Multivisceral IgG4-related disease presenting as recurrent massive gastrointestinal bleeding: a case report and literature review
Abstract Background IgG4-related disease (IgG4-RD) is a newly recognized autoimmune systemic disorder characterized by elevated levels of serum IgG4 and abundant infiltration of IgG4-positive plasmacytes in the affected organs. The liver, biliary system and pancreas are the most commonly affected or...
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| Format: | Article |
| Language: | English |
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BMC
2018-09-01
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| Series: | BMC Gastroenterology |
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| Online Access: | http://link.springer.com/article/10.1186/s12876-018-0867-y |
| _version_ | 1818291111219691520 |
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| author | Xuexue Deng Ronghua Fang Jianshu Zhang Rongqiong Li |
| author_facet | Xuexue Deng Ronghua Fang Jianshu Zhang Rongqiong Li |
| author_sort | Xuexue Deng |
| collection | DOAJ |
| description | Abstract Background IgG4-related disease (IgG4-RD) is a newly recognized autoimmune systemic disorder characterized by elevated levels of serum IgG4 and abundant infiltration of IgG4-positive plasmacytes in the affected organs. The liver, biliary system and pancreas are the most commonly affected organs. However, involvement of the digestive tract is very rare. To date, only a few cases of isolated gastric IgG4-RD have been reported. Case presentation We present a case of IgG4-RD of the liver, gallbladder, pancreas and duodenum, which was clinically misinterpreted and thereafter over-treated. A 52-year-old male presented with obstructive jaundice for 3 years, melena for 5 months and hematemesis for 10 days. Three years prior, the patient had undergone biopsies of pancreatic lesions, liver lesions, cholecystectomy and choledochojejunostomy. Histopathology showed chronic inflammatory changes. Endoscopy at admission revealed a duodenal ulcer with active bleeding. Despite medical management, the patient presented with repeated gastrointestinal bleeding. Upon evaluation, serum IgG4 levels were found to be elevated. Histopathology of the duodenal ulcer biopsy and repeated examination of the gallbladder and pancreatic and liver biopsies confirmed IgG4 positive plasma cell infiltration. A definitive diagnosis of IgG4-RD was made and steroid administration was initiated. At last follow up, 11 months to-the-day after initiating steroid treatment, the patient was asymptomatic. Conclusions Notably, IgG4-RD of multiple digestive organs is still very rare. As a systemic disease, it is characterized by the infiltration of IgG4-bearing plasma cells and raised IgG4 levels. Histopathology findings remain the diagnostic gold standard for this disorder. |
| first_indexed | 2024-12-13T02:38:52Z |
| format | Article |
| id | doaj.art-0da7a61d7c364116a8edd0d98cc82909 |
| institution | Directory Open Access Journal |
| issn | 1471-230X |
| language | English |
| last_indexed | 2024-12-13T02:38:52Z |
| publishDate | 2018-09-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Gastroenterology |
| spelling | doaj.art-0da7a61d7c364116a8edd0d98cc829092022-12-22T00:02:20ZengBMCBMC Gastroenterology1471-230X2018-09-011811710.1186/s12876-018-0867-yMultivisceral IgG4-related disease presenting as recurrent massive gastrointestinal bleeding: a case report and literature reviewXuexue Deng0Ronghua Fang1Jianshu Zhang2Rongqiong Li3Department of General Medicine, West China Hospital, Sichuan UniversityDepartment of General Medicine, West China Hospital, Sichuan UniversityDepartment of General Medicine, West China Hospital, Sichuan UniversityDepartment of General Medicine, West China Hospital, Sichuan UniversityAbstract Background IgG4-related disease (IgG4-RD) is a newly recognized autoimmune systemic disorder characterized by elevated levels of serum IgG4 and abundant infiltration of IgG4-positive plasmacytes in the affected organs. The liver, biliary system and pancreas are the most commonly affected organs. However, involvement of the digestive tract is very rare. To date, only a few cases of isolated gastric IgG4-RD have been reported. Case presentation We present a case of IgG4-RD of the liver, gallbladder, pancreas and duodenum, which was clinically misinterpreted and thereafter over-treated. A 52-year-old male presented with obstructive jaundice for 3 years, melena for 5 months and hematemesis for 10 days. Three years prior, the patient had undergone biopsies of pancreatic lesions, liver lesions, cholecystectomy and choledochojejunostomy. Histopathology showed chronic inflammatory changes. Endoscopy at admission revealed a duodenal ulcer with active bleeding. Despite medical management, the patient presented with repeated gastrointestinal bleeding. Upon evaluation, serum IgG4 levels were found to be elevated. Histopathology of the duodenal ulcer biopsy and repeated examination of the gallbladder and pancreatic and liver biopsies confirmed IgG4 positive plasma cell infiltration. A definitive diagnosis of IgG4-RD was made and steroid administration was initiated. At last follow up, 11 months to-the-day after initiating steroid treatment, the patient was asymptomatic. Conclusions Notably, IgG4-RD of multiple digestive organs is still very rare. As a systemic disease, it is characterized by the infiltration of IgG4-bearing plasma cells and raised IgG4 levels. Histopathology findings remain the diagnostic gold standard for this disorder.http://link.springer.com/article/10.1186/s12876-018-0867-yIgG4-related diseasePlasma cellsLiverGallbladderDuodenalPancreatic |
| spellingShingle | Xuexue Deng Ronghua Fang Jianshu Zhang Rongqiong Li Multivisceral IgG4-related disease presenting as recurrent massive gastrointestinal bleeding: a case report and literature review BMC Gastroenterology IgG4-related disease Plasma cells Liver Gallbladder Duodenal Pancreatic |
| title | Multivisceral IgG4-related disease presenting as recurrent massive gastrointestinal bleeding: a case report and literature review |
| title_full | Multivisceral IgG4-related disease presenting as recurrent massive gastrointestinal bleeding: a case report and literature review |
| title_fullStr | Multivisceral IgG4-related disease presenting as recurrent massive gastrointestinal bleeding: a case report and literature review |
| title_full_unstemmed | Multivisceral IgG4-related disease presenting as recurrent massive gastrointestinal bleeding: a case report and literature review |
| title_short | Multivisceral IgG4-related disease presenting as recurrent massive gastrointestinal bleeding: a case report and literature review |
| title_sort | multivisceral igg4 related disease presenting as recurrent massive gastrointestinal bleeding a case report and literature review |
| topic | IgG4-related disease Plasma cells Liver Gallbladder Duodenal Pancreatic |
| url | http://link.springer.com/article/10.1186/s12876-018-0867-y |
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