Reversible Posterior Leukoencephalopathy in Systemic Lupus Erythematosus With Thrombotic Thrombocytopenic Purpura

Cassano, Gustavo; Gongora, Vanina; Zunino, Alfredo; Roverano, Susana; Paira, Sergio

doi:10.1097/01.rhu.0000255805.15227.92

Cited by 9 publications

(5 citation statements)

References 14 publications

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“…Reviewing previous reports on RPLS in patients with SLE, there are two types of RPLS-SLE: ''hypertensive RPLS,'' being reversible with conventional antihypertensive and anticonvulsive treatments concomitant with inactive SLE, and ''immunological RPLS,'' which requires immunosuppressive therapy and is considered a neurological manifestation of active SLE. Table 1 summarizes RPLS-SLE cases from the literature review [4,5,[7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]. Among these reports, there were 28 patients with SLE for whom adequate clinical information was provided for meta-analysis.…”

Section: Discussionmentioning

confidence: 99%

Clinical features of reversible posterior leukoencephalopathy syndrome in patients with systemic lupus erythematosus

et al. 2011

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To characterize reversible posterior leukoencephalopathy syndrome (RPLS) in systemic lupus erythematosus (SLE) in terms of treatments for resolution and its clinical course, we reviewed 28 cases of RPLS in SLE including our cases in view of the treatment. Of these, 15 cases improved with blood pressure control and 13 required immunosuppressive therapy for activity of SLE presenting neurological manifestations. Patients without immunosuppressants at onset of RPLS more frequently required immunosuppressive therapy to recover it than those precedingly using these agents [31% (4/13) versus 87% (13/15), p = 0.008, chi-square test]. Brain magnetic resonance imaging (MRI) is important for diagnosis of RPLS-SLE in the patient with SLE who develops neurological disturbance and rapidly increasing blood pressure. When 7-day therapy for hypertension and convulsion does not reverse the manifestations, immunosuppressive treatments would be recommended to reverse RPLS.

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Section: Discussionmentioning

confidence: 99%

Clinical features of reversible posterior leukoencephalopathy syndrome in patients with systemic lupus erythematosus

et al. 2011

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“…112,113 In recent years, reports of PRES in patients with connective tissue diseases, the majority of them with systemic lupus erythematosus (SLE) and also Wegener granulomatosis, polyarteriitis nodosa, scleroderma, psoriasis, Graves disease, and rheumatoid factor positive arthropathy have accumulated. 1,3,6,15,53,[114][115][116][117][118][119][120][121][122][123][124][125][126][127][128][129][130][131][132][133] The particular role of the underlying connective tissue disease in the pathogenesis of PRES is not clear in those cases, because comorbidity like hypertension, renal involvement with renal insufficiency, infection, or recent immunosuppressive treatment with corticosteroids, cyclophosphamide, cyclosporine, mycophenolate mofetil, rituximab, and azathioprine, all factors separately associated with PRES, were present in most patients. 133 Interestingly, withdrawal of putatively causative immunosuppressive drugs on one hand, and administration of high-dose corticosteroids or adjunctive immunosuppressive drugs, including cyclophosphamide or mycophenolate mofetil on the other hand have been reported to lead to successful treatment of PRES.…”

Section: Vascular and Cerebral Perfusion Imagingmentioning

confidence: 99%

Posterior Reversible Encephalopathy Syndrome

Staykov

Schwab

2011

J Intensive Care Med

134

144

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Posterior reversible encephalopathy syndrome (PRES) is characterized by headache, altered mental status, visual disturbances, and seizures. Radiological features typically include edema of the posterior cerebral regions, especially of the parietooccipital lobes. Atypical imaging features, such as involvement of anterior cerebral regions, deep white matter, and the brain stem are also frequently seen. Vasoconstriction is common in vascular imaging. Different conditions have been associated with PRES, but toxemia of pregnancy, solid organ or bone marrow transplantation, immunosuppressive treatment, cancer chemotherapy, autoimmune diseases, and hypertension are most commonly described. The pathophysiology of PRES is unclear and different hypotheses are being discussed. Posterior reversible encephalopathy syndrome is best managed by monitoring and treatment in the setting of a neurointensive care unit. The prognosis is usually benign with complete reversal of clinical symptoms within several days, when adequate treatment is immediately initiated. Treatment of severe hypertension, seizures, and withdrawal of causative agents represent the hallmarks of specific therapy in PRES. Delay in diagnosis and treatment may lead to permanent neurological sequelae. Therefore, awareness of PRES is of crucial importance for the intensivist.Keywords posterior reversible encephalopathy syndrome, reversible posterior leukoencephalopathy syndrome, hypertensive encephalopathy, eclampsia

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“…In 2007, we described the first case of PRES associated with SLE and thrombotic thrombocytopenic purpura 2 . In that case, the neurologic symptoms, the characteristic MR images, and the prompt full response to treatment enabled the diagnosis (Figure 1).…”

Section: To the Editormentioning

confidence: 99%