Henoch–Schönlein Purpura with Posterior Reversible Encephalopathy Syndrome

Stefek, Bryan; Beck, Michael; Ioffreda, Michael D.; Gardner, Lyndsay; Stefanski, Michael

doi:10.1016/j.jpeds.2015.07.066

Cited by 11 publications

(11 citation statements)

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“…In total, we identified 28 cases with IgAV and reversible encephalopathy ( 6 – 10 , 17 – 36 ). Twenty six cases were associated with seizures ( 6 – 10 , 17 – 19 , 21 – 31 , 33 , 34 , 36 ), and thereof 14 cases fulfilled all clinico-radiological criteria of PRES ( 6 , 7 , 9 , 10 , 17 – 25 ).…”

Section: Resultsmentioning

confidence: 99%

“…In total, we identified 28 cases with IgAV and reversible encephalopathy (6)(7)(8)(9)(10)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36). Twenty six cases were associated with seizures (6-10, 17-19, 21-31, 33, 34, 36), and thereof 14 cases fulfilled all clinico-radiological criteria of PRES (6, 7, 9, 10, 17-25).…”

Section: Seizures and Pres In Igavmentioning

confidence: 99%

“…Additionally, multiple manifestations in other organs, such as the kidney (glomerulonephritis), intestine (intussusception), lungs (alveolar hemorrhage), and central nervous system (seizures, cerebrovascular thrombosis) have been reported (2,4,5). Posterior reversible encephalopathy syndrome (PRES) has been anecdotally reported in patients with IgAV (6)(7)(8)(9)(10).…”

Section: Introductionmentioning

confidence: 99%

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Focal Seizures and Posterior Reversible Encephalopathy Syndrome as Presenting Signs of IgA Vasculitis/Henoch-Schoenlein Purpura—An Educative Case and Systematic Review of the Literature

et al. 2021

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Background: IgA vasculitis/Henoch-Schoenlein purpura (IgAV/HSP) is a systemic small vessel vasculitis of unknown pathogenesis predominantly affecting children. While skin, GI tract, joints, and kidneys are frequently affected and considered, central nervous system (CNS) involvement of this disease is underestimated.Methods: We provide a case report and systematically review the literature on IgAV, collecting data on the spectrum of neurological manifestations.Results: We report on a 7-year-old girl with IgAV who presented with diplopia and afebrile focal seizures, which preceded the onset of purpura. Cranial magnetic resonance imaging was consistent with posterior reversible encephalopathy syndrome (PRES), showing typical focal bilateral parietal swelling and cortical and subcortical high signal intensities on T2-fluid attenuated inversion recovery (FLAIR) images predominantly without diffusion restriction. Cerebrospinal fluid analysis and blood tests excluded systemic inflammation or vasculitis. Interestingly, hypertension was not a hallmark of the developing disease in the initial phase of PRES manifestation. Renal disease and other secondary causes for PRES were also excluded. Supportive- and steroid treatment resulted in restitution ad integrum. Reviewing the literature, we identified 28 other cases of IgAV with CNS involvement. Severe CNS involvement includes seizures, cerebral edema, or hemorrhage, as well as PRES. Thirteen patients fulfilled all diagnostic criteria of PRES. The mean age was 11.2 years (median 8.0, range 5-42 years), with no reported bias toward gender or ethnic background. Treatment regimens varied from watchful waiting to oral and intravenously steroids up to plasmapheresis. Three cases showed permanent CNS impairment.Conclusion: Collectively, our data demonstrate that (I) severe CNS involvement such as PRES is an underappreciated feature of IgAV, (II) CNS symptoms may precede other features of IgAV, (III) PRES can occur in IgAV, and differentiation from CNS vasculitis is challenging, (IV) pathogenesis of PRES in the context of IgAV remains elusive, which hampers treatment decisions. We, therefore, conclude that clinical awareness and the collection of structured data are necessary to elucidate the pathophysiological connection of IgAV and PRES.

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Section: Resultsmentioning

confidence: 99%

Section: Seizures and Pres In Igavmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Focal Seizures and Posterior Reversible Encephalopathy Syndrome as Presenting Signs of IgA Vasculitis/Henoch-Schoenlein Purpura—An Educative Case and Systematic Review of the Literature

et al. 2021

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“…Subjects presenting with neurologic symptoms often exhibit multiorgan involvement, renal impairment, and arterial hypertension. 11,12 PRES has become increasingly recognised in childhood, especially in the setting of autoimmune diseases, cancer, and renal diseases. 13 However, the precise mechanism of PRES remains to be elucidated and appears to be multifactorial.…”

Section: Discussionmentioning

confidence: 99%

“…Acute elevation in blood pressure has long been considered the most frequent precipitating factor of PRES both in patients with and without HSP, but it may be absent in up to 20%–40% of cases. 12,13 Additionally, systemic inflammatory states and impaired renal function have been shown to predispose to PRES, and therefore HSP patients presenting with severe nephritis are especially prone to developing PRES owing to multiple concomitant risk factors – mainly hypoalbuminaemia, proteinuria, and nephrotic syndrome. 14,15 We also cannot rule out a possible contributing role of corticosteroid therapy in exacerbating arterial hypertension and thereby potentially triggering PRES due to their effect on mineralocorticoid receptors.…”

Section: Discussionmentioning

confidence: 99%

Posterior reversible encephalopathy syndrome as a complication of Henoch–Schönlein purpura in a seven-year-old girl

et al. 2017

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Introduction Henoch-Schönlein purpura is a multisystem small vessel vasculitis. Neurologic manifestations are uncommon. Posterior reversible encephalopathy syndrome is a rare complication of Henoch-Schönlein purpura with typical clinical and neuroimaging findings that occurs most commonly in the setting of severe hypertension and renal injury. Case presentation A seven-year-old girl was admitted to our institution presenting with clinical and laboratory findings suggestive of Henoch-Schönlein purpura. Glucocorticoid therapy was initiated, but five days following her admission, she developed altered consciousness, seizures, arterial hypertension, and cortical blindness. Brain MRI scan revealed areas of vasogenic oedema in parieto-occipital lobes, consistent with posterior reversible encephalopathy syndrome. She was immediately initiated on antihypertensives and antiepileptics, which successfully improved her neurologic symptoms. Further laboratory work-up disclosed a rapidly progressive glomerulonephritis secondary to Henoch-Schönlein purpura that was the likely cause of her sudden blood pressure elevation. Immunosuppressive therapy was undertaken, and at one-year follow-up, the patient exhibited complete renal and neurologic recovery. Conclusion Posterior reversible encephalopathy syndrome is a severe complication of Henoch-Schönlein purpura. If promptly diagnosed and treated, children with Henoch-Schönlein purpura presenting with posterior reversible encephalopathy syndrome usually have a good prognosis. Clinicians should be familiar with the characteristic presentation of posterior reversible encephalopathy syndrome and be aware that hypertension and renal injury may predispose Henoch-Schönlein purpura patients to developing this complication.

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