A case of PRES in an active lupus nephritis patient after treatment of corticosteroid and cyclophosphamide

Abstract

Posterior reversible encephalopathy syndrome (PRES) is primarily a radiological diagnosis. The syndrome is characterized by headache, altered mental status, seizures, and bilateral posterior white matter edema in a nonvascular distribution on neuroimaging with resolution of findings usually in 7-14 days (Casey et al. in AJNR Am J Neuroradiol 21:1199-1206, 2000). In most cases, computed tomography of the brain will show hypodense lesions in the parieto-occipital lobe. Although this syndrome is uncommon, prompt and accurate recognition allows early treatment, which has been shown to produce favorable outcomes. It is hypothesized that the dysfunction can be caused by a failure of autoregulation systemic hypertension or by the cytotoxic effects of vasculitides and immunosuppressive drugs. The present report is a possible second case of cyclophosphamide-induced PRES in a 16-year-old girl with systemic lupus erythematous and lupus nephritis. The initial suspected diagnosis was an ischemic stroke, but it was later changed, with resolution of symptoms after management of the underlying cause.

A Rare Case of Cyclophosphamide-Induced Posterior Reversible Encephalopathy Syndrome in a Patient with Anti-GBM Vasculitis, and Review of Current Literature

“…Chang-Hoon Lee reported a case of a 42-year-old woman with systemic lupus erythematosus (SLE) who presented with renal failure secondary to glomerulonephritis and developed PRES after two cycles of cyclophosphamide [30]. Jabrane et al reported a case of a 16-year-old girl with SLE and lupus nephritis who presented with renal failure and developed sudden onset of a headache, blurring of vision, followed by three episodes of generalized seizures after being treated with IV cyclophosphamide pulse therapy at 300 mg/m 2 of the body surface [28]. Zekić et al reported a case of an 18-year-old girl with of SLE who presented with renal failure due to lupus nephritis and acute arthritis and developed PRES after administration of the second dose of cyclophosphamide [25].…”

Fatal outcome of posterior reversible encephalopathy syndrome (PRES) in a lupus nephropathy patient: A case report

“…A lumbar puncture could be useful to eliminate meningitis, or a malignant cause. However, cerebrospinal fluid in PRES often only shows moderately elevated protein levels with less than 5 leucocytes/µl [12] . Differential diagnosis for PRES in a patient with a pre-established diagnosis of SLED, includes lupus cerebritis, neuropsychiatric-lupus, meningitis, ischemia, hemorrhage, a primary seizure disorder and many others, and PRES should be considered an elimination diagnosis with supportive findings.…”

“…The treatment of PRES remains symptomatic and etiological, and consists of rapid maintain of blood pressure and neuro-reanimation to avoid permanent damage. Patients presenting with PRES who are also on immunosuppressive therapy should have their medication temporarily discontinued or a decrease in dosage until the patient improves [12] . The prognosis in usually favorable in the classic form of PRES, as there is complete regression of the clinical and radiological signs once the underlying cause is managed promptly, nevertheless this reversible outcome is not always found.…”

Oral cyclophosphamide-induced posterior reversible encephalopathy syndrome in a patient with ANCA-associated vasculitis: A case report

“…It took longer to develop PRES with oral cyclophosphamide compared to IV drugs. Furthermore, two patients with ANCA-associated vasculitis and one with lupus nephritis who used IV injection developed PRES in 3 d[ 5 , 6 , 11 ]. Among two cases of oral cyclophosphamide-induced PRES, Cha et al [ 7 ] reported a 36-year-old woman with anti-GBM Ab glomerulonephritis treated with oral methylprednisolone, plasmapheresis and oral cyclophosphamide who suddenly developed PRES after 3 mo of treatment.…”