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 signs l...

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Main Authors: Masami Tokura, Tomoko Niwano, Kenji Nagasaka
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2019/4865024
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author Masami Tokura
Tomoko Niwano
Kenji Nagasaka
author_facet Masami Tokura
Tomoko Niwano
Kenji Nagasaka
author_sort Masami Tokura
collection DOAJ
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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publishDate 2019-01-01
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series Case Reports in Rheumatology
spelling doaj-art-c41f4952c70c4d66b0cee7e0007aeb632025-08-20T02:18:57ZengWileyCase Reports in Rheumatology2090-68892090-68972019-01-01201910.1155/2019/48650244865024Successful Treatment of Sjögren’s Syndrome Presenting as a Condition Similar to Chronic Capillary Leak Syndrome Using Combination Therapy with High-Dose Intravenous Immunoglobulin and GlucocorticoidMasami Tokura0Tomoko Niwano1Kenji Nagasaka2Department of Rheumatology, Ome Municipal General Hospital, Ome, JapanDepartment of Rheumatology, Ome Municipal General Hospital, Ome, JapanDepartment of Rheumatology, Ome Municipal General Hospital, Ome, JapanA 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.http://dx.doi.org/10.1155/2019/4865024
spellingShingle Masami Tokura
Tomoko Niwano
Kenji Nagasaka
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
Case Reports in Rheumatology
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 sjogren s syndrome presenting as a condition similar to chronic capillary leak syndrome using combination therapy with high dose intravenous immunoglobulin and glucocorticoid
url http://dx.doi.org/10.1155/2019/4865024
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