Acute interstitial pneumonia

disease

On this page

Also known as acute interstitial pneumonitisAIPHamman-rich syndrome

Summary

Acute interstitial pneumonia (MONDO:0019203) is a disease and 1 clinical trial. 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

Prevalence: 1-9 / 100 000 (Europe) [Orphanet-validated]
Phenotypes (HPO): 33
Clinical trials: 1

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Point prevalence	1-9 / 100 000	3.8	Europe	Validated

Signs & symptoms

Clinical features (HPO)

33 HPO clinical features (Orphanet curated; top 33 by frequency):

HPO ID	Term	Frequency
HP:0002094	Dyspnea	Very frequent (80-99%)
HP:0002110	Bronchiectasis	Very frequent (80-99%)
HP:0002113	Pulmonary infiltrates	Very frequent (80-99%)
HP:0002878	Respiratory failure	Very frequent (80-99%)
HP:0012418	Hypoxemia	Very frequent (80-99%)
HP:0025177	Peribronchovascular interstitial thickening	Very frequent (80-99%)
HP:0025179	Ground-glass opacification on pulmonary HRCT	Very frequent (80-99%)
HP:0025392	Nodular pattern on pulmonary HRCT	Very frequent (80-99%)
HP:0025393	Reticulonodular pattern on pulmonary HRCT	Very frequent (80-99%)
HP:0030879	Interlobular septal thickening on pulmonary HRCT	Very frequent (80-99%)
HP:0032177	Parenchymal consolidation	Very frequent (80-99%)
HP:0000822	Hypertension	Frequent (30-79%)
HP:0000961	Cyanosis	Frequent (30-79%)
HP:0001945	Fever	Frequent (30-79%)
HP:0002202	Pleural effusion	Frequent (30-79%)
HP:0002789	Tachypnea	Frequent (30-79%)
HP:0012378	Fatigue	Frequent (30-79%)
HP:0030830	Crackles	Frequent (30-79%)
HP:0031246	Nonproductive cough	Frequent (30-79%)
HP:0045051	Decreased DLCO	Frequent (30-79%)
HP:0001698	Pericardial effusion	Occasional (5-29%)
HP:0002206	Pulmonary fibrosis	Occasional (5-29%)
HP:0002716	Lymphadenopathy	Occasional (5-29%)
HP:0002829	Arthralgia	Occasional (5-29%)
HP:0003259	Elevated circulating creatinine concentration	Occasional (5-29%)
HP:0003326	Myalgia	Occasional (5-29%)
HP:0003565	Elevated erythrocyte sedimentation rate	Occasional (5-29%)
HP:0011227	Elevated circulating C-reactive protein concentration	Occasional (5-29%)
HP:0012398	Peripheral edema	Occasional (5-29%)
HP:0031631	Subpleural honeycombing	Occasional (5-29%)
HP:0031851	Reduced hematocrit	Occasional (5-29%)
HP:0100749	Chest pain	Occasional (5-29%)
HP:0100750	Atelectasis	Occasional (5-29%)

Identifiers

Disease identifiers

Field	Value
Canonical name	acute interstitial pneumonia
Mondo ID	MONDO:0019203
Orphanet	79126
DOID	DOID:2800
ICD-10-CM	J84.114
ICD-11	2116884221
NCIT	C35806
SNOMED CT	236302005
UMLS	C0085786
MedGen	39340
GARD	0012835
MedDRA	10066728
Is cancer (heuristic)	no

Also known as: acute interstitial pneumonitis · AIP · Hamman-rich syndrome

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 › acute interstitial pneumonia

Related subtypes (9): lymphoid interstitial pneumonia, desquamative interstitial pneumonia, cryptogenic organizing pneumonia, combined pulmonary fibrosis-emphysema syndrome, 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: 1.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT02036970	PHASE2	COMPLETED	Bardoxolone Methyl Evaluation in Patients With Pulmonary Hypertension (PH) - LARIAT

Drugs tested across these trials (top 30)

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

Drugs: Bardoxolone Methyl