Serpiginous choroidopathy

Serpiginous Choroidopathy Serpiginous choroidopathy, also known as geographic choroidopathy, is a rare, chronic, progressive inflammatory disease affecting the choroid, a layer of blood vessels and connective tissue between the sclera and the retina in the eye. This condition is characterized by serpentine or geographic lesions that can lead to significant visual impairment.

Clinical Presentation

Patients with serpiginous choroidopathy typically present with symptoms such as blurred vision, scotomas (blind spots), and metamorphopsia (distorted vision). The disease often affects both eyes, although it may initially present unilaterally. The lesions usually start at the optic disc and spread in a serpentine pattern.

Pathophysiology

The exact cause of serpiginous choroidopathy is unknown, but it is believed to be an autoimmune process. The inflammation primarily affects the choroid and the retinal pigment epithelium (RPE), leading to atrophy and scarring. The disease is characterized by periods of activity and remission.

Diagnosis

Diagnosis of serpiginous choroidopathy is primarily clinical, supported by imaging studies. Fluorescein angiography and indocyanine green angiography are used to visualize the characteristic lesions. Optical coherence tomography (OCT) can also be helpful in assessing the extent of retinal involvement.

Differential Diagnosis

The differential diagnosis for serpiginous choroidopathy includes:

• Multifocal choroiditis
• Birdshot chorioretinopathy
• Acute posterior multifocal placoid pigment epitheliopathy (APMPPE)
• Tuberculous choroiditis

Treatment

There is no cure for serpiginous choroidopathy, but treatment aims to control inflammation and preserve vision. Corticosteroids are commonly used to reduce inflammation. Immunosuppressive agents such as cyclosporine, azathioprine, or mycophenolate mofetil may be used in more severe cases. Anti-VEGF therapy has also been explored in some cases.

Prognosis

The prognosis for serpiginous choroidopathy varies. Some patients experience a relatively benign course with minimal visual loss, while others may suffer from significant visual impairment due to the progressive nature of the disease and the potential for choroidal neovascularization.

Research and Future Directions

Research into the pathogenesis and treatment of serpiginous choroidopathy is ongoing. Advances in imaging techniques and a better understanding of the immune mechanisms involved may lead to improved management strategies in the future.

See Also

• Choroiditis
• Retinal diseases
• Autoimmune diseases

NIH genetic and rare disease info

• NIH info on Serpiginous choroidopathy

Serpiginous choroidopathy is a rare disease.