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Start free with EleplanIdiopathic chronic eosinophilic pneumonia
ORPHA:2902Disease
Also called Chronic eosinophilic pneumonia
What it is
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.
Key facts
- Prevalence
- 1-9 / 1 000 000 (annual incidence, Iceland)
- Age of onset
- All ages
- Inheritance
- Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
16- Asthma
- Atopic dermatitis
- Autoimmunity
- Crackles
- Dyspnea
- Elevated circulating C-reactive protein concentration
- Elevated erythrocyte sedimentation rate
- Hypereosinophilia
- Hypersensitivity pneumonitis
- Hypoxemia
- Increased circulating IgE concentration
- Increased total leukocyte count
- Nonproductive cough
- Restrictive ventilatory defect
- Sputum eosinophilia
- Wheezing
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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