Overview
Serpiginous choroiditis, also known as geographic helicoid peripapillary choroidopathy or serpiginous choroidopathy, is a rare, chronic, progressive inflammatory eye disease that primarily affects the choroid and retinal pigment epithelium (RPE). The condition is characterized by recurrent episodes of inflammation that typically begin around the optic disc (peripapillary region) and extend outward in a serpentine or snake-like pattern, leading to progressive destruction of the choroid and overlying RPE. It predominantly affects the posterior segment of the eye and can be unilateral or bilateral, though bilateral involvement is more common. Patients typically present with blurred vision, central or paracentral scotomas (blind spots), and metamorphopsia (visual distortion). The disease follows a relapsing and remitting course, with each recurrence causing further atrophy of the choroid and RPE. When the macula becomes involved, significant and often permanent visual loss can occur. The condition most commonly affects otherwise healthy adults, typically between the ages of 30 and 70, with a slight male predominance reported in some series. The exact cause of serpiginous choroiditis remains unknown, though an autoimmune mechanism targeting choroidal vasculature is suspected. An association with tuberculosis has been described in endemic regions, leading to the concept of tubercular serpiginous-like choroiditis, which must be distinguished from the idiopathic form. Treatment typically involves systemic immunosuppressive therapy, including corticosteroids, antimetabolites (such as azathioprine or mycophenolate mofetil), and alkylating agents (such as cyclophosphamide) for refractory cases. Anti-VEGF agents and biologic therapies have also been used in select cases. In tubercular serpiginous-like choroiditis, antitubercular therapy is a critical component of management. Early and aggressive treatment is important to limit disease progression and preserve vision.
Also known as:
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 Serpiginous choroiditis.
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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 Serpiginous choroiditis.
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Common questions about Serpiginous choroiditis
What is Serpiginous choroiditis?
Serpiginous choroiditis, also known as geographic helicoid peripapillary choroidopathy or serpiginous choroidopathy, is a rare, chronic, progressive inflammatory eye disease that primarily affects the choroid and retinal pigment epithelium (RPE). The condition is characterized by recurrent episodes of inflammation that typically begin around the optic disc (peripapillary region) and extend outward in a serpentine or snake-like pattern, leading to progressive destruction of the choroid and overlying RPE. It predominantly affects the posterior segment of the eye and can be unilateral or bilatera
How is Serpiginous choroiditis inherited?
Serpiginous choroiditis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Serpiginous choroiditis typically begin?
Typical onset of Serpiginous choroiditis is adult. Age of onset can vary across affected individuals.
Which specialists treat Serpiginous choroiditis?
1 specialists and care centers treating Serpiginous choroiditis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.