Idiopathic chronic eosinophilic pneumonia

Summary

Idiopathic chronic eosinophilic pneumonia (MONDO:0017363) is a disease. A subtype of idiopathic eosinophilic 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 (Iceland) [Orphanet-validated]
• Phenotypes (HPO): 27

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.23	Iceland	Validated

Signs & symptoms

Clinical features (HPO)

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

HPO ID	Term	Frequency
HP:0031983	Abnormal pulmonary thoracic imaging finding	Very frequent (80-99%)
HP:0032177	Parenchymal consolidation	Very frequent (80-99%)
HP:0001047	Atopic dermatitis	Frequent (30-79%)
HP:0001974	Leukocytosis	Frequent (30-79%)
HP:0002091	Restrictive ventilatory defect	Frequent (30-79%)
HP:0002094	Dyspnea	Frequent (30-79%)
HP:0002099	Asthma	Frequent (30-79%)
HP:0002960	Autoimmunity	Frequent (30-79%)
HP:0003212	Increased circulating IgE level	Frequent (30-79%)
HP:0003565	Elevated erythrocyte sedimentation rate	Frequent (30-79%)
HP:0006516	Hypersensitivity pneumonitis	Frequent (30-79%)
HP:0011227	Elevated circulating C-reactive protein concentration	Frequent (30-79%)
HP:0012418	Hypoxemia	Frequent (30-79%)
HP:0030828	Wheezing	Frequent (30-79%)
HP:0030830	Crackles	Frequent (30-79%)
HP:0031246	Nonproductive cough	Frequent (30-79%)
HP:0032017	Sputum eosinophilia	Frequent (30-79%)
HP:0032061	Hypereosinophilia	Frequent (30-79%)
HP:0001824	Weight loss	Occasional (5-29%)
HP:0001945	Fever	Occasional (5-29%)
HP:0002829	Arthralgia	Occasional (5-29%)
HP:0025406	Asthenia	Occasional (5-29%)
HP:0030166	Night sweats	Occasional (5-29%)
HP:0002202	Pleural effusion	Very rare (<1-4%)
HP:0011024	Abnormality of the gastrointestinal tract	Very rare (<1-4%)
HP:0011354	Generalized abnormality of skin	Very rare (<1-4%)
HP:0100750	Atelectasis	Very rare (<1-4%)

Identifiers

Disease identifiers

Field	Value
Canonical name	idiopathic chronic eosinophilic pneumonia
Mondo ID	MONDO:0017363
MeSH	C535590
Orphanet	2902
ICD-11	958353326
UMLS	C2930941
MedGen	443938
GARD	0001130
NORD	967
Is cancer (heuristic)	no

Also known as: Carrington syndrome · Carrington’s disease · Carrington’s pulmonary eosinophilia · Chronic Eosinophilic Pneumonia · chronic eosinophilic pneumonia · chronic eosinophilic pneumonia (CEP) · chronic idiopathic eosinophilic pneumonia · eosinophilic idiopathic chronic pneumopathy

Disease family

This is a subtype of idiopathic eosinophilic 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 › eosinophilic pneumonia › idiopathic eosinophilic pneumonia › idiopathic chronic eosinophilic pneumonia

Related subtypes (1): idiopathic acute eosinophilic pneumonia

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: 0.