Idiopathic acute eosinophilic pneumonia

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:724J82
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Idiopathic acute eosinophilic pneumonia (IAEP) is a rare lung condition where a type of white blood cell called an eosinophil builds up in the lungs for no clearly identified reason. 'Idiopathic' means doctors cannot find a specific cause, though in many cases a recent change in smoking habits, exposure to dust, or a new environment seems to trigger it. The lungs become inflamed, making it hard to breathe properly and causing the body to struggle to get enough oxygen. People with IAEP usually become very sick very quickly — often within days to a few weeks. Common symptoms include a high fever, a dry cough, chest pain, and severe shortness of breath that can become life-threatening if not treated promptly. Many patients end up needing oxygen support or even a breathing machine in the hospital. Blood tests and a procedure called bronchoalveolar lavage (where doctors rinse and sample fluid from the lungs) are used to confirm the diagnosis. The good news is that IAEP responds very well to corticosteroids, a type of anti-inflammatory medicine. Most patients recover fully and do not have the disease come back, especially if they avoid the trigger. Early recognition and treatment are key to a good outcome.

Also known as:

Key symptoms:

Sudden, severe shortness of breathHigh feverDry coughChest pain or tightnessFeeling very tired or weakLow oxygen levels in the bloodRapid breathingMuscle achesNight sweatsLoss of appetite

Clinical phenotype terms (11)— hover any for plain English
Abnormal eosinophil morphologyHP:0001879Abnormal pleura morphologyHP:0002103Abnormal pattern of respirationHP:0002793
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Idiopathic acute eosinophilic pneumonia.

View clinical trials →

No actively recruiting trials found for Idiopathic acute eosinophilic pneumonia at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Idiopathic acute eosinophilic pneumonia community →

No specialists are currently listed for Idiopathic acute eosinophilic pneumonia.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Idiopathic acute eosinophilic pneumonia.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Idiopathic acute eosinophilic pneumoniaForum →

No community posts yet. Be the first to share your experience with Idiopathic acute eosinophilic pneumonia.

Start the conversation →

Latest news about Idiopathic acute eosinophilic pneumonia

No recent news articles for Idiopathic acute 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.What do you think triggered my IAEP, and how can I avoid it in the future?,How long will I need to take steroids, and what side effects should I watch for?,How will you know when my lungs have fully recovered, and what tests will you use?,Is there a risk that IAEP will come back, and what warning signs should bring me to the emergency room?,Do I need to avoid smoking or other environmental exposures permanently?,Should I see a lung specialist (pulmonologist) for follow-up after I leave the hospital?,Are there any clinical trials or research studies I should know about?

Common questions about Idiopathic acute eosinophilic pneumonia

What is Idiopathic acute eosinophilic pneumonia?

Idiopathic acute eosinophilic pneumonia (IAEP) is a rare lung condition where a type of white blood cell called an eosinophil builds up in the lungs for no clearly identified reason. 'Idiopathic' means doctors cannot find a specific cause, though in many cases a recent change in smoking habits, exposure to dust, or a new environment seems to trigger it. The lungs become inflamed, making it hard to breathe properly and causing the body to struggle to get enough oxygen. People with IAEP usually become very sick very quickly — often within days to a few weeks. Common symptoms include a high feve

How is Idiopathic acute eosinophilic pneumonia inherited?

Idiopathic acute eosinophilic pneumonia follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Idiopathic acute eosinophilic pneumonia typically begin?

Typical onset of Idiopathic acute eosinophilic pneumonia is adult. Age of onset can vary across affected individuals.