Overview
Eosinophilic angiocentric fibrosis (EAF) is an extremely rare condition that affects the upper airways, most commonly the nose, sinuses, and the area around the eye sockets (orbits). It is sometimes also called eosinophilic angiocentric fibrosis of the upper respiratory tract. In this disease, a specific type of white blood cell called an eosinophil builds up in the tissues of the nose and sinuses, causing inflammation and scarring (fibrosis) around small blood vessels. Over time, this scarring can lead to blockage of the nasal passages, difficulty breathing through the nose, and destruction of nearby structures. The most common symptoms include progressive nasal obstruction (stuffy nose that keeps getting worse), facial pain or pressure, and sometimes a visible mass or swelling in the nose or around the eye. The condition tends to progress slowly over months to years. It is not cancerous, but it can be locally destructive, meaning it can damage the tissues where it grows. Because EAF is so rare, there is no single established treatment. Surgery to remove the abnormal tissue is the most common approach, but the disease can come back after surgery. Corticosteroids (anti-inflammatory medications) and other immunosuppressive drugs have been tried with varying success. Some patients have been treated with rituximab or other targeted therapies. The condition is considered benign (not life-threatening), but it can significantly affect quality of life due to chronic nasal obstruction and the need for repeated treatments. EAF is sometimes considered to be on a spectrum with a related condition called IgG4-related disease, though this relationship is still being studied.
Also known as:
Key symptoms:
Progressive nasal blockage or stuffinessDifficulty breathing through the noseFacial pain or pressureNasal mass or swelling inside the noseSwelling around the eye socketNosebleedsLoss of sense of smellNasal discharge or crustingDestruction of nasal cartilage or boneTearing or watery eyes if the tear duct is blockedFacial deformity in advanced casesRecurrent sinus infections
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Eosinophilic angiocentric fibrosis.
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Specialists
View all specialists →No specialists are currently listed for Eosinophilic angiocentric fibrosis.
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 Eosinophilic angiocentric fibrosis.
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Caregiver Resources
NORD Caregiver Resources
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Mental Health Support
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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 extensive is the disease in my case, and what areas are affected?,Is surgery recommended, and what is the chance the disease will come back after surgery?,What medications might help slow the disease, and what are their side effects?,Could this be related to IgG4-related disease, and should I be tested for that?,How often will I need follow-up imaging or check-ups?,Are there any clinical trials or new treatments being studied for this condition?,What symptoms should prompt me to seek urgent medical attention?
Common questions about Eosinophilic angiocentric fibrosis
What is Eosinophilic angiocentric fibrosis?
Eosinophilic angiocentric fibrosis (EAF) is an extremely rare condition that affects the upper airways, most commonly the nose, sinuses, and the area around the eye sockets (orbits). It is sometimes also called eosinophilic angiocentric fibrosis of the upper respiratory tract. In this disease, a specific type of white blood cell called an eosinophil builds up in the tissues of the nose and sinuses, causing inflammation and scarring (fibrosis) around small blood vessels. Over time, this scarring can lead to blockage of the nasal passages, difficulty breathing through the nose, and destruction o
How is Eosinophilic angiocentric fibrosis inherited?
Eosinophilic angiocentric fibrosis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Eosinophilic angiocentric fibrosis typically begin?
Typical onset of Eosinophilic angiocentric fibrosis is adult. Age of onset can vary across affected individuals.