Lymphoid interstitial pneumonia
diseaseOn this page
Also known as diffuse hyperplasia of bronchus-associated lymphoid tissueLIPlymphocytic interst. pneumonitislymphocytic interstitial pneumonialymphocytic interstitial pneumonitis
Summary
Lymphoid interstitial pneumonia (MONDO:0009537) is a disease and 12 clinical trials. Top therapeutic interventions include doxycycline anhydrous, metformin, 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: Unknown (Worldwide)
- Phenotypes (HPO): 34
- Clinical trials: 12
Clinical features
Signs & symptoms
Clinical features (HPO)
34 HPO clinical features (Orphanet curated; top 34 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0012735 | Cough | Very frequent (80-99%) |
| HP:0000964 | Eczematoid dermatitis | Frequent (30-79%) |
| HP:0001217 | Clubbing | Frequent (30-79%) |
| HP:0001508 | Failure to thrive | Frequent (30-79%) |
| HP:0002091 | Restrictive ventilatory defect | Frequent (30-79%) |
| HP:0002094 | Dyspnea | Frequent (30-79%) |
| HP:0002960 | Autoimmunity | Frequent (30-79%) |
| HP:0005948 | Multiple pulmonary cysts | Frequent (30-79%) |
| HP:0012378 | Fatigue | Frequent (30-79%) |
| HP:0012418 | Hypoxemia | Frequent (30-79%) |
| HP:0025179 | Ground-glass opacification on pulmonary HRCT | Frequent (30-79%) |
| HP:0030057 | Autoimmune antibody positivity | Frequent (30-79%) |
| HP:0030828 | Wheezing | Frequent (30-79%) |
| HP:0030830 | Crackles | Frequent (30-79%) |
| HP:0031691 | Severe viral infection | Frequent (30-79%) |
| HP:0032177 | Parenchymal consolidation | Frequent (30-79%) |
| HP:0045051 | Decreased DLCO | Frequent (30-79%) |
| HP:0000105 | Enlarged kidney | Occasional (5-29%) |
| HP:0000988 | Skin rash | Occasional (5-29%) |
| HP:0001097 | Keratoconjunctivitis sicca | Occasional (5-29%) |
| HP:0001370 | Rheumatoid arthritis | Occasional (5-29%) |
| HP:0001824 | Weight loss | Occasional (5-29%) |
| HP:0001945 | Fever | Occasional (5-29%) |
| HP:0002110 | Bronchiectasis | Occasional (5-29%) |
| HP:0002240 | Hepatomegaly | Occasional (5-29%) |
| HP:0002721 | Immunodeficiency | Occasional (5-29%) |
| HP:0003549 | Abnormality of connective tissue | Occasional (5-29%) |
| HP:0011947 | Respiratory tract infection | Occasional (5-29%) |
| HP:0025178 | Subpleural interstitial thickening | Occasional (5-29%) |
| HP:0025180 | Centrilobular ground-glass opacification on pulmonary HRCT | Occasional (5-29%) |
| HP:0030880 | Raynaud phenomenon | Occasional (5-29%) |
| HP:0030950 | Pulmonary venous hypertension | Occasional (5-29%) |
| HP:0100721 | Mediastinal lymphadenopathy | Occasional (5-29%) |
| HP:0002206 | Pulmonary fibrosis | Very rare (<1-4%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | lymphoid interstitial pneumonia |
| Mondo ID | MONDO:0009537 |
| MeSH | C562489 |
| OMIM | 247610 |
| Orphanet | 79128 |
| DOID | DOID:0050159 |
| ICD-10-CM | J84.2 |
| ICD-11 | 1140418798 |
| NCIT | C27558 |
| SNOMED CT | 44274007 |
| UMLS | C0264511 |
| MedGen | 82682 |
| GARD | 0016700 |
| MedDRA | 10062997 |
| Is cancer (heuristic) | no |
Also known as: diffuse hyperplasia of bronchus-associated lymphoid tissue · LIP · lymphocytic interst. pneumonitis · lymphocytic interstitial pneumonia · lymphocytic interstitial pneumonitis · lymphoid interstitial 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 › lymphoid interstitial pneumonia
Related subtypes (9): 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: 12.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 6 |
| PHASE2 | 4 |
| PHASE4 | 1 |
| EARLY_PHASE1 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT04839692 | PHASE4 | COMPLETED | Assessment and Augmentation of Lip Appearance in Specific Study Populations |
| NCT03529422 | PHASE2 | ACTIVE_NOT_RECRUITING | Durvalumab With Radiotherapy for Adjuvant Treatment of Intermediate Risk SCCHN |
| NCT03854032 | PHASE2 | ACTIVE_NOT_RECRUITING | Nivolumab and BMS986205 in Treating Patients With Stage II-IV Squamous Cell Cancer of the Head and Neck |
| NCT02036970 | PHASE2 | COMPLETED | Bardoxolone Methyl Evaluation in Patients With Pulmonary Hypertension (PH) - LARIAT |
| NCT03076281 | PHASE2 | TERMINATED | Metformin Hydrochloride and Doxycycline in Treating Patients With Head and Neck Squamous Cell Carcinoma |
| NCT03109873 | EARLY_PHASE1 | COMPLETED | Metformin Hydrochloride in Affecting Cytokines and Exosomes in Patients With Head and Neck Cancer |
| NCT06052501 | Not specified | RECRUITING | 3D Evaluation of the Lip Support in a Full-arch Implant-supported Rehabilitations |
| NCT04362891 | Not specified | COMPLETED | Four Hyaluronic Acid Fillers for Lip Augmentation |
| NCT04540913 | Not specified | COMPLETED | RHA® 3 Versus Restylane-L® for Lip Augmentation |
| NCT04753866 | Not specified | COMPLETED | Facial Scanning for Lip Support Assessment in Full Arch Implant Rehabilitations |
| NCT04843345 | Not specified | SUSPENDED | Sjogren’s Lung Study |
| NCT05610293 | Not specified | UNKNOWN | T1 Squamous Cell Carcinomas of the Lip |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| DOXYCYCLINE ANHYDROUS | 4 | 1 |
| METFORMIN | 4 | 1 |
| BARDOXOLONE METHYL | 3 | 1 |
| CHEMBL4460360 | 0 | 1 |
| CHEMBL5205741 | 0 | 1 |
Related Atlas pages
- Drugs: Doxycycline, Metformin, Bardoxolone Methyl