PURTSCHER-LIKE RETINOPATHY ASSOCIATED WITH ATYPICAL HEMOLYTIC UREMIC SYNDROME: CASE REPORT AND REVIEW OF OUTCOMES. Issue Volume 17:Issues 2(2023) (March 2023)

• Record Type: Journal Article
• Authors: Gange, William S.; Haghighi, Arezu; Toy, Brian C.
• Is Part Of: Retinal cases & brief reports. Volume 17:Issues 2(2023)
• Page Start: 154
• Page End: 159
• Publication Date: 2023-03
• Subjects: atypical hemolytic uremic syndrome -- eculizumab -- Purtscher-like retinopathy; Retina -- Diseases -- Periodicals; Retina -- Periodicals; Retinal Diseases -- Periodicals; Retina -- Case Reports; Retinal Diseases -- Case Reports; 617.7
• DOI: 10.1097/ICB.0000000000001126
• Languages: English
• ISSNs: 1935-1089

Abstract

Abstract : Atypical hemolytic uremic syndrome is a rare, genetic, life-threatening thrombotic microangiopathy, characterized by uncontrolled activation of the complement system. The case of a patient with severe Purtscher-like retinopathy associated with atypical hemolytic uremic syndrome that was treated with eculizumab is presented. Ophthalmic outcomes of previous published cases are reviewed. Abstract : Purpose: The purpose of this study was to report the case of a patient presenting with newly diagnosed atypical hemolytic uremic syndrome (aHUS) and Purtscher-like retinopathy. Methods: This is an observational case report and review of literature. A 38-year-old woman presented with 3 months of rashes, fevers, arthralgias, and abdominal pain. Initial workup was suggestive of hypereosinophilic syndrome or adult-onset Still's disease. The patient developed acute renal failure and progressively blurry vision bilaterally over the course of 5 days. Funduscopic examination was notable for numerous Purtscher flecken and cotton–wool spots, with rare intraretinal hemorrhages at the posterior pole. The constellation of renal failure, hemolytic anemia, and thrombocytopenia prompted a workup for thrombotic microangiopathy that was remarkable for a mutation in the gene coding for complement protein C9. Results: The patient was diagnosed with aHUS and treated with intravenous pulse dose steroids for 3 days and an extended course of eculizumab. The patient's renal failure resolved, and her visual acuity improved, although she had residual visual field constriction and developed bilateral optic atrophy. Outcomes of other cases of Purtscher-like retinopathy related to aHUS are reviewed. Conclusion: Purtscher-like retinopathy is a rare but severe ophthalmic complication of aHUS. Eculizumab is an effective treatment for the systemic illness caused by aHUS, and anatomical resolution of Purtscher-like retinopathy may follow, although visual prognosis remains guarded. Recovery of visual acuity may lag behind resolution of macular edema in these patients.