ORPHA:2902

Idiopathic chronic eosinophilic pneumonia

Synonyms: Chronic eosinophilic pneumonia

Type: Disease
Group: Disorder
Inheritance: Not applicable
Age of onset: All ages
Status: active

A rare, severe, interstitial lung disease characterized by insidious onset with subacute or chronic non-specific respiratory manifestations (dyspnea, cough, wheezing) often associated with systemic manifestations (fatigue, malaise, weight loss) and a history of asthma (up to half of patients). Eosinophilia is present in most cases, usually in excess of 1000 cells/mm3.

Names in Orphadata languages

12 of 12 languages

Lang	Name	Synonyms
en	Idiopathic chronic eosinophilic pneumonia	Chronic eosinophilic pneumonia
fr	Pneumopathie chronique idiopathique à éosinophiles	Maladie de Carrington · Pneumonie éosinophile chronique
de	Pneumopathie, eosinophile idiopathische, chronische	Carrington-Krankheit
es	Neumonía eosinofílica crónica idiopática	NECI · Síndrome de Carrington
it	Polmonite cronica idiopatica con eosinofili	Sindrome di Carrington
nl	Idiopathische chronische eosinofiele pneumonie	Chronische eosinofiele pneumonie
pt	Pneumopatia eosinofílica crónica idiopática	Síndrome Carrington
pl	Idiopatyczne przewlekłe eozynofilowe zapalenie płuc	Przewlekłe eozynofilowe zapalenie płuc
cs	Idiopatická chronická eozinofilní pneumonie	Chronická eozinofilní pneumonie
uk	Ідіопатична хронічна еозинофільна пневмонія	Хронічна еозинофільна пневмонія
zh	特发性慢性嗜酸细胞性肺炎	慢性嗜酸粒细胞性肺炎
tr	İdiyopatik kronik eozinofilik pnömoni	Kronik eozinofilik pnömoni

Definition in:

Cross-references

qualifier = Orphanet mapping relation

Source	Code	Qualifier	ICD relation	Validation
ICD-10	J82	NTBT	attributed	validated
ICD-11	CB02.11	E	specific	validated
MONDO	0017363	E		validated
UMLS	C2930941	E		validated
GARD	1130	E		validated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

Classification

Preferential parent (Orphanet linearisation):

ORPHA:97955Rare respiratory disease

Parents in the classifications (2):

ORPHA:182101Idiopathic eosinophilic pneumonia
rare respiratory diseases; rare transplant-related disorders
ORPHA:98052Rare allergic respiratory disease
rare allergic disease

No children.

Genes

0 direct

Orphanet records no gene directly on this code.

Prevalence and incidence

2 records

Type	Class	Value	Area	Qualification	Validation	Source
Point prevalence	Unknown	—	Worldwide	Class only	Not yet validated	ORPHANET
Annual incidence	1-9 / 1 000 000	0.23 / 100 000	Iceland	Value and class	Validated	17277407[PMID]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

HPO phenotypes

27 annotations · validated 2021-08-26

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

Run the differential on the 2 obligate / very frequent terms

Annotation source: 29735405[PMID]_33898133[PMID]

Sources for this card

Orphanet page: orpha.net/en/disease/detail/2902
This record as JSON: /data/d/2902.json