Frosted Branch Angiitis associated with Mixed Connective Tissue Disease

Abstract

Purpose: 

To present a case of frosted branch angiitis associated with an exacerbation of mixed connective tissue disease.

Methods: 

Single case report

Results: 

A 31-year-old woman presented with a flare of her long-standing mixed connective tissue disease (MCTD) after a change in her immunosuppressive medications. She developed blurred vision and floaters first in the left eye but eventually in both eyes. Fundoscopy showed patchy perivascular sheathing of tertiary branch venules surrounded by retinal hemorrhages characterized as frosted branch angiitis (FBA). The patient’s MCTD symptoms and retinal vasculitis improved with continued immunosuppressive therapy. At 1 month follow-up, her visual acuity had improved to 20/20 bilaterally with complete resolution on fundoscopy.

Conclusion: 

To the authors’ knowledge, this is the first report of FBA seen in association with MCTD. The improvement in the patient’s visual acuity and fundoscopic findings in this case supports the role of immunosuppressive therapy to treat secondary FBA associated with an autoimmune condition such as MCTD. However, it is recommended that a comprehensive medical work-up is performed to exclude an infective cause, particularly in immunocompromised patients.