Cryptogenic organizing pneumonia

Summary

Cryptogenic organizing pneumonia (MONDO:0015264) is a disease and 10 clinical trials. Top therapeutic interventions include montelukast and bardoxolone methyl. A subtype of idiopathic interstitial pneumonia — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Prevalence: 1-9 / 1 000 000 (Spain) [Orphanet-validated]
• Phenotypes (HPO): 27
• Clinical trials: 10

Clinical features

Epidemiology

Prevalence records

3 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 100 000	1.1	Iceland	Validated
Point prevalence	1-9 / 1 000 000	0.81	Spain	Validated
Point prevalence	1-9 / 1 000 000	0.92	Greece	Validated

Signs & symptoms

Clinical features (HPO)

27 HPO clinical features (Orphanet curated; top 27 by frequency):

HPO ID	Term	Frequency
HP:0012735	Cough	Very frequent (80-99%)
HP:0032177	Parenchymal consolidation	Very frequent (80-99%)
HP:0001945	Fever	Frequent (30-79%)
HP:0001974	Leukocytosis	Frequent (30-79%)
HP:0002039	Anorexia	Frequent (30-79%)
HP:0002091	Restrictive ventilatory defect	Frequent (30-79%)
HP:0002094	Dyspnea	Frequent (30-79%)
HP:0003565	Elevated erythrocyte sedimentation rate	Frequent (30-79%)
HP:0011897	Neutrophilia	Frequent (30-79%)
HP:0012378	Fatigue	Frequent (30-79%)
HP:0025179	Ground-glass opacification on pulmonary HRCT	Frequent (30-79%)
HP:0030830	Crackles	Frequent (30-79%)
HP:0031246	Nonproductive cough	Frequent (30-79%)
HP:0000961	Cyanosis	Occasional (5-29%)
HP:0001824	Weight loss	Occasional (5-29%)
HP:0002105	Hemoptysis	Occasional (5-29%)
HP:0011227	Elevated circulating C-reactive protein concentration	Occasional (5-29%)
HP:0030828	Wheezing	Occasional (5-29%)
HP:0031994	Bronchial breath sound	Occasional (5-29%)
HP:0032016	Abnormal sputum	Occasional (5-29%)
HP:0100749	Chest pain	Occasional (5-29%)
HP:0002098	Respiratory distress	Very rare (<1-4%)
HP:0002107	Pneumothorax	Very rare (<1-4%)
HP:0002829	Arthralgia	Very rare (<1-4%)
HP:0012418	Hypoxemia	Very rare (<1-4%)
HP:0025421	Pneumomediastinum	Very rare (<1-4%)
HP:0030166	Night sweats	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	cryptogenic organizing pneumonia
Mondo ID	MONDO:0015264
EFO	EFO:1001300
MeSH	D018549
Orphanet	1302
DOID	DOID:0050157
ICD-10-CM	J84.116
ICD-11	1937662238
NCIT	C62586
SNOMED CT	68409003, 719218000
UMLS	C0242770
MedGen	116663
GARD	0001620
NORD	873
Is cancer (heuristic)	no

Also known as: bronchiolitis obliterans organizing pneumonia · constrictive bronchiolitis · COP · cryptogenic organizing pneumonitis · idiopathic BOOP · idiopathic bronchiolitis obliterans organising pneumonia · idiopathic bronchiolitis obliterans organizing pneumonia · organising pneumonia · Organizing Pneumonia · organizing pneumonia

Disease family

This is a subtype of idiopathic interstitial pneumonia. 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 › respiratory tract infectious disorder › pneumonia › idiopathic interstitial pneumonia › cryptogenic organizing pneumonia

Related subtypes (9): lymphoid interstitial pneumonia, desquamative interstitial pneumonia, combined pulmonary fibrosis-emphysema syndrome, acute interstitial pneumonia, respiratory bronchiolitis-interstitial lung disease syndrome, non-specific interstitial pneumonia, idiopathic pleuroparenchymal fibroelastosis, follicular bronchiolits, idiopathic pulmonary fibrosis

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

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 10.

Phase distribution (across all retrieved trials)

Phase	Trials
Not specified	6
PHASE2	3
EARLY_PHASE1	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01287078	PHASE2	COMPLETED	Cyclosporine Inhalation Solution (CIS) in Lung Transplant and Hematopoietic Stem Cell Transplant Recipients for the Treatment of Bronchiolitis Obliterans Syndrome
NCT01432080	PHASE2	TERMINATED	Steroids, Azithromycin, Montelukast, and Symbicort (SAMS) for Viral Respiratory Tract Infection Post Allotransplant
NCT02036970	PHASE2	COMPLETED	Bardoxolone Methyl Evaluation in Patients With Pulmonary Hypertension (PH) - LARIAT
NCT03237364	EARLY_PHASE1	COMPLETED	Iraq- Afghanistan War Lung Injury Using 19F MRI (DIAL1001006)
NCT06203964	Not specified	ACTIVE_NOT_RECRUITING	Organizing Pneumonia in Lung Transplant Recipients, a Restrospective Exploratory Study (OPIL-Study)
NCT02032381	Not specified	COMPLETED	Prospective Study of Belated Pulmonary Complications Occurring in Children Treated With Allogeneic Hematopoietic Stem Cells.
NCT04054622	Not specified	UNKNOWN	Patient-ventilator Asychrony During Non-invasive Ventilation When COPD Patients Doing Exercise
NCT04489758	Not specified	COMPLETED	4DX for the Diagnosis of Constrictive Bronchiolitis
NCT04511884	Not specified	UNKNOWN	Screening Microorganism of Cryptogenic Mechanical Pneumonia Through Next Generation Sequencing to Lung Tissue Fluid
NCT04813575	Not specified	UNKNOWN	COVID-19 Pathophysiology of Long Term Implications

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
MONTELUKAST	4	1
BARDOXOLONE METHYL	3	1
CHEMBL4460360	0	1
CHEMBL5205741	0	1