Abstract: PUB225
Purtscher-Like Retinopathy: A Cue for Underlying Thrombotic Microangiopathy
Session Information
Category: Glomerular Diseases
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Allon, Michael, University of Alabama at Birmingham, Birmingham, Alabama, United States
- Rajasekaran, Arun, University of Alabama at Birmingham, Birmingham, Alabama, United States
Introduction
Purtscher-like retinopathy (PLR) is a retinal disorder characterized by acute visual loss and retinal findings including cotton-wool spots and intraretinal hemorrhages. PLR is a rare ophthalmological manifestation of systemic thrombotic microangiopathy (TMA). We describe a patient who presented to the hospital with PLR and was subsequently diagnosed as having atypical hemolytic uremic syndrome (aHUS) with multiple organ involvement.
Case Description
A 36-year-old woman presented with a sudden onset of blurred vision, marked dyspnea, and decreased urine output. Vital signs were stable on admission. Fundoscopy revealed multiple bilateral peripapillary yellow-white patches like cotton wool spots, intraretinal hemorrhages, and macular edema suggestive of PLR. She was found to have worsening anemia (Hb 6.8 gm/dl), thrombocytopenia (platelet count 40,000/mm3), and oliguric acute kidney injury (serum creatinine 8.3 mg/dl; unknown baseline renal function). Other pertinent labs included elevated LDH levels (1074 U/L), undetectable haptoglobin levels, normal coagulation profile, elevated D-Dimer (4303 ng/ml), and decreased fibrinogen levels (136 mg/dl). C3 levels were low (85 mg/dl), with normal C4 and CH50 levels. Autoimmune and infectious serologies were unremarkable. Serum vitamin B12 level was normal (628 pg/ml). A peripheral smear demonstrated numerous schistocytes. Stool shiga-toxin testing was negative. ADAMTS13 levels were normal (68%). Genetic testing panel for aHUS was unremarkable. A renal biopsy demonstrated TMA without segmental sclerosis, fibrinoid necrosis, karyorrhexis or crescents. No immune complex deposits were seen. She was treated with pulse dose steroids with slight improvement in her vision; and received 2 doses of Eculizumab. Renal function and hematological parameters failed to improve, and she was started on intermittent hemodialysis. Hospitalization was complicated by seizures secondary to posterior reversible encephalopathy syndrome because of systemic TMA. She later died owing to anoxic brain injury.
Discussion
aHUS is a subtype of TMA characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. It occurs owing to dysregulation of the alternative complement pathway leading to vascular endothelial damage. The presence of PLR should alert physicians to evaluate for an underlying TMA. Unfortunately, no effective treatment for PLR exists.