Overview
Idiopathic chronic eosinophilic pneumonia (ICEP), also known simply as chronic eosinophilic pneumonia or Carrington disease, is a rare lung condition in which a type of white blood cell called eosinophils builds up in the lungs and causes inflammation. The word "idiopathic" means the exact cause is unknown. This disease is not inherited and is not caused by an infection or a known trigger, though it is sometimes seen in people with asthma or allergies. The main symptoms include a persistent cough, difficulty breathing, fever, night sweats, weight loss, and a general feeling of being unwell. Some people also experience chest pain or wheezing. Symptoms usually develop gradually over weeks to months. A hallmark feature on chest imaging is areas of cloudiness in the outer parts of the lungs, sometimes described as the "photographic negative of pulmonary edema." The good news is that ICEP responds very well to corticosteroid treatment, such as prednisone. Most patients feel dramatically better within days to weeks of starting steroids. However, the disease tends to relapse when steroids are reduced or stopped, so many patients need long-term low-dose treatment. In some cases, steroid-sparing medications may be considered. While the disease can be frustrating because of its tendency to come back, it is generally not life-threatening and most people maintain a good quality of life with proper management.
Also known as:
Key symptoms:
Persistent coughShortness of breathFeverNight sweatsUnexplained weight lossGeneral feeling of being unwell or fatiguedWheezingChest pain or tightnessDifficulty exercising due to breathlessnessWorsening asthma symptomsLoss of appetiteBody aches
Clinical phenotype terms (27)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
1 availableImatinib Mesylate
Adult patients with hypereosinophilic syndrome (HES) and/or chronic eosinophilic leukemia (CEL) who have the FIP1L1-PDGFRα fusion kinase (mutational analysis or fluorescence in situ hybridization [FIS…
Adult patients with hypereosinophilic syndrome (HES) and/or chronic eosinophilic leukemia (CEL) who have the FIP1L1-PDGFRα fusion kinase (mutational analysis or fluorescence in situ hybridization [FISH] demonstration of CHIC2 allele deletion) and for patients with HES and/or CEL who are FIP1L1-PDGFRα fusion kinase negative or unknown
Clinical Trials
View all trials with filters →No actively recruiting trials found for Idiopathic chronic eosinophilic pneumonia at this time.
New trials open frequently. Follow this disease to get notified.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Idiopathic chronic eosinophilic pneumonia.
Community
No community posts yet. Be the first to share your experience with Idiopathic chronic eosinophilic pneumonia.
Start the conversation →Latest news about Idiopathic chronic eosinophilic pneumonia
No recent news articles for Idiopathic chronic eosinophilic pneumonia.
Follow this condition to be notified when news becomes available.
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.How long will I need to take corticosteroids, and what side effects should I watch for?,What are the chances my disease will come back when we lower the steroid dose?,Are there steroid-sparing treatments that might work for me?,How often will I need follow-up blood tests and imaging?,Could my asthma and this lung condition be related, and how should both be managed together?,Are there any newer biologic therapies being studied for this condition?,What symptoms should prompt me to seek urgent medical care?
Common questions about Idiopathic chronic eosinophilic pneumonia
What is Idiopathic chronic eosinophilic pneumonia?
Idiopathic chronic eosinophilic pneumonia (ICEP), also known simply as chronic eosinophilic pneumonia or Carrington disease, is a rare lung condition in which a type of white blood cell called eosinophils builds up in the lungs and causes inflammation. The word "idiopathic" means the exact cause is unknown. This disease is not inherited and is not caused by an infection or a known trigger, though it is sometimes seen in people with asthma or allergies. The main symptoms include a persistent cough, difficulty breathing, fever, night sweats, weight loss, and a general feeling of being unwell. S
How is Idiopathic chronic eosinophilic pneumonia inherited?
Idiopathic chronic eosinophilic pneumonia follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Idiopathic chronic eosinophilic pneumonia typically begin?
Typical onset of Idiopathic chronic eosinophilic pneumonia is adult. Age of onset can vary across affected individuals.
Which specialists treat Idiopathic chronic eosinophilic pneumonia?
1 specialists and care centers treating Idiopathic chronic eosinophilic pneumonia are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.