Reversible posterior leucoencephalopathy syndrome in systemic lupus and vasculitis

Abstract

Objectives-Reversible posterior leucoencephalopathy syndrome (RPLS) may develop in patients with renal insuYciency, hypertension, and immunosuppression, and is managed by prompt antihypertensive and anticonvulsant treatment. Four patients with renal insuYciency and fluid overload associated with Wegener's granulomatosis (one patient) and systemic lupus erythematosus (SLE) (three patients) are described, whose clinical picture and neuroimaging indicated RPLS. Case reports-All patients had headache, seizures, visual abnormalities, and transient motor deficit, and were hypertensive at the onset of the symptoms. Head computed tomography (CT) scan and magnetic resonance imaging showed predominantly posterior signal abnormalities, which were more conspicuous on T 2 weighted spin echo images than on CT scan. All patients had some form of cytotoxic treatment shortly before the syndrome developed, and dramatically responded to blood pressure control and anticonvulsant treatment. In two patients with SLE, dialysis was required for renal insuYciency. Discussion-Follow up neuroimaging studies showed almost complete resolution of signal abnormalities, and suggested that RPLS was associated with cerebral oedema without concomitant infarction. The treatment of hypertension and neurotoxic condition such as uraemia appears of primary importance, while immunosuppressive treatment may cause further damage of the blood-brain barrier.

Rituximab-related posterior reversible encephalopathy syndrome in HUS patient post kidney transplant

“…When the upper limit of cerebral autoregulation is exceeded, arterioles dilate and cerebral blood flow increases resulting brain hyperperfusion causes extravasation of fluid and blood products into the brain parenchyma [7,16,23]. An alternative theory proposes that endothelial dysfunction has also been implicated in the pathogenesis of PRES, especially in cases associated with preeclampsia or cytotoxic drugs [24,25].…”

“…The syndrome was described in association with acute hypertension, preeclampsia or eclampsia, renal disease, sepsis, and exposure to immunosuppressive agents [8][9][10][11][12][13][14]. It has been less commonly described in the setting of autoimmune disease [15][16][17][18][19].…”

Oligosymptomatic Reversible Posterior Leukoencephalopathy in a Patient With Lupus Nephritis

“…The variability of clinical and radiological findings leads into a wide differential diagnosis [4,5] including venous thrombosis, central nervous system vasculitis, stroke, central nervous system infections or even the onset of a demielinating disease.…”

“…There has been described many etiologies [3,4] for this neurological syndrome, such as severe hypertension, renal diseases, immunossupressive drugs, eclampsia or preeclampsia, vasculitis, use of contrasts or even blood transfusion.…”

Immunotherapies for Neurological Manifestations in the Context of Systemic Autoimmunity

“…As hypertensive and immunosuppression related PRES is more common, patients with SLE and vasculitis with relevant CNS symptoms should be carefully evaluated. Often, escalation of immunosuppression can aggravate symptoms instead of resolving them [171]. However, in patients with SLE with high disease activity, a reduction of immunosuppression is usually not advocated, as the participation of inflammatory mechanisms is highly likely [168,172].…”