Resection of lesions in the ileum of patients with IgG4-related disease may ameliorate disease progression without steroid administration
Abstract Background IgG4-related disease (IgG4-RD) is a pathological condition that is characterized by an infiltrate composed of IgG4-positive plasma cells and recently recognized as an immune-mediated condition. It causes tissue throughout the body to become stiff and thickened due to autoimmune r...
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Japan Surgical Society
2018-12-01
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| Series: | Surgical Case Reports |
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| Online Access: | http://link.springer.com/article/10.1186/s40792-018-0546-9 |
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| author | Akihiro Watanabe Takashi Goto Hitomi Kamo Ryuji Komine Naomi Kuroki Takanobu Sugase Tsuyoshi Takaya Rintaro Koga Hiroshi Hojo Shoji Taniguchi Kazuhiko Ibusuki Kazumi Koga |
| author_facet | Akihiro Watanabe Takashi Goto Hitomi Kamo Ryuji Komine Naomi Kuroki Takanobu Sugase Tsuyoshi Takaya Rintaro Koga Hiroshi Hojo Shoji Taniguchi Kazuhiko Ibusuki Kazumi Koga |
| author_sort | Akihiro Watanabe |
| collection | DOAJ |
| description | Abstract Background IgG4-related disease (IgG4-RD) is a pathological condition that is characterized by an infiltrate composed of IgG4-positive plasma cells and recently recognized as an immune-mediated condition. It causes tissue throughout the body to become stiff and thickened due to autoimmune reactions that cause fibrosis and scarring. Disease-related changes commonly occur in the salivary glands, bile duct, pancreas, and lungs, but are seldom seen in the small bowel. A diagnosis of IgG4-RD is suspected if a high level of IgG4 is found on a blood test. The ideal diagnostic method is pathological examination, but because the clinical manifestations of IgG4-RD are very diverse and nonspecific, the disease may often go undiagnosed until an unrelated biopsy or resection specimen is obtained. The most common treatment for IgG4-RD is steroid use. However, tapering or stopping steroid administration is seen to result in recurrence in approximately 50% of cases. A complete cure is therefore considered extremely difficult. Case presentation A 69-year-old man with gastrointestinal obstruction underwent small bowel resection for two lesions. On histopathological examination, the specimen showed features of IgG4-RD. We performed several tests to detect other characteristics of IgG4-RD, but were unable to find any. The patient is being followed up regularly for a year and is being observed for any symptoms of recurrence. Conclusions We present a case of IgG4-RD wherein the ileum wall was significantly sclerosed, leading to gastrointestinal tract obstruction; therefore, we resected two sections of the ileum. We believe that resection of IgG4-RD lesions can help avoid long-term steroid use in patients, because the surgery completely eliminates the pathological origins of the condition. |
| format | Article |
| id | doaj-art-17b0982df4fe405b8d5738cea1d623ff |
| institution | DOAJ |
| issn | 2198-7793 |
| language | English |
| publishDate | 2018-12-01 |
| publisher | Japan Surgical Society |
| record_format | Article |
| series | Surgical Case Reports |
| spelling | doaj-art-17b0982df4fe405b8d5738cea1d623ff2025-08-20T03:15:12ZengJapan Surgical SocietySurgical Case Reports2198-77932018-12-01411610.1186/s40792-018-0546-9Resection of lesions in the ileum of patients with IgG4-related disease may ameliorate disease progression without steroid administrationAkihiro Watanabe0Takashi Goto1Hitomi Kamo2Ryuji Komine3Naomi Kuroki4Takanobu Sugase5Tsuyoshi Takaya6Rintaro Koga7Hiroshi Hojo8Shoji Taniguchi9Kazuhiko Ibusuki10Kazumi Koga11Department of Gastrointestinal and General Surgery, Mitsui Memorial HospitalDepartment of Gastrointestinal and General Surgery, Koga General HospitalDepartment of Gastrointestinal and General Surgery, Koga General HospitalDepartment of Gastrointestinal and General Surgery, Mitsui Memorial HospitalDepartment of Gastrointestinal and General Surgery, Koga General HospitalDepartment of Gastrointestinal and General Surgery, Koga General HospitalDepartment of Gastrointestinal and General Surgery, Koga General HospitalDepartment of Gastrointestinal and General Surgery, Koga General HospitalDepartment of Gastrointestinal and General Surgery, Koga General HospitalDepartment of Gastrointestinal and General Surgery, Koga General HospitalDepartment of Gastrointestinal and General Surgery, Koga General HospitalDepartment of Gastrointestinal and General Surgery, Koga General HospitalAbstract Background IgG4-related disease (IgG4-RD) is a pathological condition that is characterized by an infiltrate composed of IgG4-positive plasma cells and recently recognized as an immune-mediated condition. It causes tissue throughout the body to become stiff and thickened due to autoimmune reactions that cause fibrosis and scarring. Disease-related changes commonly occur in the salivary glands, bile duct, pancreas, and lungs, but are seldom seen in the small bowel. A diagnosis of IgG4-RD is suspected if a high level of IgG4 is found on a blood test. The ideal diagnostic method is pathological examination, but because the clinical manifestations of IgG4-RD are very diverse and nonspecific, the disease may often go undiagnosed until an unrelated biopsy or resection specimen is obtained. The most common treatment for IgG4-RD is steroid use. However, tapering or stopping steroid administration is seen to result in recurrence in approximately 50% of cases. A complete cure is therefore considered extremely difficult. Case presentation A 69-year-old man with gastrointestinal obstruction underwent small bowel resection for two lesions. On histopathological examination, the specimen showed features of IgG4-RD. We performed several tests to detect other characteristics of IgG4-RD, but were unable to find any. The patient is being followed up regularly for a year and is being observed for any symptoms of recurrence. Conclusions We present a case of IgG4-RD wherein the ileum wall was significantly sclerosed, leading to gastrointestinal tract obstruction; therefore, we resected two sections of the ileum. We believe that resection of IgG4-RD lesions can help avoid long-term steroid use in patients, because the surgery completely eliminates the pathological origins of the condition.http://link.springer.com/article/10.1186/s40792-018-0546-9IgG4-related sclerosing diseaseSmall bowel resectionSteroid |
| spellingShingle | Akihiro Watanabe Takashi Goto Hitomi Kamo Ryuji Komine Naomi Kuroki Takanobu Sugase Tsuyoshi Takaya Rintaro Koga Hiroshi Hojo Shoji Taniguchi Kazuhiko Ibusuki Kazumi Koga Resection of lesions in the ileum of patients with IgG4-related disease may ameliorate disease progression without steroid administration Surgical Case Reports IgG4-related sclerosing disease Small bowel resection Steroid |
| title | Resection of lesions in the ileum of patients with IgG4-related disease may ameliorate disease progression without steroid administration |
| title_full | Resection of lesions in the ileum of patients with IgG4-related disease may ameliorate disease progression without steroid administration |
| title_fullStr | Resection of lesions in the ileum of patients with IgG4-related disease may ameliorate disease progression without steroid administration |
| title_full_unstemmed | Resection of lesions in the ileum of patients with IgG4-related disease may ameliorate disease progression without steroid administration |
| title_short | Resection of lesions in the ileum of patients with IgG4-related disease may ameliorate disease progression without steroid administration |
| title_sort | resection of lesions in the ileum of patients with igg4 related disease may ameliorate disease progression without steroid administration |
| topic | IgG4-related sclerosing disease Small bowel resection Steroid |
| url | http://link.springer.com/article/10.1186/s40792-018-0546-9 |
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