title: Reversal of threatening blindness after initiation of eculizumab in Purtscher-like retinopathy secondary to atypical hemolytic uremic syndrome.
abstract: Purtscher-like retinopathy, a rare manifestation of systemic thrombotic microangiopathy, is a potentially visually debilitating condition with no effective proven treatment. Distinct pathogenic pathways have been proposed as etiological factors. We revisit the etiology of Purtscher-like retinopathy based on the rapid response and profound visual improvement after initiation of systemic intravenous eculizumab, an inhibitor of the complement cascade, in a patient with Purtscher-like retinopathy secondary to familial atypical hemolytic uremic syndrome (aHUS) due to a mutation in complement factor H. We hypothesize that the efficacy of eculizumab in this patient provides evidence for pathogenic events in the retina similar to those encountered in the renal microvasculature of aHUS patients, namely complement-mediated thromboembolization as a result of activation of the complement cascade in endothelial cells with release of tissue factor and development and amplification of a procoagulant state. To the best of our knowledge, this is the first report in the literature of eculizumab as an effective therapeutic strategy in Purtscher-like retinopathy.
authors.affiliation: Department of Ophthalmology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands. j.e.decarvalho@amc.uva.nl.
authors.forename: J E
authors.identifier: http://orcid.org/0000-0001-6748-620X
authors.initials: JE
authors.lastname: Ramos de Carvalho
authors.affiliation: Department of Ophthalmology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: R O
authors.initials: RO
authors.lastname: Schlingemann
authors.affiliation: Division of Internal and External Medicine, Department of Nephrology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: M
authors.initials: M
authors.lastname: Oranje
authors.affiliation: Division of Internal and External Medicine, Department of Nephrology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: F J
authors.initials: FJ
authors.lastname: Bemelman
authors.affiliation: Department of Ophthalmology, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: M J
authors.initials: MJ
authors.lastname: van Schooneveld
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage*; D065766:Atypical Hemolytic Uremic Syndrome / Q000150:complications*; D001766:Blindness / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D004305:Dose-Response Relationship, Drug; D005260:Female; D005451:Fluorescein Angiography; D005500:Follow-Up Studies; D005654:Fundus Oculi; D006801:Humans; D020127:Recovery of Function; D012164:Retinal Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D041623:Tomography, Optical Coherence / Q000379:methods; D014792:Visual Acuity; D055815:Young Adult
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab
keywords: Atypical hemolytic uremic syndrome; Complement; Eculizumab; Purtscher-like retinopathy