Reversible posterior leukoencephalopathy syndrome (RPLS) in a patient with systemic lupus erythematosus (SLE) and lupus nephritis

Abstract

A 20-year-old Asian woman with a well-established history of systematic lupus erythematosus (SLE) and lupus nephritis (WHO stage IVB) presented to the emergency department with generalized arthralgia and headaches. Her current medications included cellcept 1000mg daily and prednisone 30mg daily. The patient had had low normal blood pressure in the past, and experienced episodes of hypotension with lisinopril. Initial laboratory workup revealed serum creatinine of 4.5mg/dl (baseline 0.5mg/dl), C3 57 g/l, C4 200 IU/ml, CRP 356mg/dl, ESR 122mm/h and a spot urine protein to creatinine ratio of 3309mg/g. Urine microscopy showed persistent nephritic sediments. Physical examination was significant for a malar rash. Initial blood pressure (BP) was 96/66mmHg. The patient was diagnosed with lupus flair-up and received methylprednisone 125mg IV, in the emergency room. Within the next hour, her BP increased to 140/110mmHg. After admission, the patient was continued on IV hydrocortisone, 100mg every 6 h. Renal function improved and returned to baseline over the next 3 days. Simultaneously, the patient gained 10 lbs weight and developed significant dependent oedema. Her BP progressively increased, despite multiple antihypertensive medications. On day 3, the cellcept was discontinued due to gram negative bacteremia, and IV antibiotics were started. On day 4, BP rose to 190/110mmHg. The patient experienced a transient seizure and cortical blindness. She was transferred to ICU, and started on aggressive BP management with IV antihypertensives. MRI of the brain Fig. 1. Fluid-attenuated inversion recovery (FLAIR) MRI image of the patient on 5 October 2004, showing no abnormality.