Acute zonal occult outer retinopathy

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1Active trials1Specialists8Treatment centers

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Overview

Acute Zonal Occult Outer Retinopathy, commonly known as AZOOR, is a rare eye condition that affects the outer layer of the retina — the light-sensitive tissue at the back of the eye. In AZOOR, one or more zones of the outer retina stop working properly, leading to blind spots and other vision changes. The condition most often affects young to middle-aged women, and it typically starts in one eye, though it can eventually involve both eyes. Patients usually notice the sudden appearance of blind spots (scotomas) in their field of vision, along with flashing lights (photopsia). Some people also experience a general dimming of vision or changes in how they see colors. The condition can be alarming because symptoms often come on quickly. In many cases, the visual loss stabilizes over time, but some patients experience progressive worsening. The exact cause of AZOOR is not fully understood. It is thought to involve an autoimmune or inflammatory process that damages the photoreceptor cells in the outer retina. There is currently no proven cure or standard treatment for AZOOR. Some doctors have tried corticosteroids or other immunosuppressive medications to reduce inflammation, but results have been mixed. Management focuses on monitoring vision changes and providing supportive care. Research is ongoing to better understand the disease and develop effective therapies.

Also known as:

Key symptoms:

Sudden blind spots in the field of visionFlashing lights or shimmering in vision (photopsia)Blurred or dimmed visionLoss of peripheral visionChanges in color visionSensitivity to lightWhite or pale spots on the retinaRapid onset of visual symptoms, often in one eye firstEnlarged blind spot on visual field testingGradual involvement of the other eye in some cases

Clinical phenotype terms (24)— hover any for plain English
Abnormality of refractionHP:0000539ScotomaHP:0000575Constriction of peripheral visual fieldHP:0001133Abnormal fundus fluorescein angiographyHP:0030604Blind-spot enlargementHP:0030644PhotopsiaHP:0030786Marcus Gunn pupilHP:0200057Central scotomaHP:0000603Retinal pigment epithelial atrophyHP:0007722Retinal pigment epithelial mottlingHP:0007814VitritisHP:0011531HemianopiaHP:0012377Vitreous inflammatory cellsHP:0030662
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 ↗

FDA & Trial Timeline

1 event
May 2019New Non-invasive Modalities for Assessing Retinal Structure and Function

Randy Kardon — NA

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Acute zonal occult outer retinopathy.

1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

1 recruitingView all trials with filters →
N/A1 trial
New Non-invasive Modalities for Assessing Retinal Structure and Function
N/A
Actively Recruiting
PI: Randy H Kardon, MD, PhD (University of Iowa Department of Ophthalmology) · Sites: Iowa City, Iowa · Age: 1899 yrs

Specialists

1 foundView all specialists →
RP
Randy H Kardon, MD, PhD
IOWA CITY, IA
Specialist
PI on 1 active trial

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 Acute zonal occult outer retinopathy.

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Community

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Latest news about Acute zonal occult outer retinopathy

Disease timeline:

New recruiting trial: New Non-invasive Modalities for Assessing Retinal Structure and Function

A new clinical trial is recruiting patients for Acute zonal occult outer retinopathy

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

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 much of my retina is currently affected, and is the disease active or stable?,What tests will you use to monitor my condition over time, and how often should I be seen?,Are there any treatments you would recommend trying in my specific case?,What are the chances that my other eye will also be affected?,Should I be referred to a low vision rehabilitation specialist?,Are there any clinical trials or research studies I could participate in?,What symptoms should prompt me to seek urgent care between appointments?

Common questions about Acute zonal occult outer retinopathy

What is Acute zonal occult outer retinopathy?

Acute Zonal Occult Outer Retinopathy, commonly known as AZOOR, is a rare eye condition that affects the outer layer of the retina — the light-sensitive tissue at the back of the eye. In AZOOR, one or more zones of the outer retina stop working properly, leading to blind spots and other vision changes. The condition most often affects young to middle-aged women, and it typically starts in one eye, though it can eventually involve both eyes. Patients usually notice the sudden appearance of blind spots (scotomas) in their field of vision, along with flashing lights (photopsia). Some people also

How is Acute zonal occult outer retinopathy inherited?

Acute zonal occult outer retinopathy follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Acute zonal occult outer retinopathy typically begin?

Typical onset of Acute zonal occult outer retinopathy is adult. Age of onset can vary across affected individuals.

Are there clinical trials for Acute zonal occult outer retinopathy?

Yes — 1 recruiting clinical trial is currently listed for Acute zonal occult outer retinopathy on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Acute zonal occult outer retinopathy?

1 specialists and care centers treating Acute zonal occult outer retinopathy are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.