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Case Report: Guillain-Barré Syndrome as Primary Presentation of Systemic Lupus Erythematosus (SLE-GBS) in a Teenage Girl

Peripheral nervous system involvement accounts for fewer than 10% of SLE cases with neuropsychiatric manifestations. Guillain-Barré syndrome (GBS) as the presenting, major manifestation of pediatric SLE is extremely rare, and the best treatment approach is unknown. A 14-year-old, previously healthy...

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Autores principales: Beshir, Elham, Belt, Ernestina, Chencheri, Nidheesh, Saqib, Aqdas, Pallavidino, Marco, Terheggen, Ulrich, Abdalla, Abdalla, Herlitz, Leal, Sharif, Elsadeg, Bitzan, Martin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8968442/
https://www.ncbi.nlm.nih.gov/pubmed/35372162
http://dx.doi.org/10.3389/fped.2022.838927
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author Beshir, Elham
Belt, Ernestina
Chencheri, Nidheesh
Saqib, Aqdas
Pallavidino, Marco
Terheggen, Ulrich
Abdalla, Abdalla
Herlitz, Leal
Sharif, Elsadeg
Bitzan, Martin
author_facet Beshir, Elham
Belt, Ernestina
Chencheri, Nidheesh
Saqib, Aqdas
Pallavidino, Marco
Terheggen, Ulrich
Abdalla, Abdalla
Herlitz, Leal
Sharif, Elsadeg
Bitzan, Martin
author_sort Beshir, Elham
collection PubMed
description Peripheral nervous system involvement accounts for fewer than 10% of SLE cases with neuropsychiatric manifestations. Guillain-Barré syndrome (GBS) as the presenting, major manifestation of pediatric SLE is extremely rare, and the best treatment approach is unknown. A 14-year-old, previously healthy female teenager developed classic features of GBS with ascending bilateral muscle weakness leading to respiratory insufficiency, associated with protein-cell dissociation in cerebro-spinal fluid, nerve root enhancement by MRI and reduction in compound muscle action potential amplitude. SLE was diagnosed serologically and histologically (lupus nephritis WHO class II). Despite immediate treatment with intravenous immunoglobulin (IVIg), methylprednisolone pulses and subsequently, rituximab, the patient required prolonged mechanical ventilation. She achieved full recovery following 14 PLEX treatments and two more rituximab infusions. Anti-dsDNA, C3, C4 and urinalysis normalized while anti-Smith and Sjögren antibodies persisted 15 months after disease onset, with no other lupus manifestations. Review of the literature revealed two pediatric cases of GBS at the onset of SLE and a third case with GBS 6 years after the diagnosis of SLE. Conventional GBS therapy may not be adequate to treat SLE-GBS. SLE should be included in the differential diagnosis of GBS. Importantly, treatment experiences and outcomes of such cases need be reported to inform future treatment recommendations.
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spelling pubmed-89684422022-04-01 Case Report: Guillain-Barré Syndrome as Primary Presentation of Systemic Lupus Erythematosus (SLE-GBS) in a Teenage Girl Beshir, Elham Belt, Ernestina Chencheri, Nidheesh Saqib, Aqdas Pallavidino, Marco Terheggen, Ulrich Abdalla, Abdalla Herlitz, Leal Sharif, Elsadeg Bitzan, Martin Front Pediatr Pediatrics Peripheral nervous system involvement accounts for fewer than 10% of SLE cases with neuropsychiatric manifestations. Guillain-Barré syndrome (GBS) as the presenting, major manifestation of pediatric SLE is extremely rare, and the best treatment approach is unknown. A 14-year-old, previously healthy female teenager developed classic features of GBS with ascending bilateral muscle weakness leading to respiratory insufficiency, associated with protein-cell dissociation in cerebro-spinal fluid, nerve root enhancement by MRI and reduction in compound muscle action potential amplitude. SLE was diagnosed serologically and histologically (lupus nephritis WHO class II). Despite immediate treatment with intravenous immunoglobulin (IVIg), methylprednisolone pulses and subsequently, rituximab, the patient required prolonged mechanical ventilation. She achieved full recovery following 14 PLEX treatments and two more rituximab infusions. Anti-dsDNA, C3, C4 and urinalysis normalized while anti-Smith and Sjögren antibodies persisted 15 months after disease onset, with no other lupus manifestations. Review of the literature revealed two pediatric cases of GBS at the onset of SLE and a third case with GBS 6 years after the diagnosis of SLE. Conventional GBS therapy may not be adequate to treat SLE-GBS. SLE should be included in the differential diagnosis of GBS. Importantly, treatment experiences and outcomes of such cases need be reported to inform future treatment recommendations. Frontiers Media S.A. 2022-03-17 /pmc/articles/PMC8968442/ /pubmed/35372162 http://dx.doi.org/10.3389/fped.2022.838927 Text en Copyright © 2022 Beshir, Belt, Chencheri, Saqib, Pallavidino, Terheggen, Abdalla, Herlitz, Sharif and Bitzan. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Pediatrics
Beshir, Elham
Belt, Ernestina
Chencheri, Nidheesh
Saqib, Aqdas
Pallavidino, Marco
Terheggen, Ulrich
Abdalla, Abdalla
Herlitz, Leal
Sharif, Elsadeg
Bitzan, Martin
Case Report: Guillain-Barré Syndrome as Primary Presentation of Systemic Lupus Erythematosus (SLE-GBS) in a Teenage Girl
title Case Report: Guillain-Barré Syndrome as Primary Presentation of Systemic Lupus Erythematosus (SLE-GBS) in a Teenage Girl
title_full Case Report: Guillain-Barré Syndrome as Primary Presentation of Systemic Lupus Erythematosus (SLE-GBS) in a Teenage Girl
title_fullStr Case Report: Guillain-Barré Syndrome as Primary Presentation of Systemic Lupus Erythematosus (SLE-GBS) in a Teenage Girl
title_full_unstemmed Case Report: Guillain-Barré Syndrome as Primary Presentation of Systemic Lupus Erythematosus (SLE-GBS) in a Teenage Girl
title_short Case Report: Guillain-Barré Syndrome as Primary Presentation of Systemic Lupus Erythematosus (SLE-GBS) in a Teenage Girl
title_sort case report: guillain-barré syndrome as primary presentation of systemic lupus erythematosus (sle-gbs) in a teenage girl
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8968442/
https://www.ncbi.nlm.nih.gov/pubmed/35372162
http://dx.doi.org/10.3389/fped.2022.838927
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