title: Progressive outer retinal necrosis syndrome in the course of systemic lupus erythematosus.
abstract: Progressive outer retinal necrosis syndrome (PORN) is a severe clinical variant of necrotizing herpetic chorioretinitis, which occurs almost exclusively in patients with advanced acquired immunodeficiency syndrome (AIDS). To date, only a few cases of PORN have been reported in patients, mostly among those who were immunocompromised. To our knowledge, only one case of PORN in a patient with systemic lupus erythematosus (SLE) has been described. We report the case of a 44-year old HIV-negative patient with lupus nephritis, whom was being treated by mycophenolate mophetil (MMF), arechin and prednisone. After 14 months of MMF therapy, the patient revealed PORN symptoms; and several months later, the patient developed Type B primary central nervous system lymphoma (PCNSL). PORN is usually compared to acute retinal necrosis (ARN) syndrome, because of having the same causative agent: varicella zoster virus (VZV). There are also some similarities in clinical findings. Our observation supports the hypothesis that PORN symptoms in HIV-negative patients can be an intermediate form between ARN and PORN, and can vary according to the patient's immune status.
authors.affiliation: Department of Ophthalmology, Wrocław Medical University, Wroclaw, Poland.
authors.forename: A
authors.initials: A
authors.lastname: Turno-Kręcicka
authors.affiliation: Department of Ophthalmology, Wrocław Medical University, Wroclaw, Poland martyna_tomczyk@wp.pl.
authors.forename: M
authors.initials: M
authors.lastname: Tomczyk-Socha
authors.affiliation: Department of General and Interventional Radiology and Neuroradiology, Wrocław Medical University, Wroclaw, Poland.
authors.forename: A
authors.initials: A
authors.lastname: Zimny
mesh_terms: D000328:Adult; D000998:Antiviral Agents / Q000627:therapeutic use; D016543:Central Nervous System Neoplasms / Q000150:complications; D002738:Chloroquine / Q000031:analogs & derivatives / Q000627:therapeutic use; D002825:Chorioretinitis / Q000209:etiology / Q000821:virology*; D017809:Fatal Outcome; D005260:Female; D018023:HIV Seronegativity; D006562:Herpes Zoster / Q000150:complications* / Q000188:drug therapy; D014645:Herpesvirus 3, Human; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy; D008223:Lymphoma / Q000150:complications; D008279:Magnetic Resonance Imaging; D009173:Mycophenolic Acid / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D015882:Retinal Necrosis Syndrome, Acute; D014792:Visual Acuity
chemical_list: D000998:Antiviral Agents; C023676:chloroquine diphosphate; D002738:Chloroquine; D009173:Mycophenolic Acid; D011241:Prednisone
keywords: Case study; chorioretinitis; herpetic chorioretinitis; immune status; necrotizing chorioretinitis; primary central nervous system lymphoma; progressive outer retinal necrosis syndrome; retina; retinal necrosis; systemic lupus erythematosus; varicella zoster virus