Bietti crystalline dystrophy

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ORPHA:41751OMIM:210370H35.5
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6Active trials5Specialists8Treatment centers

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

Bietti crystalline dystrophy (also called Bietti crystalline corneoretinal dystrophy or BCD) is a rare inherited eye disease that slowly damages the retina — the light-sensitive layer at the back of the eye. Tiny shiny crystal deposits build up in the retina and sometimes in the outer edge of the cornea (the clear front part of the eye). Over time, the cells in the retina break down, making it harder and harder to see. The disease is named after the Italian doctor G.B. Bietti, who first described it in 1937. The most common symptoms are difficulty seeing in dim light or at night (night blindness), loss of side (peripheral) vision, and gradually worsening central vision. Most people notice their first symptoms in their 20s or 30s, though the timing can vary. The disease progresses slowly over decades and can eventually lead to significant vision loss or legal blindness. Right now there is no cure for Bietti crystalline dystrophy, and no FDA-approved treatment exists to stop or reverse the damage. Care focuses on monitoring vision changes, using low-vision aids, and protecting the eyes. However, researchers are actively studying gene therapy and other approaches, which gives real hope for future treatments.

Also known as:

Key symptoms:

Difficulty seeing in low light or at night (night blindness)Loss of side (peripheral) visionBlurry or reduced central vision over timeShiny crystal deposits visible in the retina on eye examSensitivity to bright light (photophobia)Colors may appear less vividDifficulty reading or recognizing faces as disease progressesGradual shrinking of the visual fieldPossible crystal deposits at the edge of the cornea (visible to an eye doctor)

Clinical phenotype terms (19)— hover any for plain English
Decreased light- and dark-adapted electroretinogram amplitudeHP:0000654Constriction of peripheral visual fieldHP:0001133Retinal pigment epithelial atrophyHP:0007722Crystalline corneal dystrophyHP:0007760Choriocapillaris atrophyHP:0030491Subretinal depositsHP:0031528Chorioretinal atrophyHP:0000533Central scotomaHP:0000603Large central visual field defectHP:0001129Severely reduced visual acuityHP:0001141Retinal pigment epithelial mottlingHP:0007814Cystoid macular edemaHP:0011505Retinal thinning on OCTHP:0030329Paracentral scotomaHP:0030528
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

10 events
Mar 2026Compassionate Administration of ZVS101e Injection for Extended Treatment

Chigenovo Co., Ltd — NA

TrialENROLLING BY INVITATION
Jun 2025BCD to Measure the ED95 of Remimazolam-Assisted Sedation in Arteriovenous Fistula Creation Surgery

Gang Chen — PHASE4

TrialNOT YET RECRUITING
Mar 2025A Study of Pharmacokinetics, Safety, and Immunogenicity of BCD-057 100 mg/mL, BCD-057 50 mg/mL, and Humira 100 mg/mL in Healthy Subjects

Biocad — PHASE1

TrialRECRUITING
Jan 2025Dara-BCD for Rare MGRS

Peking University People's Hospital — PHASE2

TrialNOT YET RECRUITING
Dec 2024Efficacy and Safety of ZVS101e in Patients With Bietti 's Crystalline Dystrophy

Chigenovo Co., Ltd — PHASE3

TrialRECRUITING
Dec 2024Study to Evaluate the Efficacy and Safety of VGR-R01 Gene Therapy in Patients With Bietti Crystalline Dystrophy

Shanghai Vitalgen BioPharma Co., Ltd. — PHASE3

TrialACTIVE NOT RECRUITING
Mar 2024Safety and Efficacy Study of NGGT001 in Bietti Crystalline Corneoretinal Dystrophy Subjects

NGGT (Suzhou) Biotechnology Co., Ltd. — PHASE1, PHASE2

TrialACTIVE NOT RECRUITING
Mar 2023Safety and Tolerability of VGR-R01 for Patients With Bietti Crystalline Dystrophy

Shanghai Vitalgen BioPharma Co., Ltd. — PHASE1

TrialACTIVE NOT RECRUITING
Feb 2023Safety and Efficacy of ZVS101e in Patients With Bietti 's Crystalline Dystrophy

Chigenovo Co., Ltd — PHASE1, PHASE2

TrialACTIVE NOT RECRUITING
Feb 2023Safety and Efficacy Study of NGGT001 in Bietti Crystalline Corneoretinal Dystrophy

Xiamen Ophthalmology Center Affiliated to Xiamen University — EARLY_PHASE1

TrialACTIVE NOT RECRUITING

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

Treatments

No FDA-approved treatments are currently listed for Bietti crystalline dystrophy.

6 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

6 recruitingView all trials with filters →
Phase 32 trials
Efficacy and Safety of ZVS101e in Patients With Bietti 's Crystalline Dystrophy
Phase 3
Actively Recruiting
PI: Jinlu Zhang, MD (Chigenovo Co., Ltd) · Sites: Beijing, Beijing Municipality; Guangzhou, Guangdong +6 more · Age: 1899 yrs
Study to Evaluate the Efficacy and Safety of VGR-R01 Gene Therapy in Patients With Bietti Crystalline Dystrophy
Phase 3
Active
· Sites: Shanghai, Shanghai Municipality · Age: 1869 yrs
Phase 11 trial
Safety and Tolerability of VGR-R01 for Patients With Bietti Crystalline Dystrophy
Phase 1
Active
PI: Wenbin Wei (Beijing Tongren Hospital) · Sites: Shanghai, Shanghai Municipality · Age: 1869 yrs
N/A1 trial
Compassionate Administration of ZVS101e Injection for Extended Treatment
N/A
Enrolling by Invitation
· Sites: Beijing, Beijing Municipality; Guangzhou, Guangdong +3 more · Age: 1875 yrs

Specialists

5 foundView all specialists →
WW
Wenbin Wei
Specialist
PI on 3 active trials
JM
Jinlu Zhang, MD
Specialist
PI on 1 active trial
JM
James F Hejtmancik, M.D.
Specialist
PI on 2 active trials
PB
Pamela Becker
Specialist
PI on 7 active trials16 Bietti crystalline dystrophy publications
XM
Xiaorong Li, MD
ALBUQUERQUE, NM
Specialist
PI on 2 active trials

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 Bietti crystalline dystrophy.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Bietti crystalline dystrophy

Disease timeline:

New recruiting trial: A Study of Pharmacokinetics, Safety, and Immunogenicity of BCD-057 100 mg/mL, BCD-057 50 mg/mL, and Humira 100 mg/mL in Healthy Subjects

A new clinical trial is recruiting patients for Bietti crystalline dystrophy

New recruiting trial: Efficacy and Safety of ZVS101e in Patients With Bietti 's Crystalline Dystrophy

A new clinical trial is recruiting patients for Bietti crystalline dystrophy

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 quickly do you expect my vision to change, and what signs should I watch for?,Should I have genetic testing, and should my family members be tested too?,Are there any clinical trials for gene therapy or other treatments that I might qualify for?,What low-vision aids or rehabilitation services do you recommend for me right now?,How often should I have eye exams, and what tests will you use to track the disease?,Are there any lifestyle changes — like diet or sun protection — that might help protect my vision?,Where can I find support groups or patient communities for people with Bietti crystalline dystrophy?

Common questions about Bietti crystalline dystrophy

What is Bietti crystalline dystrophy?

Bietti crystalline dystrophy (also called Bietti crystalline corneoretinal dystrophy or BCD) is a rare inherited eye disease that slowly damages the retina — the light-sensitive layer at the back of the eye. Tiny shiny crystal deposits build up in the retina and sometimes in the outer edge of the cornea (the clear front part of the eye). Over time, the cells in the retina break down, making it harder and harder to see. The disease is named after the Italian doctor G.B. Bietti, who first described it in 1937. The most common symptoms are difficulty seeing in dim light or at night (night blindn

How is Bietti crystalline dystrophy inherited?

Bietti crystalline dystrophy follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Bietti crystalline dystrophy typically begin?

Typical onset of Bietti crystalline dystrophy is adult. Age of onset can vary across affected individuals.

Are there clinical trials for Bietti crystalline dystrophy?

Yes — 6 recruiting clinical trials are currently listed for Bietti crystalline dystrophy on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Bietti crystalline dystrophy?

5 specialists and care centers treating Bietti crystalline dystrophy are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.