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Khalil El Karoui, Moglie Le Quintrec, Eric Dekeyser, Aude Servais, Aurélie Hummel, Fouad Fadel, Fadi Fakhouri, Posterior reversible encephalopathy syndrome in systemic lupus erythematosus, Nephrology Dialysis Transplantation, Volume 23, Issue 2, February 2008, Pages 757–763, https://doi.org/10.1093/ndt/gfm811
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Introduction
Neurologic or psychiatric abnormalities can occur frequently in patients with systemic lupus erythematosus (SLE). The American College of Reumatology have already described a wide variety of neuropsychiatric syndromes associated with SLE [1]. Posterior reversible encephalopathy syndrome (PRES), which can be associated with various underlying diseases including SLE, is probably an under-diagnosed syndrome in nephrology departments. We present three cases of PRES in SLE patients (table 1), then summarize and discuss all reported cases.
Cases
Case 1
A 47-year-old Asian woman, with a 13-year history of SLE that included stade III proliferative glomerulonephritis 5 years previously, presented with diffuse oedema, proteinuria and haematuria. She was treated with prednisone and furosemide. Laboratory investigation revealed haemoglobin 67 g/l, serum creatinine 77 μmol/l, haptoglobin 0.14 g/l, serum protein 58 g/l, serum albumin 19.4 g/l, C3 279 mg/l, C4 60 mg/l, CH50 40%, indirect immunofluorescence against anti-nuclear antibodies 1/800 and anti-DNA antibodies 92% (Farr test, N < 20%). Anticardiolipin antibodies and anti-beta2GP1 antibodies were negative. No schizocytes were present. Twenty-four hours after having received two packed red blood cells transfusion, she developed high blood pressure (BP: 199/90 mmHg), diffuse headache, photophobia and vomiting. Neurologic examination revealed no other abnormalities. Funduscopy was normal. Cranial T2 fluid attenuated inversion recovery (FLAIR)-weighted MRI revealed bilateral parietal and occipital white matter hyperintensities, which were more pronounced on the left side. Diffusion-weighted imaging (DWI) scans showed increased apparent diffusion coefficient (ADC), suggestive of vasogenic oedema. Cerebrospinal fluid examination was normal (protein concentration: 0.34 g/l, glucose concentration: 2.8 mmol/l, no elements, negative gram stain and bacteriological cultures). She was treated with nicardipine and urapidil with normalization of the BP and resolution of all symptoms within a few days. Renal biopsy disclosed a WHO Class IV lupus nephritis (LN), and she received intravenous pulses of methylprednisolone and cyclophosphamide. Repeat MRI 12 days later revealed complete resolution of the parietal and occipital oedema.
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