IgG4-Related Disease Complicated with Diffuse and Chronic Gastrointestinal Inflammation Leading to Small Intestinal Perforation
© Japan College of Rheumatology 2024. Published by Oxford University Press. All rights reserved. For commercial re-use, please contact reprintsoup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site–for further information please contact journals.permissions@oup.com..
Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by elevated serum IgG4, IgG4+ cell infiltration, storiform fibrosis, and obliterative phlebitis. While IgG4-RD can affect various organs, gastrointestinal tract involvement is less common. Here, we report a 70-year-old female with IgG4-RD complicated with diffuse and chronic gastrointestinal inflammation which led to small intestinal perforation. She had been suffering from anorexia, abdominal pain, vomiting, and diarrhea, and hospitalized due to recurrent ileus. Consequently, she was referred due to small intestinal perforation required for surgical intervention. Pathology revealed acute and chronic inflammation with massive IgG4+ plasmacytes infiltration into mucosa of the small intestine, and ischemic change secondarily caused by chronic inflammation. Random biopsies from the mucosa of stomach, duodenum, ileum, and colon, also revealed diffuse and massive IgG4+ plasmacytes infiltration in stomach, duodenum, small intestine, and colon. She was diagnosed with IgG4-RD based on the pathological findings and elevated serum IgG4 levels. Glucocorticoid rapidly ameliorated the symptoms. IgG4-RD may cause gastrointestinal manifestations and histopathological assessment should be considered, even in the absence of specific characteristics of IgG4-RD.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - year:2024 |
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| Enthalten in: |
Modern rheumatology case reports - (2024) vom: 28. März |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Ino, Kazuma [VerfasserIn] |
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| Links: |
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| Themen: |
Diffuse and chronic gastrointestinal inflammation |
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| Anmerkungen: |
Date Revised 28.03.2024 published: Print-Electronic Citation Status Publisher |
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| doi: |
10.1093/mrcr/rxae012 |
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NLM370368681 |
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| 520 | |a Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by elevated serum IgG4, IgG4+ cell infiltration, storiform fibrosis, and obliterative phlebitis. While IgG4-RD can affect various organs, gastrointestinal tract involvement is less common. Here, we report a 70-year-old female with IgG4-RD complicated with diffuse and chronic gastrointestinal inflammation which led to small intestinal perforation. She had been suffering from anorexia, abdominal pain, vomiting, and diarrhea, and hospitalized due to recurrent ileus. Consequently, she was referred due to small intestinal perforation required for surgical intervention. Pathology revealed acute and chronic inflammation with massive IgG4+ plasmacytes infiltration into mucosa of the small intestine, and ischemic change secondarily caused by chronic inflammation. Random biopsies from the mucosa of stomach, duodenum, ileum, and colon, also revealed diffuse and massive IgG4+ plasmacytes infiltration in stomach, duodenum, small intestine, and colon. She was diagnosed with IgG4-RD based on the pathological findings and elevated serum IgG4 levels. Glucocorticoid rapidly ameliorated the symptoms. IgG4-RD may cause gastrointestinal manifestations and histopathological assessment should be considered, even in the absence of specific characteristics of IgG4-RD | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Immunoglobulin (Ig) G4-related disease (IgG4-RD) | |
| 650 | 4 | |a diffuse and chronic gastrointestinal inflammation | |
| 650 | 4 | |a glucocorticoid | |
| 650 | 4 | |a ileus | |
| 650 | 4 | |a small intestinal perforation | |
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| 700 | 1 | |a Akiya, Masashi |e verfasserin |4 aut | |
| 700 | 1 | |a Kajita, Sabine |e verfasserin |4 aut | |
| 700 | 1 | |a Okina, Sosei |e verfasserin |4 aut | |
| 700 | 1 | |a Sakamoto, Junichi |e verfasserin |4 aut | |
| 700 | 1 | |a Tanaka, Tomoki |e verfasserin |4 aut | |
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| 700 | 1 | |a Wada, Tatsuhiko |e verfasserin |4 aut | |
| 700 | 1 | |a Tanaka, Sumiaki |e verfasserin |4 aut | |
| 700 | 1 | |a Oku, Kenji |e verfasserin |4 aut | |
| 700 | 1 | |a Yamaoka, Kunihiro |e verfasserin |4 aut | |
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