Successful Treatment of Sjögren's Syndrome Presenting as a Condition Similar to Chronic Capillary Leak Syndrome Using Combination Therapy with High-Dose Intravenous Immunoglobulin and Glucocorticoid

A 70-year-old woman with Sjögren's syndrome (SS) complained of generalized edema. Computed tomography showed thoracoabdominal fluid, suggesting serositis with SS. 35 mg/day of prednisolone as a monotherapy was ineffective. Moreover, hemoconcentration with hypoalbuminemia without inflammatory si...

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Autores principales: Tokura, Masami, Niwano, Tomoko, Nagasaka, Kenji
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2019
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6425372/
https://www.ncbi.nlm.nih.gov/pubmed/30956836
http://dx.doi.org/10.1155/2019/4865024
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author Tokura, Masami
Niwano, Tomoko
Nagasaka, Kenji
author_facet Tokura, Masami
Niwano, Tomoko
Nagasaka, Kenji
author_sort Tokura, Masami
collection PubMed
description A 70-year-old woman with Sjögren's syndrome (SS) complained of generalized edema. Computed tomography showed thoracoabdominal fluid, suggesting serositis with SS. 35 mg/day of prednisolone as a monotherapy was ineffective. Moreover, hemoconcentration with hypoalbuminemia without inflammatory signs lead us to consider the systemic capillary leak syndrome (SCLS). Additional treatment with intravenous immunoglobulin (IVIG) and prednisolone dramatically decreased the thoracoabdominal fluid. However, when reducing the prednisolone dose, the thoracoabdominal fluid reincreased. Retreatment with IVIG without increasing the prednisolone dose was ineffective. However, additional prednisolone of 35 mg/day was effective, suggesting SCLS with SS might require combination therapy with IVIG and glucocorticoid.
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spelling pubmed-64253722019-04-07 Successful Treatment of Sjögren's Syndrome Presenting as a Condition Similar to Chronic Capillary Leak Syndrome Using Combination Therapy with High-Dose Intravenous Immunoglobulin and Glucocorticoid Tokura, Masami Niwano, Tomoko Nagasaka, Kenji Case Rep Rheumatol Case Report A 70-year-old woman with Sjögren's syndrome (SS) complained of generalized edema. Computed tomography showed thoracoabdominal fluid, suggesting serositis with SS. 35 mg/day of prednisolone as a monotherapy was ineffective. Moreover, hemoconcentration with hypoalbuminemia without inflammatory signs lead us to consider the systemic capillary leak syndrome (SCLS). Additional treatment with intravenous immunoglobulin (IVIG) and prednisolone dramatically decreased the thoracoabdominal fluid. However, when reducing the prednisolone dose, the thoracoabdominal fluid reincreased. Retreatment with IVIG without increasing the prednisolone dose was ineffective. However, additional prednisolone of 35 mg/day was effective, suggesting SCLS with SS might require combination therapy with IVIG and glucocorticoid. Hindawi 2019-03-04 /pmc/articles/PMC6425372/ /pubmed/30956836 http://dx.doi.org/10.1155/2019/4865024 Text en Copyright © 2019 Masami Tokura et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Tokura, Masami
Niwano, Tomoko
Nagasaka, Kenji
Successful Treatment of Sjögren's Syndrome Presenting as a Condition Similar to Chronic Capillary Leak Syndrome Using Combination Therapy with High-Dose Intravenous Immunoglobulin and Glucocorticoid
title Successful Treatment of Sjögren's Syndrome Presenting as a Condition Similar to Chronic Capillary Leak Syndrome Using Combination Therapy with High-Dose Intravenous Immunoglobulin and Glucocorticoid
title_full Successful Treatment of Sjögren's Syndrome Presenting as a Condition Similar to Chronic Capillary Leak Syndrome Using Combination Therapy with High-Dose Intravenous Immunoglobulin and Glucocorticoid
title_fullStr Successful Treatment of Sjögren's Syndrome Presenting as a Condition Similar to Chronic Capillary Leak Syndrome Using Combination Therapy with High-Dose Intravenous Immunoglobulin and Glucocorticoid
title_full_unstemmed Successful Treatment of Sjögren's Syndrome Presenting as a Condition Similar to Chronic Capillary Leak Syndrome Using Combination Therapy with High-Dose Intravenous Immunoglobulin and Glucocorticoid
title_short Successful Treatment of Sjögren's Syndrome Presenting as a Condition Similar to Chronic Capillary Leak Syndrome Using Combination Therapy with High-Dose Intravenous Immunoglobulin and Glucocorticoid
title_sort successful treatment of sjögren's syndrome presenting as a condition similar to chronic capillary leak syndrome using combination therapy with high-dose intravenous immunoglobulin and glucocorticoid
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6425372/
https://www.ncbi.nlm.nih.gov/pubmed/30956836
http://dx.doi.org/10.1155/2019/4865024
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