequent (80-99%) |
| HP:0032342 | Reduced forced expiratory volume in one second | Very frequent (80-99%) |
| HP:0032359 | Decreased forced expiratory flow 25-75% | Very frequent (80-99%) |
| HP:0002090 | Pneumonia | Frequent (30-79%) |
| HP:0011947 | Respiratory tract infection | Frequent (30-79%) |
| HP:0031246 | Nonproductive cough | Frequent (30-79%) |
| HP:0045051 | Decreased DLCO | Frequent (30-79%) |
| HP:0002110 | Bronchiectasis | Occasional (5-29%) |
| HP:0002960 | Autoimmunity | Occasional (5-29%) |
| HP:0025179 | Ground-glass opacification on pulmonary HRCT | Very rare (<1-4%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | bronchiolitis obliterans syndrome |
| Mondo ID | MONDO:0015265 |
| EFO | EFO:0007183 |
| Orphanet | 1303 |
| DOID | DOID:2799 |
| ICD-11 | 592711730 |
| SNOMED CT | 40100001 |
| UMLS | C0006272 |
| MedGen | 665 |
| GARD | 0005961 |
| Is cancer (heuristic) | no |
Also known as: BO · BOOP · bronchiolitis obliterans · bronchiolitis obliterans with obstructive pulmonary disease · constrictive bronchiolitis · organised pneumonia · organising pneumonia · organized pneumonia · organizing pneumonia
Data availability: 1 HPO phenotype.
Disease family
This is a subtype of bronchiolitis. 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 › lower respiratory tract disorder › lung disorder › bronchiolitis › bronchiolitis obliterans syndrome
Related subtypes (5): acute bronchiolitis, toxic bronchiolitis, respiratory syncytial virus bronchiolitis, postinfectious bronchiolitis obliterans, Mac-Leod-Swyer-James-Syndrome
Subtypes (2): transplant-related bronchiolitis obliterans, non-transplant-related bronchiolitis obliterans
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, 6 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.
| Drug | Development status |
|---|---|
| Azithromycin | Phase 3 (in late-stage trials) |
| Clarithromycin | Phase 3 (in late-stage trials) |
| Cyclosporine | Phase 3 (in late-stage trials) |
| Fluticasone Propionate | Phase 3 (in late-stage trials) |
| Salmeterol | Phase 3 (in late-stage trials) |
| Tacrolimus Anhydrous | Phase 3 (in late-stage trials) |
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Bortezomib, Budesonide, Formoterol, INTERFERON GAMMA-1B, Lactose, Anhydrous, Nintedanib, Pirfenidone, Ruxolitinib.
Clinical trials & evidence
Clinical trials
Clinical trials: 85.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 44 |
| PHASE2 | 15 |
| PHASE3 | 8 |
| PHASE1/PHASE2 | 6 |
| PHASE4 | 5 |
| PHASE1 | 4 |
| EARLY_PHASE1 | 2 |
| PHASE2/PHASE3 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT00367419 | PHASE4 | COMPLETED | Use of Erythromycin in Mustard-Induced Bronchiolitis |
| NCT01009619 | PHASE4 | COMPLETED | Azithromycin in Bronchiolitis Obliterans Syndrome |
| NCT01211509 | PHASE4 | COMPLETED | Montelukast in Bronchiolitis Obliterans Syndrome |
| NCT01212406 | PHASE4 | COMPLETED | Vitamin D in Bronchiolitis Obliterans Syndrome |
| NCT05299567 | PHASE4 | COMPLETED | Efficacy and Safety of Azithromycin in Patients With Bronchiolitis Obliterans |
| NCT02181257 | PHASE3 | ACTIVE_NOT_RECRUITING | Extracorporeal Photopheresis for Medicare Recipients of Lung Allografts |
| NCT00381147 | PHASE3 | COMPLETED | Use of Clarithromycin in Mustard-Induced Bronchiolitis |
| NCT01334892 | PHASE2/PHASE3 | TERMINATED | L-CsA in the Prevention of Bronchiolitis Obliterans Syndrome (BOS) in Lung Transplant (LT) Patients |
| NCT01429844 | PHASE3 | COMPLETED | Tacrolimus Versus Cyclosporine for Immunosuppression After Lung Transplantation |
| NCT01439958 | PHASE3 | TERMINATED | Long-term Safety Follow-up Study in the Prevention of Bronchiolitis Obliterans Syndrome (BOS) |
| NCT03656926 | PHASE3 | COMPLETED | Efficacy + Safety of Liposome Cyclosporine A to Treat Bronchiolitis Obliterans Post Double Lung Transplant (BOSTON-2) |
| NCT03657342 | PHASE3 | COMPLETED | Efficacy + Safety of Liposome Cyclosporine A to Treat Bronchiolitis Obliterans Post Single Lung Transplant (BOSTON-1) |
| NCT04039347 | PHASE3 | TERMINATED | Extension Trial on Efficacy / Safety of L-CsA + SoC in Treating BOS in Post Single or Double Lung Transplant (BOSTON-3) |
| NCT04655508 | PHASE3 | TERMINATED | Efficacy of Fluticasone Propionate Associated With Salmeterol Using Inhalation Chamber Versus Placebo to Improve the Respiratory Function in Children Over Six Years of Age Who Underwent Allogeneic Hematopoietic Stem Cell Transplantation With a Decline of FEV1 ≥10% From Pre Transplantation |
| NCT02669251 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Alvelestat (MPH966), an Oral Neutrophil Elastase Inhibitor, in Bronchiolitis Obliterans Syndrome After Allogeneic Hematopoietic Stem Cell Transplantation |
| NCT03805477 | PHASE2 | RECRUITING | Nintedanib in Patients With Bronchiolitis Obliterans Syndrome Following Hematopoietic Stem Cell Transplantation |
| NCT05922761 | PHASE2 | RECRUITING | BElumosudil for Bronchiolitis Obliterans Prevention/Therapy (BEBOP) |
| NCT06018766 | PHASE2 | RECRUITING | LAM-001 in Lung Transplant Recipients With Bronchiolitis Obliterans Syndrome. |
| NCT00029328 | PHASE1/PHASE2 | COMPLETED | Etanercept for Non-Infectious Lung Injury Following Bone Marrow Transplantation |
| NCT00141726 | PHASE2 | COMPLETED | Study of Enbrel (Etanercept) for the Treatment Sub-Acute Pulmonary Dysfunction After Allogeneic Stem Cell Transplant |
| NCT00656058 | PHASE2 | COMPLETED | Montelukast to Treat Bronchiolitis Obliterans |
| NCT01163786 | PHASE2 | TERMINATED | A Proposal to Test the Efficacy and Tolerability of Bortezomib in Pulmonary Chronic GVHD |
| NCT01273207 | PHASE2 | COMPLETED | Extension Study (Extended Access) of Cyclosporine Inhalation Solution (CIS) in Lung Transplant and Hematopoietic Stem Cell Transplant Recipients for the Treatment of Bronchiolitis Obliterans |
| NCT01287078 | PHASE2 | COMPLETED | Cyclosporine Inhalation Solution (CIS) in Lung Transplant and Hematopoietic Stem Cell Transplant Recipients for the Treatment of Bronchiolitis Obliterans Syndrome |
| NCT01307462 | PHASE2 | COMPLETED | Targeted Therapy of Bronchiolitis Obliterans Syndrome |
| NCT01432080 | PHASE2 | TERMINATED | Steroids, Azithromycin, Montelukast, and Symbicort (SAMS) for Viral Respiratory Tract Infection Post Allotransplant |
| NCT01560689 | PHASE2 | COMPLETED | Inhaled BUDESONIDE/FORMOTEROL in Bronchiolitis Obliterans Following Stem Cell Transplantation |
| NCT01650545 | PHASE1/PHASE2 | COMPLETED | Aerosol Liposomal Cyclosporine for Chronic Rejection in Lung Transplant Recipients |
| NCT02488304 | PHASE2 | TERMINATED | Prospective Study for Transplant Optimization Using Functional Imaging (TROFI) |
| NCT02543073 | PHASE1/PHASE2 | UNKNOWN | MSC for Treatment of Interstitial Lung Disease After Allo-HSCT |
| NCT03603899 | PHASE1/PHASE2 | COMPLETED | Hp129 Xenon Imaging and BOS in Lung Transplantation |
| NCT03978637 | PHASE1/PHASE2 | TERMINATED | Safety and Efficacy of Itacitinib in Participants With Bronchiolitis Obliterans Syndrome Following Lung Transplantation |
| NCT04107675 | PHASE2 | TERMINATED | A Safety Study of Liposomal Cyclosporine A to Treat Bronchiolitis After Hematopoietic Transplant (BOSTON-4) |
| NCT04908735 | PHASE2 | TERMINATED | Ruxolitinib for Early Lung Dysfunction After Hematopoietic Stem Cell Transplant |
| NCT05413356 | PHASE2 | UNKNOWN | Ruxolitinib for Newly Diagnosed Bronchiolitis Obliterans Syndrome |
| NCT01175655 | PHASE1 | COMPLETED | A Study to Evaluate the Potential of Mesenchymal Stromal Cells to Treat Obliterative Bronchiolitis After Lung Transplantation |
| NCT02181712 | PHASE1 | COMPLETED | Mesenchymal Stem Cell Therapy for Lung Rejection |
| NCT03315741 | PHASE1 | COMPLETED | The Safety and Tolerability of Pirfenidone for BOS After HCT |
| NCT04239989 | PHASE1 | TERMINATED | Itacitinib for the Treatment of Bronchiolitis Obliterans Syndrome After Donor Hematopoietic Cell Transplant |
| NCT00378677 | EARLY_PHASE1 | UNKNOWN | Dry Powder Inhalation of Cyclosporine A in Lung Transplant Patients With Bronchiolitis Obliterans Syndrome |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| MONTELUKAST | 4 | 3 |
| AZITHROMYCIN | 4 | 2 |
| BELUMOSUDIL | 4 | 1 |
| CHOLECALCIFEROL | 4 | 1 |
| CLARITHROMYCIN | 4 | 1 |
| ERGOCALCIFEROL | 4 | 1 |
| ERYTHROMYCIN | 4 | 1 |
| NINTEDANIB | 4 | 1 |
| ITACITINIB | 3 | 3 |
| ALVELESTAT | 2 | 1 |
| RILDINADSTROCEL | 2 | 1 |
| CHEMBL4299381 | 0 | 7 |
| CHEMBL5205127 | 0 | 1 |
| CHEMBL5430305 | 0 | 1 |
| CHEMBL5415229 | 0 | 1 |