Posterior reversible encephalopathy syndrome--an underrecognized manifestation of systemic lupus erythematosus

Abstract

Objective: Posterior reversible encephalopathy syndrome (PRES) is a rare, recently described neurologic condition identifiable by clinical presentation and magnetic resonance image (MRI) appearance. It is associated with renal insufficiency, hypertension, and rheumatologic diseases. Patients present with headache, seizures, loss of vision and altered mental function, and a pattern on imaging studies of predominantly transient, posterior cerebral hyperintensities on T2-weighted MRI. There is a high likelihood of presentation of this syndrome to a rheumatologist.

Methods: Three recent cases of systemic lupus erythematosus (SLE) with PRES, along with 9 previously reported cases, are reviewed.

Results: All 3 patients presented with seizures and subacute visual changes in association with lupus nephritis. The first presented with hypertension, complete visual field loss, and status epilepticus 2 weeks after starting oral cyclosporine therapy for refractory lupus nephritis. The second patient was normotensive and presented with seizures and visual symptoms while in hospital with SLE-related pancreatitis and nephritis. The third patient had headache and seizures with severe lupus disease activity including nephritis, pancytopenia, and pulmonary hemorrhage. Cranial MRI showed predominantly posterior signal abnormalities on T2-weighted images, which resolved after cessation of cyclosporine in the first case, treatment with IV cyclophosphamide in the second case, and treatment with cyclophosphamide and plasmapheresis in the final case. Literature review showed that PRES is a manifestation of SLE or a consequence of therapy with calcineurin inhibitors or rituximab. The hallmark features are visual loss and seizures. Severe hypertension (> 170/110 mm Hg) and renal failure were present in the majority of previously identified cases of SLE and PRES. Our second case was normotensive but had marked lupus disease activity. PRES can lead to cerebral infarction.

Conclusion: With increasing availability of MRI, PRES will be identified more frequently. Swift action to identify potential offending agents, controlling hypertension, and treating active disease can lead to reversal of radiologic and neurologic findings.