Posterior Reversible Encephalopathy Syndrome: An Emerging Disease Manifestation in Systemic Lupus Erythematosus

Barber, Claire; Leclerc, Rachel; Gladman, Dafna D.; Urowitz, Murray B.; Fortin, Paul R.

doi:10.1016/j.semarthrit.2011.07.001

Cited by 44 publications

(34 citation statements)

References 29 publications

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“…[1][2][3][4] Systemic lupus erythematosus (SLE) is one of the predisposing conditions related to PRES, particularly when accompanied by lupus nephritis. [4][5][6][7][8][9] However, SLE is an uncommon autoimmune disease in which the occurrence of PRES is even rarer. This rarity makes it difficult to clarify the clinical course of PRES in patients with SLE, and there are some inconsistencies between the studies performed with case reports.…”

Section: Introductionmentioning

confidence: 99%

Posterior reversible encephalopathy syndrome in Korean patients with systemic lupus erythematosus: risk factors and clinical outcome

et al. 2013

Lupus

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Posterior reversible encephalopathy syndrome (PRES) is an uncommon neurologic condition associated with systemic lupus erythematosus (SLE). This study aimed to demonstrate the risk factors and clinical outcome of PRES in patients with SLE. Fifteen patients with SLE were diagnosed with PRES by characteristic clinical manifestations and magnetic resonance imaging (MRI) features from 2000 to 2012. Clinical profiles and outcomes were assessed for this study population. Additionally, 48 SLE patients with neurologic symptoms who underwent brain MRI were included for comparative analyses. The median age and duration of SLE in patients with PRES was 27 and 6.1 years, respectively. Comparison between patients with and without PRES revealed significant differences in the presentation of hypertension and seizure, lupus nephritis with renal insufficiency, treatment with high-dose steroid and cyclophosphamide, recent transfusion, and lupus activity measured by SLE disease activity index. Renal failure was the single independent factor with a high odds ratio of 129.250 by multivariate analysis. Of 15 patients, four experienced relapse and two died of sepsis during hospitalization. Our results suggest that lupus nephritis with renal dysfunction and other related clinical conditions can precede the occurrence of PRES in patients with SLE. It is important to perform early brain imaging for a timely diagnosis of PRES when clinically suspected.

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Section: Introductionmentioning

confidence: 99%

Posterior reversible encephalopathy syndrome in Korean patients with systemic lupus erythematosus: risk factors and clinical outcome

et al. 2013

Lupus

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“…However, RPLS can also occur as a neurological manifestation of active lupus and sometimes requires intensive immunosuppressive therapy (13). Recently, RPLS has been increasingly considered to be one of the neuropsychiatric syndromes of active lupus (14). In this patient, an anti-hypertensive agent was not administered immediately following her admission because of the significantly decreased cerebral blood flow that was observed in the bilateral cerebellum and the right frontal and temporal lobes.…”

Section: Discussionmentioning

confidence: 99%

Successful Use of Intensive Immunosuppressive Therapy for Treating Simultaneously Occurring Cerebral Lesions and Pulmonary Arterial Hypertension in a Patient with Systemic Lupus Erythematosus

et al. 2014

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A 59-year-old woman who had been diagnosed with systemic lupus erythematosus (SLE) was admitted to our hospital due to paralysis in all of her limbs. The patient presented with dysarthria, cerebellar ataxia and hypoxia. Magnetic resonance imaging (MRI) revealed vasogenic edema in the brain stem and the cerebellum. She was diagnosed with neuropsychiatric lupus syndrome (NPSLE) and pulmonary arterial hypertension (PAH), and was successfully treated using immunosuppressive therapy. To our knowledge, this is the first reported case of simultaneously developing NPSLE and PAH.

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“…5 Hay que destacar que la encefalopatía puede presentarse en pacientes normotensos y sin inmunosupresores previos. 8,14 Un 20%-95% de pacientes con LES tienen manifestaciones neurológicas agrupadas bajo la denominación de lupus neuropsiquiátrico (LNPS) y pueden ser la forma de presentación del LES con escasos síntomas sistémicos. 6,15 Las manifestaciones clínicas son similares a la EPR y, por lo tanto, difíciles de diferenciar.…”

Section: Discussionunclassified

“…8,14,15 Algunos autores recomiendan tratar un LES con alta actividad y EPR con drogas inmunosupresoras para evitar daños mayores a otros órganos. Otros, en un LES con baja actividad que recibe inmunosupresores y desarrolla la EPR, recomiendan reducir la dosis o retirarlos.…”

Section: Discussionunclassified

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Encefalopatía reversible posterior en una niña con lupus eritematoso sistémico. Presentación de un caso

Marín¹

2015

Arch Argent Pediat

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Arch Argent Pediatr 2015;113(5):e271-e274 / e271Presentación de casos clínicos RESUMEN La encefalopatía posterior reversible es una patología de baja frecuencia en pediatría. Las manifestaciones clínicas características son cefaleas, convulsiones, trastornos visuales y de conciencia asociadas a imágenes típicas en la resonancia magnética del sistema nervioso. Por lo general, se manifiesta en pacientes con eclampsia, trasplante de órganos sólidos, enfermedades hematológicas, renales y autoinmunes, entre otras causas menos frecuentes, y suele desencadenarse luego de un cuadro de hipertensión arterial o el uso de drogas inmunosupresoras. Factores patogénicos menos habituales, como transfusión sanguínea, uso de inmunoglobulinas o una infección subyacente, pueden estar asociados. Se describe una paciente con lupus eritematoso sistémico, que desarrolló la encefalopatía al estar expuesta a múltiples factores etiopatogénicos. Palabras clave: síndrome de encefalopatía posterior reversible, lupus eritematoso sistémico, pediatría. ABSTRACTPosterior reversible encephalopathy is a rare disease in children. Clinical manifestations include headache, seizures, visual disturbances and altered consciousness associated with typical magnetic resonance images of the nervous system. The syndrome usually manifests in patients with eclampsia, solid organ transplantation, haematologic, renal and autoimmune diseases among other less common causes and it is often triggered after a hypertensive crisis or use of immunosuppressive drugs. Less common pathogenic factors as blood transfusion, use of immunoglobulins or an underlying infection can be associated. In this case a girl with systemic lupus erythematosus and exposed to multiple etiopathogenic factors developed posterior reversible encephalopathy.

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Posterior Reversible Encephalopathy Syndrome: An Emerging Disease Manifestation in Systemic Lupus Erythematosus

Cited by 44 publications

References 29 publications

Posterior reversible encephalopathy syndrome in Korean patients with systemic lupus erythematosus: risk factors and clinical outcome

Posterior reversible encephalopathy syndrome in Korean patients with systemic lupus erythematosus: risk factors and clinical outcome

Successful Use of Intensive Immunosuppressive Therapy for Treating Simultaneously Occurring Cerebral Lesions and Pulmonary Arterial Hypertension in a Patient with Systemic Lupus Erythematosus

Encefalopatía reversible posterior en una niña con lupus eritematoso sistémico. Presentación de un caso

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