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IgG4-related disease of the ileocecal region mimicking malignancy: A case report

INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease characterized by chronic fibrosing inflammation with abundant IgG4-positive plasma cells, and responds well to steroids. Previous reports of IgG4-RD have focused on pancreatic and extrapancreatic including the gastrointe...

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Autores principales: Hiyoshi, Yukiharu, Oki, Eiji, Zaitsu, Yoko, Ando, Koji, Ito, Shuhei, Saeki, Hiroshi, Morita, Masaru, Yamamoto, Hidetaka, Baba, Hideo, Maehara, Yoshihiko
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4189076/
https://www.ncbi.nlm.nih.gov/pubmed/25194601
http://dx.doi.org/10.1016/j.ijscr.2014.08.003
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author Hiyoshi, Yukiharu
Oki, Eiji
Zaitsu, Yoko
Ando, Koji
Ito, Shuhei
Saeki, Hiroshi
Morita, Masaru
Yamamoto, Hidetaka
Baba, Hideo
Maehara, Yoshihiko
author_facet Hiyoshi, Yukiharu
Oki, Eiji
Zaitsu, Yoko
Ando, Koji
Ito, Shuhei
Saeki, Hiroshi
Morita, Masaru
Yamamoto, Hidetaka
Baba, Hideo
Maehara, Yoshihiko
author_sort Hiyoshi, Yukiharu
collection PubMed
description INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease characterized by chronic fibrosing inflammation with abundant IgG4-positive plasma cells, and responds well to steroids. Previous reports of IgG4-RD have focused on pancreatic and extrapancreatic including the gastrointestinal tract, however, the colonic IgG4-RD is rare. PRESENTATION OF CASE: We herein report the case of a 74-year-old female with edematous wall thickening of the terminal ileum to the lower ascending colon confirmed by several preoperative imaging studies, who underwent right hemi-colectomy for suspected malignant lymphoma. The resected specimen showed an irregular wall thickness with subserosal sclerosis, and the lesion was 10 cm in length from the terminal ileum to the ascending colon. The patient was diagnosed with IgG4-RD by pathological examinations, which demonstrated an increased number of IgG4-positive plasma cells (150/HPF), and an elevated IgG4/IgG ratio (50%). DISCUSSION: Gastrointestinal IgG4-RD appears to be difficult to diagnose prior to surgical resection because of its rarity, and the similarity of its features to malignancy. The measurement of the serum IgG4 levels, immunohistochemical examination of biopsy specimens and use of several imaging modalities might help us to diagnose the disease without surgical resection, and this disease can generally be treated with steroid therapy. However, surgical resection for IgG4-RD may still be also necessary for patients with concerns regarding malignancy or with intractable gastrointestinal obstruction caused by this disease. CONCLUSION: Gastrointestinal IgG4-RD often mimics malignancy, and we should therefore consider this disease in the differential diagnosis of colonic lesions in order to optimize the treatment.
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spelling pubmed-41890762014-10-13 IgG4-related disease of the ileocecal region mimicking malignancy: A case report Hiyoshi, Yukiharu Oki, Eiji Zaitsu, Yoko Ando, Koji Ito, Shuhei Saeki, Hiroshi Morita, Masaru Yamamoto, Hidetaka Baba, Hideo Maehara, Yoshihiko Int J Surg Case Rep Article INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease characterized by chronic fibrosing inflammation with abundant IgG4-positive plasma cells, and responds well to steroids. Previous reports of IgG4-RD have focused on pancreatic and extrapancreatic including the gastrointestinal tract, however, the colonic IgG4-RD is rare. PRESENTATION OF CASE: We herein report the case of a 74-year-old female with edematous wall thickening of the terminal ileum to the lower ascending colon confirmed by several preoperative imaging studies, who underwent right hemi-colectomy for suspected malignant lymphoma. The resected specimen showed an irregular wall thickness with subserosal sclerosis, and the lesion was 10 cm in length from the terminal ileum to the ascending colon. The patient was diagnosed with IgG4-RD by pathological examinations, which demonstrated an increased number of IgG4-positive plasma cells (150/HPF), and an elevated IgG4/IgG ratio (50%). DISCUSSION: Gastrointestinal IgG4-RD appears to be difficult to diagnose prior to surgical resection because of its rarity, and the similarity of its features to malignancy. The measurement of the serum IgG4 levels, immunohistochemical examination of biopsy specimens and use of several imaging modalities might help us to diagnose the disease without surgical resection, and this disease can generally be treated with steroid therapy. However, surgical resection for IgG4-RD may still be also necessary for patients with concerns regarding malignancy or with intractable gastrointestinal obstruction caused by this disease. CONCLUSION: Gastrointestinal IgG4-RD often mimics malignancy, and we should therefore consider this disease in the differential diagnosis of colonic lesions in order to optimize the treatment. Elsevier 2014-08-10 /pmc/articles/PMC4189076/ /pubmed/25194601 http://dx.doi.org/10.1016/j.ijscr.2014.08.003 Text en © 2014 The Authors http://creativecommons.org/licenses/by-nc-nd/3.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/3.0/).
spellingShingle Article
Hiyoshi, Yukiharu
Oki, Eiji
Zaitsu, Yoko
Ando, Koji
Ito, Shuhei
Saeki, Hiroshi
Morita, Masaru
Yamamoto, Hidetaka
Baba, Hideo
Maehara, Yoshihiko
IgG4-related disease of the ileocecal region mimicking malignancy: A case report
title IgG4-related disease of the ileocecal region mimicking malignancy: A case report
title_full IgG4-related disease of the ileocecal region mimicking malignancy: A case report
title_fullStr IgG4-related disease of the ileocecal region mimicking malignancy: A case report
title_full_unstemmed IgG4-related disease of the ileocecal region mimicking malignancy: A case report
title_short IgG4-related disease of the ileocecal region mimicking malignancy: A case report
title_sort igg4-related disease of the ileocecal region mimicking malignancy: a case report
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4189076/
https://www.ncbi.nlm.nih.gov/pubmed/25194601
http://dx.doi.org/10.1016/j.ijscr.2014.08.003
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