Abstract
Purpose: :
Adult Onset Stills Disease (AOSD) is a rare systemic inflammatory disorder which may present with a Purtschers-like Retinopathy. There were no long-term or large series identified in the literature which address visual outcomes or optimal treatment strategies.
Methods: :
Retrospective case series of all identified cases of Purtschers-like Retinopathy as a result of AOSD at Wills Eye Institute.
Results: :
2 cases were identified with an age range of 23-28 years old (average age 25.5 years). The visual acuity at evaluation ranged from 20/25 to Counting Fingers (CF). In one case, the patient required intensive care unit (ICU) admission, but his AOSD was successfully treated with high dose corticosteroids. By 8 months follow-up, the vision remained CF similar to his initial evaluation. Angiography revealed resolving microangiopathy and macular ischemia and macular atrophy. There were no signs of neovascularization. In the other case, the patient suffered acute renal failure and pulmonary edema requiring an extended (4 month) ICU admission. Her visual acuity rapidly deteriorated from 20/25 to hand motions (HM) concurrent with the worsening of her systemic disease within the first few days of evaluation. She was eventually diagnosed with AOSD with concurrent macrophage activation syndrome, which was resistant to high dose steroids. Plasmapheresis and intravenous Immunoglobulin failed to halt a progression of her systemic disease. Anakinra and Cyclosporine eventually controlled the systemic autoimmune disease. By 6 months follow-up, she remained HM vision in both eyes. Angiography demonstrated severe macular and retinal ischemia with neovascularization of the posterior and anterior segment in both eyes. She had early neovascular glaucoma in the left eye, and this required pan-retinal photocoagulation and intraocular bevacizumab therapy.
Conclusions: :
Purtscher-like Retinopathy secondary to AOSD has a poor visual prognosis related to irreversible macular ischemia. High dose steroids are a mainstay of initial therapy, but in the setting of concurrent macrophage activation syndrome, steroid sparing therapy may be necessary. Severe ischemia from retinal arteriole and choroidal occlusion may give rise neovascularization which requires treatment.
Keywords: retina • immunomodulation/immunoregulation • neovascularization