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Retinal vasculopathy associated with mixed connective tissue disease
Yong-Kyu Kim1, Se Joon Woo, Yun Jong Lee
1Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea.
Ocular Immunology and Inflammation
|February 5, 2010
Summary
Mixed connective tissue disease (MCTD) can cause severe, intractable retinal vasculopathy affecting both eyes. This case highlights the limited efficacy of standard treatments for this rare complication.
Area of Science:
- Ophthalmology
- Rheumatology
- Medical Case Reports
Background:
- Mixed connective tissue disease (MCTD) is a systemic autoimmune disorder with overlapping features of lupus, scleroderma, and polymyositis.
- Ocular manifestations of MCTD are diverse, but retinal vasculopathy is uncommon and can lead to significant vision loss.
Observation:
- A 35-year-old woman with a history of MCTD and central retinal vein occlusion presented with acute vision decrease, retinal hemorrhages, and macular edema.
- Despite aggressive treatment with systemic and intravitreal steroids and immunosuppressants, her condition progressed.
Findings:
- The patient developed severe, bilateral retinal vasculopathy involving both arteries and veins, leading to extensive retinal nonperfusion.
- Visual acuity ultimately deteriorated to hand motion, indicating a poor prognosis.
Implications:
- This case underscores that MCTD can be associated with severe, treatment-refractory retinal vasculopathy.
- Ophthalmologists and rheumatologists should consider MCTD in the differential diagnosis of unexplained, severe retinal vascular disease.
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