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bronchiolitis obliterans syndrome (BOOP) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

bronchiolitis obliterans syndrome

MONDO:0015265

Also known as:BOOPbronchiolitis fibrosa obliteransconstrictive bronchiolitis

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Overview

A lung disorder that is mainly associated with chronic allograft dysfunction after lung transplantation and that is characterized by inflammation and fibrosis of bronchiolar walls that reduce the diameter of the bronchioles and result in progressive and irreversible airflow obstruction.

TreatmentsNo approved treatments · 4 in pipeline

Clinical Trials19 registered · 9 recruiting

Diagnosis

Biomarker and diagnostic research for bronchiolitis obliterans syndrome has been reported in the published literature.

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

No approved treatments are currently available for bronchiolitis obliterans syndrome. An additional 4 compounds hold orphan drug designation.

Orphan Drug Designations

While no drugs are FDA-approved specifically for bronchiolitis obliterans syndrome, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.

The following drugs have received orphan drug designation from the FDA for bronchiolitis obliterans syndrome. Orphan designation reflects regulatory interest and does not indicate approval for treatment.

Brand Name	Generic Name	Sponsor

Prognosis

Estimated prevalence: Unknown (Unknown prevalence).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

19 clinical trials registered, 9 recruiting. Interventions under study include other interventions, drug therapy, procedural interventions, and gene therapy. Pipeline includes 3 PHASE3, 5 PHASE2, 1 PHASE1. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT04098445](https://clinicaltrials.gov/study/NCT04098445)

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 6:45 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:1303

GARD

Genetic and Rare Diseases Info Center

GARD:5961

Questions for Your Care Team

→Are any of the 19 active clinical trials a good fit for my situation?
→With 4 compounds in the orphan drug pipeline, which are furthest along in development and which clinical trials should I be aware of?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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Research Type	Count	% of Total
Research summaries	36	24%
Disease patterns and progression	31	21%
Clinical study results	22	15%
Laboratory research	22	15%
Testing and diagnosis research	16	11%
Patient case studies	10	7%
Other research	6	4%
New treatment approaches	4	3%

bronchiolitis obliterans syndrome

Overview

Diagnosis

Treatment

Orphan Drug Designations

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Clinical Trials

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