Idiopathic interstitial pneumonia
diseaseOn this page
Also known as idiopathic fibrosing alveolitisidiopathic interstitial pneumonitisIIPnoninfectious pneumonia
Summary
Idiopathic interstitial pneumonia (MONDO:0002429) is a disease (an umbrella term covering 10 Mondo subtypes) and 14 clinical trials. Top therapeutic interventions include bardoxolone methyl and cc-90001. A subtype of pneumonia — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Umbrella term: 10 Mondo subtypes
- Clinical trials: 14
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | idiopathic interstitial pneumonia |
| Mondo ID | MONDO:0002429 |
| MeSH | D054988 |
| Orphanet | 98300 |
| DOID | DOID:2797 |
| ICD-10-CM | J84.11 |
| NCIT | C35714 |
| SNOMED CT | 700249006 |
| UMLS | C2350236 |
| MedGen | 389939 |
| GARD | 0013337 |
| Is cancer (heuristic) | no |
Also known as: idiopathic fibrosing alveolitis · idiopathic interstitial pneumonitis · IIP · IIp · noninfectious pneumonia
Disease family
This is a subtype of 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
Related subtypes (10): pleuropneumonia, bacterial pneumonia, bronchopneumonia, contagious pleuropneumonia, eosinophilic pneumonia, recurrent pneumonia, viral pneumonia, pneumocystosis, acute fibrinous and organizing pneumonia, bronchiolocentric pattern of interstitial pneumonia
Subtypes (10): lymphoid interstitial pneumonia, desquamative 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: 14.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 10 |
| PHASE2 | 2 |
| PHASE1/PHASE2 | 1 |
| EARLY_PHASE1 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT02036970 | PHASE2 | COMPLETED | Bardoxolone Methyl Evaluation in Patients With Pulmonary Hypertension (PH) - LARIAT |
| NCT02594839 | PHASE1/PHASE2 | COMPLETED | Safety and Efficacy of Allogeneic Mesenchymal Stem Cells in Patients With Rapidly Progressive Interstitial Lung Disease |
| NCT03142191 | PHASE2 | TERMINATED | A Study to Evaluate the Efficacy and Safety of CC-90001 in Subjects With Idiopathic Pulmonary Fibrosis |
| NCT05365802 | EARLY_PHASE1 | RECRUITING | FAPI PET for Lung Fibrosis |
| NCT02951416 | Not specified | ACTIVE_NOT_RECRUITING | Clinical Course of Interstitial Lung Diseases: European IPF Registry and Biobank |
| NCT03041623 | Not specified | ACTIVE_NOT_RECRUITING | Japanese Idiopathic Interstitial Pneumonias Registry |
| NCT04896138 | Not specified | ENROLLING_BY_INVITATION | University of Virginia Natural History Study |
| NCT01088217 | Not specified | UNKNOWN | Role of Genetics in Idiopathic Pulmonary Fibrosis (IPF) |
| NCT01151527 | Not specified | TERMINATED | Peripheral Blood Biomarkers in Idiopathic Interstitial Pneumonias |
| NCT03747627 | Not specified | COMPLETED | BPF Genetics of ILD Study |
| NCT03836417 | Not specified | COMPLETED | Molecular Diagnosis of Idiopathic Interstitial Pneumonias: a Prospective Study |
| NCT04179058 | Not specified | UNKNOWN | Interstitial Pneumonia With Autoimmune Features: Evaluation of Connective Tissue Disease Incidence During Follow-up |
| NCT04511884 | Not specified | UNKNOWN | Screening Microorganism of Cryptogenic Mechanical Pneumonia Through Next Generation Sequencing to Lung Tissue Fluid |
| NCT05146934 | Not specified | COMPLETED | The Relationship Between Hormone Sensitivity and Imaging of Idiopathic Interstitial Pneumonia by Artificial Intelligence |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| BARDOXOLONE METHYL | 3 | 1 |
| CC-90001 | 2 | 1 |
| CHEMBL4460360 | 0 | 1 |
| CHEMBL5205741 | 0 | 1 |
Related Atlas pages
- Drugs: Bardoxolone Methyl