Posterior Reversible Encephalopathy Syndrome Secondary to Cyclophosphamide in the Treatment of Pulmonary Renal Syndrome

Abstract

SESSION TITLE: Nervous System Disorders in the ICU 2 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a clinical radiological syndrome, carrying a 15% mortality risk. It can lead to permanent neurological deficits and death if not identified and treated early. CASE PRESENTATION: A 22-year-old female with a history of Sjogren’s syndrome was admitted to the intensive care unit for acute respiratory failure. She was diagnosed with diffuse alveolar hemorrhage after bronchoalveolar lavage, along with concurrent acute glomerulonephritis. Labs were significant for creatinine of 1.47 mg/dL, as well as protein >500, large blood, and 54 RBCs/hpf on urinalysis. Urine protein to creatinine ratio was 8.3. Glomerulonephritis workup was significant for ANA 1:5120, positive anti-MPO IgG, but negative anti-dsDNA, negative anti-GBM, negative HIV and hepatitis serologies, as well as a negative syphilis screen. The patient was initially diagnosed with pulmonary renal syndrome due to MPO-associated vasculitis. She was treated with high dose pulse steroids, cyclophosphamide induction on day 2 of ICU, and 5 days of plasmapheresis with improvements in respiratory status, but not in renal function. On day 5 the patient developed acute nausea, vomiting, and tonic-clonic seizures. Her vital signs were significant for a blood pressure of 170/110 mmHg. Neurological exam was evident for lethargic appearance, and a right pronator drift. Based on documentation, a progressive increase in her blood pressure was observed since the start of cyclophosphamide. Non-contrast MRI of the brain confirmed posterior reversible encephalopathy syndrome (PRES), with evidence of vasogenic edema in the bilateral parietal, occipital lobes, and cerebellum. DISCUSSION: The most common signs and symptoms of PRES are acute hypertension, seizure, nausea and vomiting, and altered consciousness. The incidence of PRES is unknown but is reported in ages 2-90, and occurs more frequently in women. Reported risk factors associated with PRES are hypertension, renal disease, immunosuppressive therapy, eclampsia/preeclampsia, and autoimmune diseases. The pathogenesis is not fully understood, however current hypotheses suggest disruption of vascular autoregulation in the brain, leading to vascular leakage, vasogenic edema, and hemorrhage. Diagnosis involves a high degree of clinical suspicion, as there are no current guidelines available. Aggressive blood pressure control with rapid lowering of the diastolic pressure below 100 mmHg within 2-6 hours is the mainstay of therapy, along with removal of the inciting agent. CONCLUSIONS: It is important to recognize PRES early and initiate the appropriate management. Unlike its name, PRES is not always posterior, and may not always be reversible. If not treated, it can lead to permanant neurological disability and death. Reference #1: Pedraza, R. Posterior Reversible Encephalopathy Syndrome: A Review, Crit Care & Shock (2009) 12:135-143 DISCLOSURE: The following authors have nothing to disclose: Di Pan, Basera Sabharwal, Franco Vallejo No Product/Research Disclosure Information