Bilateral Occlusive Retinal Vasculitis in a Patient with Dermatomyositis

Area of Science:

• Ophthalmology
• Rheumatology
• Immunology

Background:

• Dermatomyositis (DMS) is an autoimmune disease primarily affecting muscles and skin.
• Ophthalmic manifestations of DMS commonly include episcleritis, conjunctivitis, and uveitis.
• Severe visual impairment from DMS is uncommon.

Observation:

• A 48-year-old woman with a history of DMS experienced vision loss after stopping immunosuppression.
• Clinical presentation and multimodal imaging revealed bilateral frosted branch angiitis, a severe form of retinal vasculitis.
• The patient presented with myalgias, weakness, and edema preceding vision loss.

Findings:

• The patient was treated with high-dose steroids, intravenous immunoglobulin, and intravitreal aflibercept.
• Successful anatomical and visual acuity improvement was observed post-treatment.
• This case highlights an unusual ophthalmic complication of DMS.

Implications:

• Combined anti-vascular endothelial growth factor (anti-VEGF) therapy and systemic immunosuppression may effectively manage DMS-related frosted branch angiitis.
• Retinal vasculitis should be considered in DMS patients experiencing acute vision loss.
• Prompt ophthalmologic evaluation is crucial for early diagnosis and management of DMS-related ocular complications.