Púrpura de Schönlein-Henoch: a propósito de un caso

Fernández, Inmaculada Macías

doi:10.1016/j.reuma.2009.03.008

Cited by 2 publications

(1 citation statement)

References 5 publications

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“…Se sospechaba que bacterias, virus o agentes parasitarios desencadenaban la A pesar de ser una vasculitis autolimitada, hay recidivas en solo un tercio de los casos. Es el principal factor pronóstico: a mayor edad peor pronóstico y mayor riesgo de nefritis, persistencia del (6) síndrome nefrótico y extensión de los depósitos de IgA a las paredes de los capilares .…”

Section: Introductionunclassified

Schönlein-Henoch purpura in an adult patient

Franco

Alvarado

Mendoza

et al. 2022

Rev. virtual Soc. Parag. Med. Int

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La púrpura de Schönlein-Henoch es una vasculitis por fragmentación de leucocitos inmunomediada que afecta a pequeños vasos sanguíneos. Los cuatro componentes clínicos esenciales son púrpuras, dolor abdominal, artralgia y afectación renal. El caso trata de una mujer de 50 años que ingresa por dolor abdominal y hematoquecia de 72 horas de evolución, posterior a laparotomía exploratoria. Al examen físico presenta lesiones purpúricas en tronco y extremidades inferiores de 2 meses de aparición. En paraclínicos se observa hemograma con plaquetas normales, proteínas en orina 500 mg/dL, proteinuria 2,4 g/24 hs. Ante sospecha de vasculitis con plausible inclusión cutáneo-renal, se pide anticuerpos antinucleares, ANCA y se realiza biopsia cutánea evidenciándose una vasculitis neutrofílica necrotizante de pequeños vasos. En la biopsia renal se observa en inmunofluorescencia directa depósito de IgA, C3 positivo. En relación clínica de la proteinuria y compromiso cutáneo junto con la confirmación de biopsia renal se concluye en diagnóstico de púrpura de Schönlein Henoch. El interés de este caso radica en la inconsistencia de esta patología en los adultos, a pesar de que bien podría ser de una gravedad más notable dado que existe un mayor peligro de falla renal persistente.

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Section: Introductionunclassified

Schönlein-Henoch purpura in an adult patient

Franco

Alvarado

Mendoza

et al. 2022

Rev. virtual Soc. Parag. Med. Int

View full text Add to dashboard Cite

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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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Púrpura de Schönlein-Henoch: a propósito de un caso

Cited by 2 publications

References 5 publications

Schönlein-Henoch purpura in an adult patient

Schönlein-Henoch purpura in an adult patient

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

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