A 37-year-old female with no past medical or ocular history presented with 2 weeks of redness, pain, and photophobia of her right eye (OD). She was diagnosed with acute anterior uveitis by an outside provider and started on topical difluprednate four times daily (QID) and cyclopentolate once daily (QD) OD. Upon presentation to our facility, she reported slight improvement in her symptoms 2 weeks into treatment.
On initial examination, visual acuity was 20/20 in her right and 20/20 in her left eye (OS) with unremarkable pupillary exam and normal intraocular pressure. Anterior segment examination of the right eye (OD) revealed 2 areas of anterior corneal stromal scarring and 0.5+ anterior chamber cell without flare. One area of anterior stromal scarring and trace cell without flare was seen OS. Fundoscopy revealed 0.5+ vitreous cell without haze in both eyes (OU); healthy appearing optic nerves OU; and extensive, serpentine peripapillary chorioretinal scarring OU with extension into the macula (Figures 1(a) and 1(b)). There were no hemorrhages or vascular sheathing, and the peripheral retina was unremarkable. Fluorescein angiography (FA) was performed, which demonstrated hyperfluorescence at the lesion margins visible on gross funduscopy. Fundus autofluorescence (FAF) demonstrated hyperautofluoresence at lesion margins and hypoautofluorescence in areas of atrophic retina. Pertinent laboratory workup included negative QuantiFERON-TB Gold, fluorescent treponemal antibody (FTA), and Lyme IgM and IgG. A diagnosis of serpiginous choroidopathy was made, and the patient started on oral prednisone 60 mg daily, with a taper of the difluprednate OD by one drop per week.
The patient rapidly improved, and immunomodulatory therapy was initiated with a standard steroid taper. At one-month follow-up, en face OCT reflectance and OCTA imaging were obtained, which demonstrated severe atrophy with outer retinal tubules and patchy dropout of choriocapillaris in areas of otherwise normal-appearing retina, respectively (Figure 2). Over the next 2 months, the prednisone was slowly tapered and she continued mycophenolate mofetil 1 gram twice daily without recurrence of intraocular inflammation or activation of disease at the lesion margins. Repeat SD-OCT and OCTA imaging remained stable.