Desquamative interstitial pneumonia
diseaseOn this page
Also known as DIPinterstitial pneumonitis, desquamative, familialRBILDrespiratory bronchiolitis interstitial lung disease
Summary
Desquamative interstitial pneumonia (MONDO:0009887) is a disease and 2 clinical trials. Top therapeutic interventions include 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
- Clinical trials: 2
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | desquamative interstitial pneumonia |
| Mondo ID | MONDO:0009887 |
| MeSH | C562470 |
| OMIM | 263000 |
| Orphanet | 98852 |
| DOID | DOID:0050158 |
| ICD-10-CM | J84.117 |
| ICD-11 | 1620001155 |
| NCIT | C35288 |
| SNOMED CT | 8549006 |
| UMLS | C0238378 |
| MedGen | 65962 |
| GARD | 0016864 |
| Is cancer (heuristic) | no |
Also known as: desquamative interstitial pneumonia · DIP · interstitial pneumonitis, desquamative, familial · RBILD · respiratory bronchiolitis interstitial lung disease
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 › desquamative interstitial pneumonia
Related subtypes (9): lymphoid interstitial pneumonia, cryptogenic organizing 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: 2.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 1 |
| Not specified | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT02036970 | PHASE2 | COMPLETED | Bardoxolone Methyl Evaluation in Patients With Pulmonary Hypertension (PH) - LARIAT |
| NCT02019641 | Not specified | COMPLETED | The NIH Exercise Therapy for Advanced Lung Disease Trials: Response and Adaptation to Aerobic Exercise in Patients With Interstitial Lung Disease |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| BARDOXOLONE METHYL | 3 | 1 |
| CHEMBL4460360 | 0 | 1 |
| CHEMBL5205741 | 0 | 1 |
Related Atlas pages
- Drugs: Bardoxolone Methyl