Hypokalemic Paralysis: The first presentation of Primary Sjögren’s Syndrome
Autor(a) principal: | |
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Data de Publicação: | 2012 |
Outros Autores: | , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10 |
Resumo: | The Sjögren's syndrome is a systemic autoimmune disorder characterized by chronic inflammation of the exocrine glands with extraglandular manifestations in up to 25% patients. Renal involvement occurs in 18.4-67% of cases, with tubulointerstitial nephritis being the most frequent pathology. We present the case of a 37 year-old woman admitted because of generalized grade 2 muscle weakness which developed over a week. We detected: hypokalemia, rhabdomyolysis, urinary pH 6.5, proteinuria and metabolic acidemia. The laboratory tests suggestive of distal renal tubular acidosis with hypokalaemia led to the diagnosis of lymphoplasmocytic tubulointerstitial nephritis, which was confirmed by renal biopsy, and to a clinical suspicion of Sjögren’s syndrome. Primary Sjögren's syndrome was diagnosed in this patient based on the following criteria: xerophthalmia, xerostomia, sialadenitis, positive anti-SSA and anti-SSB antibodies, and absence of criteria for lupus and rheumatoid arthritis. During hospitalization, the patient developed deep vein thrombosis. Tests showed positive antiphospholipid antibodies and the diagnosis of secondary antiphospholipid syndrome was made. She was treated with potassium, bicarbonate, steroids, ramipril and warfarin. The authors wish to highlight the extraglandular manifestations and in particular the rarity of hypokalemic paralysis as the presenting manifestation of primary Sjögren’s syndrome. |
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Hypokalemic Paralysis: The first presentation of Primary Sjögren’s SyndromeParalisia Por Hipocaliémia: Manifestação Inaugural da Síndrome de Sjögren PrimárioThe Sjögren's syndrome is a systemic autoimmune disorder characterized by chronic inflammation of the exocrine glands with extraglandular manifestations in up to 25% patients. Renal involvement occurs in 18.4-67% of cases, with tubulointerstitial nephritis being the most frequent pathology. We present the case of a 37 year-old woman admitted because of generalized grade 2 muscle weakness which developed over a week. We detected: hypokalemia, rhabdomyolysis, urinary pH 6.5, proteinuria and metabolic acidemia. The laboratory tests suggestive of distal renal tubular acidosis with hypokalaemia led to the diagnosis of lymphoplasmocytic tubulointerstitial nephritis, which was confirmed by renal biopsy, and to a clinical suspicion of Sjögren’s syndrome. Primary Sjögren's syndrome was diagnosed in this patient based on the following criteria: xerophthalmia, xerostomia, sialadenitis, positive anti-SSA and anti-SSB antibodies, and absence of criteria for lupus and rheumatoid arthritis. During hospitalization, the patient developed deep vein thrombosis. Tests showed positive antiphospholipid antibodies and the diagnosis of secondary antiphospholipid syndrome was made. She was treated with potassium, bicarbonate, steroids, ramipril and warfarin. The authors wish to highlight the extraglandular manifestations and in particular the rarity of hypokalemic paralysis as the presenting manifestation of primary Sjögren’s syndrome.O Síndrome de Sjögren é uma doença auto-imune sistémica caracterizada por inflamação crónica das glândulas exócrinas. As manifestações extraglandulares têm uma incidência até 25%. O envolvimento renal ocorre em 18,4-67% dos casos, e nestes a nefrite tubulointersticial é a lesão mais frequente. Apresenta-se o caso clínico de uma mulher de 37 anos, internada por fraqueza muscular generalizada de grau 2 com uma semana de evolução. Objectivou-se hipocaliémia, rabdomiólise, pH urinário 6,5, proteinúria, e acidémia metabólica. O estudo da acidose renal tubular distal com hipocaliémia conduziu ao diagnóstico de nefrite tubulo-intersticial linfoplasmocitária, confirmada na biópsia renal, e à suspeita de Síndrome de Sjögren. O Síndrome de Sjögren primário foi diagnosticado nesta doente por estarem presentes os seguintes critérios: xeroftalmia, xerostomia, sialadenite, anticorpos anti-SSA e anti-SSB positivos e ausência de critérios para lúpus e artrite reumatóide. Durante o internamento ocorreu trombose venosa profunda do membro inferior direito e por anticorpos antifosfolípidos positivos, foi diagnosticado síndrome antifosfolípido secundário. Foi medicada com potássio, bicarbonato, corticoterapia, ramipril e varfarina. Os autores pretendem alertar para as manifestações extraglandulares e particularmente para a raridade da paralisia por hipocaliémia como forma de apresentação do Síndrome Sjögren primário.Ordem dos Médicos2012-06-25info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10oai:ojs.www.actamedicaportuguesa.com:article/10Acta Médica Portuguesa; Vol. 25 No. 2 (2012): March-April; 122-124Acta Médica Portuguesa; Vol. 25 N.º 2 (2012): Março-Abril; 122-1241646-07580870-399Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10/25Martinho, Aurélia LCapela, AndreiaDuarte, Fernandainfo:eu-repo/semantics/openAccess2022-12-20T10:55:38Zoai:ojs.www.actamedicaportuguesa.com:article/10Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:16:18.110852Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Hypokalemic Paralysis: The first presentation of Primary Sjögren’s Syndrome Paralisia Por Hipocaliémia: Manifestação Inaugural da Síndrome de Sjögren Primário |
title |
Hypokalemic Paralysis: The first presentation of Primary Sjögren’s Syndrome |
spellingShingle |
Hypokalemic Paralysis: The first presentation of Primary Sjögren’s Syndrome Martinho, Aurélia L |
title_short |
Hypokalemic Paralysis: The first presentation of Primary Sjögren’s Syndrome |
title_full |
Hypokalemic Paralysis: The first presentation of Primary Sjögren’s Syndrome |
title_fullStr |
Hypokalemic Paralysis: The first presentation of Primary Sjögren’s Syndrome |
title_full_unstemmed |
Hypokalemic Paralysis: The first presentation of Primary Sjögren’s Syndrome |
title_sort |
Hypokalemic Paralysis: The first presentation of Primary Sjögren’s Syndrome |
author |
Martinho, Aurélia L |
author_facet |
Martinho, Aurélia L Capela, Andreia Duarte, Fernanda |
author_role |
author |
author2 |
Capela, Andreia Duarte, Fernanda |
author2_role |
author author |
dc.contributor.author.fl_str_mv |
Martinho, Aurélia L Capela, Andreia Duarte, Fernanda |
description |
The Sjögren's syndrome is a systemic autoimmune disorder characterized by chronic inflammation of the exocrine glands with extraglandular manifestations in up to 25% patients. Renal involvement occurs in 18.4-67% of cases, with tubulointerstitial nephritis being the most frequent pathology. We present the case of a 37 year-old woman admitted because of generalized grade 2 muscle weakness which developed over a week. We detected: hypokalemia, rhabdomyolysis, urinary pH 6.5, proteinuria and metabolic acidemia. The laboratory tests suggestive of distal renal tubular acidosis with hypokalaemia led to the diagnosis of lymphoplasmocytic tubulointerstitial nephritis, which was confirmed by renal biopsy, and to a clinical suspicion of Sjögren’s syndrome. Primary Sjögren's syndrome was diagnosed in this patient based on the following criteria: xerophthalmia, xerostomia, sialadenitis, positive anti-SSA and anti-SSB antibodies, and absence of criteria for lupus and rheumatoid arthritis. During hospitalization, the patient developed deep vein thrombosis. Tests showed positive antiphospholipid antibodies and the diagnosis of secondary antiphospholipid syndrome was made. She was treated with potassium, bicarbonate, steroids, ramipril and warfarin. The authors wish to highlight the extraglandular manifestations and in particular the rarity of hypokalemic paralysis as the presenting manifestation of primary Sjögren’s syndrome. |
publishDate |
2012 |
dc.date.none.fl_str_mv |
2012-06-25 |
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info:eu-repo/semantics/publishedVersion |
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info:eu-repo/semantics/article |
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article |
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10 oai:ojs.www.actamedicaportuguesa.com:article/10 |
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10 |
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oai:ojs.www.actamedicaportuguesa.com:article/10 |
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por |
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10 https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10/25 |
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info:eu-repo/semantics/openAccess |
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openAccess |
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application/pdf |
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Ordem dos Médicos |
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Ordem dos Médicos |
dc.source.none.fl_str_mv |
Acta Médica Portuguesa; Vol. 25 No. 2 (2012): March-April; 122-124 Acta Médica Portuguesa; Vol. 25 N.º 2 (2012): Março-Abril; 122-124 1646-0758 0870-399X reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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