Fuchs heterochromic iridocyclitis

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Overview

Fuchs heterochromic iridocyclitis (FHI), also known as Fuchs heterochromic uveitis or Fuchs uveitis syndrome, is a chronic, low-grade inflammatory condition of the eye that typically affects one eye (unilateral). It accounts for approximately 2–11% of all uveitis cases. The disease is characterized by heterochromia (a difference in iris color between the two eyes), with the affected eye usually appearing lighter due to iris stromal atrophy. Patients develop a chronic, mild anterior uveitis (inflammation of the front part of the eye) that is often painless and may go unnoticed for years. Characteristic fine, stellate (star-shaped) keratic precipitates are scattered diffusely across the corneal endothelium, and the eye may show mild flare in the anterior chamber without significant redness. The most important complications of Fuchs heterochromic iridocyclitis are cataract formation and secondary glaucoma. Cataracts develop in the majority of affected individuals over time and are often the presenting complaint that leads to diagnosis. Secondary open-angle glaucoma occurs in a significant proportion of patients and can be difficult to manage. Vitreous opacities (floaters) are also commonly observed. A distinctive clinical sign is the occurrence of fine hemorrhage in the anterior chamber (Amsler sign) following paracentesis or minor surgical trauma. The exact cause of FHI remains debated, though evidence increasingly suggests an association with rubella virus infection in many cases, supported by the detection of rubella virus-specific antibodies in the aqueous humor. The condition is considered sporadic and is not inherited. Treatment is primarily directed at managing complications rather than the inflammation itself, as the chronic low-grade uveitis typically does not respond well to corticosteroids and rarely requires aggressive anti-inflammatory therapy. Cataract surgery generally yields good visual outcomes, though careful monitoring for glaucoma is essential. Glaucoma may require medical therapy or surgical intervention. There is no cure for the underlying condition, and long-term ophthalmologic follow-up is recommended.

Also known as:

Clinical phenotype terms— hover any for plain English:

Iris atrophyHP:0001089Corneal keratic precipitatesHP:0025341Anterior chamber inflammatory cellsHP:0031701Vitreous floatersHP:0100832Koeppe nodulesHP:0034820Bussaca nodulesHP:0034821PapilledemaHP:0001085Chorioretinal scarHP:0007777Ocular hypertensionHP:0007906Red eyeHP:0025337Abnormal best corrected visual acuity testHP:0030534AnisocoriaHP:0009916
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 Fuchs heterochromic iridocyclitis.

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No actively recruiting trials found for Fuchs heterochromic iridocyclitis at this time.

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No specialists are currently listed for Fuchs heterochromic iridocyclitis.

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 Fuchs heterochromic iridocyclitis.

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Common questions about Fuchs heterochromic iridocyclitis

What is Fuchs heterochromic iridocyclitis?

Fuchs heterochromic iridocyclitis (FHI), also known as Fuchs heterochromic uveitis or Fuchs uveitis syndrome, is a chronic, low-grade inflammatory condition of the eye that typically affects one eye (unilateral). It accounts for approximately 2–11% of all uveitis cases. The disease is characterized by heterochromia (a difference in iris color between the two eyes), with the affected eye usually appearing lighter due to iris stromal atrophy. Patients develop a chronic, mild anterior uveitis (inflammation of the front part of the eye) that is often painless and may go unnoticed for years. Charac

How is Fuchs heterochromic iridocyclitis inherited?

Fuchs heterochromic iridocyclitis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Fuchs heterochromic iridocyclitis typically begin?

Typical onset of Fuchs heterochromic iridocyclitis is adult. Age of onset can vary across affected individuals.