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Wan Q, Xu Z, Liu X, Wu Z, Zhong Q, Wu C. A case report of IgG4-related hepatic inflammatory pseudotumor in a 3-year old boy. Front Immunol 2024; 15:1376276. [PMID: 38745658 PMCID: PMC11091244 DOI: 10.3389/fimmu.2024.1376276] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2024] [Accepted: 04/19/2024] [Indexed: 05/16/2024] Open
Abstract
Background Hepatic Inflammatory Pseudotumor (IPT) is an infrequent condition often masquerading as a malignant tumor, resulting in misdiagnosis and unnecessary surgical resection. The emerging concept of IgG4-related diseases (IgG4-RD) has gained widespread recognition, encompassing entities like IgG4-related hepatic IPT. Clinically and radiologically, corticosteroids and immunosuppressive therapies have proven effective in managing this condition. Case Presentation A 3-year-old Chinese boy presented to the clinic with an 11-month history of anemia, fever of unknown origin, and a tender hepatic mass. Blood examinations revealed chronic anemia (Hb: 6.4 g/L, MCV: 68.6 fl, MCH: 19.5 pg, reticulocytes: 1.7%) accompanied by an inflammatory reaction and an elevated serum IgG4 level (1542.2 mg/L). Abdominal contrast-enhanced computed tomography unveiled a 7.6 cm low-density mass in the right lateral lobe, while magnetic resonance imaging demonstrated slight hypointensity on T1-weighted images and slight hyperintensity on T2-weighted images, prompting suspicion of hepatic malignancy. A subsequent liver biopsy revealed a mass characterized by fibrous stroma and dense lymphoplasmacytic infiltration. Immunohistochemical analysis confirmed the presence of IgG4-positive plasma cells, leading to the diagnosis of IgG4-related hepatic IPT. Swift resolution occurred upon initiation of corticosteroid and mycophenolate mofetil therapies. Conclusion This study underscores the diagnostic approach to hepatic IPT, utilizing histopathology, immunostaining, imaging, serology, organ involvement, and therapeutic response. Early histological examination plays a pivotal role in clinical guidance, averting misdiagnosis as a liver tumor and unnecessary surgical interventions.
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Affiliation(s)
- Qian Wan
- Department of Hematology, Jiangxi Provincial Children’s Hospital, Nanchang, China
| | - Zhongjin Xu
- Department of Hematology, Jiangxi Provincial Children’s Hospital, Nanchang, China
| | - Xiaohui Liu
- Department of Rheumatology and Immunology, Jiangxi Provincial Children’s Hospital, Nanchang, China
| | - Zhuqiang Wu
- Nuclear Magnetic Resonance Room, Jiangxi Provincial Children’s Hospital, Nanchang, China
| | - Qingmei Zhong
- Department of Pathology, The Ninth Hospital of Nanchang, Nanchang, China
| | - Chongjun Wu
- Department of Hematology, Jiangxi Provincial Children’s Hospital, Nanchang, China
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Delbrune M, Wongjarupong N, Aby ES, Iwamoto C, Hassan M. Incidental Finding of Hepatic Inflammatory Pseudotumor Immunoglobulin G4-Related Disease With Underlying Chronic Hepatitis C. Cureus 2023; 15:e44066. [PMID: 37750140 PMCID: PMC10517879 DOI: 10.7759/cureus.44066] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/23/2023] [Indexed: 09/27/2023] Open
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a unique immunological disease that can impact multiple organs including a formation of a hepatic inflammatory pseudotumor (IPT). We present a case of a 67-year-old male with a history of chronic viral hepatitis C infection who had an accidental finding on magnetic resonance imaging (MRI) of a liver arterially enhancing lesion. With an extensive work-up, immunohistochemical stains for immunoglobulin G of the liver lesion was performed and showed markedly increased IgG4-positive plasma cells (> 50/HPF), which was consistent with hepatic inflammatory pseudotumor related to IgG4-RD. The patient was treated with prednisone with a complete resolution of the hepatic lesion. The diagnosis of hepatic IPT and IgG4-RD requires a high degree of clinical suspicion and coordination with a multi-disciplinary team, including pathologists. Early tissue acquisition and staining for IgG4 was essential for the early diagnosis and treatment in this case. We also provide a comprehensive summary of published reports of IgG4-RD presenting with IPT.
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Affiliation(s)
- Malique Delbrune
- Gastroenterology, Hepatology and Nutrition, University of Minnesota, Minneapolis, USA
| | - Nicha Wongjarupong
- Gastroenterology, Hepatology and Nutrition, University of Minnesota, Minneapolis, USA
| | - Elizabeth S Aby
- Gastroenterology, Hepatology and Nutrition/ Transplant Hepatology, University of Minnesota, Minneapolis, USA
| | - Carlos Iwamoto
- Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, USA
| | - Mohamed Hassan
- Gastroenterology, Hepatology and Nutrition, University of Minnesota, Minneapolis, USA
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3
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Vahidy AS, Tebha SS, Chavarria Y, KC S, Sharma S. Clinical presentation and organ-based outcomes of Multifocal fibrosclerosis: A systematic review. SAGE Open Med 2023; 11:20503121231178046. [PMID: 37275844 PMCID: PMC10233596 DOI: 10.1177/20503121231178046] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/03/2022] [Accepted: 05/09/2023] [Indexed: 06/07/2023] Open
Abstract
Objectives Multifocal fibrosclerosis is a rare disorder causing progressive fibrosis of multiple organ systems. Existing data on the disease show that there are multiple manifestations of the disease, with different outcomes. However, quantitative data are scarce, prompting the need for our investigation. Method A comprehensive systematic review was performed from inception to November 16, 2022, with the restriction of English language, not including review articles. Article screening and extraction was performed independently, and shortlisted articles were assessed for bias. Analysis was performed using SPSS Version 25 (IBM® SPSS® Statistics; Chicago, IL, USA). Data were presented as frequencies and percentages, with a confidence interval of 95%. Result This review included 134 patients, with 78 (58.2%) males. Mean age was 53.6 years and included two pediatric patients. The most common comorbidity was diabetes (9.7%). Prevalent presenting symptoms included pain (47.8%) and swelling (35.1%). A mean of 2.51 organs or anatomical sites was affected, retroperitoneum (64.2%) being most affected. The pancreas (30.0%) and digestive system (47.0%) were the organs/organ systems most affected. Patients had favorable outcomes in 79.1% of cases, 87.3% had no relapse, and 91.8% of patients survived the condition. Conclusion The findings in this study provide a quantitative measurement of the demographics, presentations, organ manifestations, and outcomes of multifocal fibrosclerosis. We found the disease to be prevalent in males in Japan or the United States, with the abdomen and its organs being commonly involved.
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Affiliation(s)
- Ahmed Safi Vahidy
- Department of Medicine, Dow University
of Health Sciences, Karachi, SD, Pakistan
| | - Sameer Saleem Tebha
- Department of Neurosurgery and
Neurology, Jinnah Medical and Dental College, Karachi, SD, Pakistan
| | - Yeny Chavarria
- Department of Neurology, University of
California, Irvine, CA, USA
| | - Sandip KC
- Department of Medicine, HAMS Hospital,
Kathmandu, BA, Nepal
| | - Shristi Sharma
- Department of Medicine, HAMS Hospital,
Kathmandu, BA, Nepal
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4
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Wang X, El Jabbour T, Chahal D, Odin JA, Crismale J, Taouli B, Thung SN, Ward SC. First Report of Immunoglobulin G4-related Hepatic Inflammatory Pseudotumor in Transplanted Liver. Transplantation 2023; 107:e154-e155. [PMID: 37097981 DOI: 10.1097/tp.0000000000004548] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/26/2023]
Affiliation(s)
- Xintong Wang
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, NY
| | - Tony El Jabbour
- Department of Pathology, West Virginia University, Morgantown, WV
| | - Daljeet Chahal
- Division of Liver Diseases and the Recanati/Miller Transplantation Institute, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY
| | - Joseph A Odin
- Division of Liver Diseases and the Recanati/Miller Transplantation Institute, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY
| | - James Crismale
- Division of Liver Diseases and the Recanati/Miller Transplantation Institute, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY
| | - Bachir Taouli
- Department of Diagnostic, Molecular and Interventional Radiology, Icahn School of Medicine at Mount Sinai, New York, NY
| | - Swan N Thung
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, NY
| | - Stephen C Ward
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, New York, NY
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5
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IgG4-Related Hepatic Pseudotumor Masquerading as a Klatskin Tumor. Case Reports Hepatol 2022; 2022:5765116. [PMID: 35800852 PMCID: PMC9256424 DOI: 10.1155/2022/5765116] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2022] [Accepted: 06/06/2022] [Indexed: 11/30/2022] Open
Abstract
Immunoglobulin G subclass 4 (IgG-4)-related disease (IgG4-RD) is an uncommon immune-mediated, fibro-inflammatory disease which has garnered recognition as a systemic condition. One manifestation of the disease in the hepatobiliary system is the development of hepatic inflammatory pseudotumors. These benign tumors are often misdiagnosed as malignant tumors and undergo unnecessary hepatic resections. We present a case of IgG4-related hepatic inflammatory pseudotumor (IPT) mimicking a Klatskin tumor. A high degree of clinical suspicion and extensive workup is imperative in reaching the correct diagnosis. IgG4-related inflammatory pseudotumor is a rare entity, but an important consideration in evaluating hepatic tumors.
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6
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IgG4-related pseudotumours: a series of 12 cases and a review of the literature. Pathology 2022; 54:563-572. [DOI: 10.1016/j.pathol.2021.11.015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2021] [Revised: 11/11/2021] [Accepted: 11/18/2021] [Indexed: 11/22/2022]
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7
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Kanagalingam G, Dulymamode KN, Jafroodifar A, Huda SA, May A, Masood U, John S. Enlarging Liver Mass: Inflammatory Pseudotumor in a Patient With Polymyalgia Rheumatica. J Investig Med High Impact Case Rep 2022; 10:23247096211070387. [PMID: 35038943 PMCID: PMC8961211 DOI: 10.1177/23247096211070387] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022] Open
Abstract
Inflammatory pseudotumors of the liver are rare, non-neoplastic liver tumors. Due to the
nonspecific clinical presentation, imaging features, and histopathological findings, they
can mimic malignant tumors requiring invasive diagnostics. We present a case of a
61-year-old female patient with a history of type 2 diabetes mellitus, hypothyroidism,
hyperlipidemia, and polymyalgia rheumatica who had initially presented with abdominal pain
for 3 weeks. Further workup showed normal liver chemistries and tumor markers: AFP and CA
19-9. Magnetic resonance imaging (MRI) of the abdomen showed a segment 6 lesion measuring
4.1 × 4.0 × 3.7 cm. A liver biopsy then confirmed the diagnosis of an inflammatory
pseudotumor of the liver with negative IgG4. On follow-up imaging, a rapid growth of this
liver lesion was noted. Laparoscopy was done but did not show any distinct liver lesion.
Follow-up imaging confirmed a decrease in the size of the mass. Interestingly, the patient
had been on a higher dose of steroids for her polymyalgia rheumatic leading up to the
follow-up imaging. This is the first case of an inflammatory pseudotumor of the liver in a
patient with polymyalgia rheumatica. With this case, we would like to increase the
awareness for inflammatory pseudotumors of the liver as a differential diagnosis of liver
lesions in patients with underlying autoimmune disorders.
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Affiliation(s)
| | | | - Abtin Jafroodifar
- Department of Radiology, SUNY Upstate Medical University, Syracuse, NY, USA
| | - Syed A Huda
- Department of Internal Medicine, SUNY Upstate Medical University, Syracuse, NY, USA
| | - Adriana May
- Department of Pathology, SUNY Upstate Medical University, Syracuse, NY, USA
| | - Umair Masood
- Department of Gastroenterology, SUNY Upstate Medical University, Syracuse, NY, USA
| | - Savio John
- Department of Gastroenterology, SUNY Upstate Medical University, Syracuse, NY, USA
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8
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Kamisawa T, Nakazawa T, Tazuma S, Zen Y, Tanaka A, Ohara H, Muraki T, Inui K, Inoue D, Nishino T, Naitoh I, Itoi T, Notohara K, Kanno A, Kubota K, Hirano K, Isayama H, Shimizu K, Tsuyuguchi T, Shimosegawa T, Kawa S, Chiba T, Okazaki K, Takikawa H, Kimura W, Unno M, Yoshida M. Clinical practice guidelines for IgG4-related sclerosing cholangitis. JOURNAL OF HEPATO-BILIARY-PANCREATIC SCIENCES 2019; 26:9-42. [PMID: 30575336 PMCID: PMC6590186 DOI: 10.1002/jhbp.596] [Citation(s) in RCA: 94] [Impact Index Per Article: 15.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
IgG4‐related sclerosing cholangitis (IgG4‐SC) is a distinct type of cholangitis frequently associated with autoimmune pancreatitis and currently recognized as a biliary manifestation of IgG4‐related disease. Although clinical diagnostic criteria of IgG4‐SC were established in 2012, differential diagnosis from primary sclerosing cholangitis and cholangiocarcinoma is sometimes difficult. Furthermore, no practical guidelines for IgG4‐SC are available. Because the evidence level of most articles retrieved through searching the PubMed, Cochrane Library, and Igaku Chuo Zasshi databases was below C based on the systematic review evaluation system of clinical practice guidelines MINDS 2014, we developed consensus guidelines using the modified Delphi approach. Three committees (a guideline creating committee, an expert panelist committee for rating statements according to the modified Delphi method, and an evaluating committee) were organized. Eighteen clinical questions (CQs) with clinical statements were developed regarding diagnosis (14 CQs) and treatment (4 CQs). Recommendation levels for clinical statements were set using the modified Delphi approach. The guidelines explain methods for accurate diagnosis, and safe and appropriate treatment of IgG4‐SC.
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Affiliation(s)
- Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan, Komagome Hospital, Tokyo, Japan
| | - Takahiro Nakazawa
- Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan
| | - Susumu Tazuma
- Department of General Internal Medicine, Hiroshima University Graduate School of Biomedical & Health Science, Hiroshima, Japan
| | - Yoh Zen
- Department of Diagnostic Pathology, Kobe University, Kobe, Japan
| | - Atsushi Tanaka
- Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
| | - Hirotaka Ohara
- Department of Community-Based Medical Education, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Takashi Muraki
- Department of Medicine, Gastroenterology, Shinshu University, Matsumoto, Nagano, Japan
| | - Kazuo Inui
- Department of Gastroenterology, Second Teaching Hospital, Fujita Health University, Nagoya, Japan
| | - Dai Inoue
- Department of Radiology, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan
| | - Takayoshi Nishino
- Department of Gastroenterology, Tokyo Womens' Medical University Yachiyo Medical Center, Yachiyo, Japan
| | - Itaru Naitoh
- Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Takao Itoi
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Tokyo, Japan
| | - Kenji Notohara
- Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan
| | - Atsushi Kanno
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan
| | - Kensuke Kubota
- Department of Endoscopy, Yokohama City University Hospital, Yokohama, Japan
| | - Kenji Hirano
- Department of Gastroenterology, Tokyo Takanawa Hospital, Tokyo, Japan
| | - Hiroyuki Isayama
- Department of Gastroenterology, Graduate School of Medicine, Juntendo University, Tokyo, Japan
| | - Kyoko Shimizu
- Department of Gastroenterology, Tokyo Womens' Medical University, Tokyo, Japan
| | | | - Tooru Shimosegawa
- Division of Gastroenterology, South-Miyagi Medical Center, Ohgawara, Japan
| | - Shigeyuki Kawa
- Department of Internal Medicine, Matsumoto Dental University, Matsumoto, Japan
| | | | - Kazuichi Okazaki
- The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Moriguchi, Japan
| | - Hajime Takikawa
- Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
| | - Wataru Kimura
- Faculty of Medicine, Departments of Gastroenterology and Gastroenterological, General, Breast, and Thyroid Surgery, Yamagata University, Yamagata, Japan
| | - Michiaki Unno
- Division of Hepato-Biliary Pancreatic Surgery, Tohoku University Graduate School, of Medicine, Sendai, Japan
| | - Masahiro Yoshida
- Department of Hepato-Biliary-Pancreatic and Gastrointestinal Surgery, School of Medicine, International University of Health and Welfare, Ichikawa, Japan
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9
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Cheong HR, Lee BE, Song GA, Kim GH, An SG, Lim W. Immunoglobulin G4-Related Inflammatory Pseudotumor Presenting as a Solitary Mass in the Stomach. Clin Endosc 2016; 49:197-201. [PMID: 26867551 PMCID: PMC4821516 DOI: 10.5946/ce.2015.074] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/04/2015] [Revised: 06/26/2015] [Accepted: 07/11/2015] [Indexed: 12/24/2022] Open
Abstract
Immunoglobulin G4 (IgG4)-related disease (IgG4RD) is a relatively recently recognized entity that is histopathologically characterized by an extensive infiltration of lymphocytes and IgG4-positive plasma cells with dense fibrosis. IgG4RD is now known to affect any organ system, and a few cases of gastrointestinal lesions have also been reported. However, solitary IgG4RD of the stomach is still very rare. Furthermore, as it can mimic malignant conditions, it is important to recognize this disease to avoid unnecessary surgery. Herein, we present a case of IgG4RD presenting as an isolated subepithelial mass in the stomach.
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Affiliation(s)
- Hong Ryeol Cheong
- Department of Internal Medicine, Pusan National University School of Medicine, Busan, Korea
| | - Bong Eun Lee
- Department of Internal Medicine, Pusan National University School of Medicine, Busan, Korea
| | - Geun Am Song
- Department of Internal Medicine, Pusan National University School of Medicine, Busan, Korea
| | - Gwang Ha Kim
- Department of Internal Medicine, Pusan National University School of Medicine, Busan, Korea
| | - Sung Gyu An
- Department of Internal Medicine, Pusan National University School of Medicine, Busan, Korea
| | - Won Lim
- Department of Internal Medicine, Pusan National University School of Medicine, Busan, Korea
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10
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Affiliation(s)
- Miyu Tajima
- Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo
- Department of Cardiology, Sakakibara Heart Institute
| | | | - Yukio Hiroi
- Department of Cardiology, National Center for Global Health and Medicine
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11
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Mid common bile duct inflammatory pseudotumor mimicking cholangiocarcinoma. A case report and literature review. Int J Surg Case Rep 2013; 5:12-5. [PMID: 24394855 PMCID: PMC3907201 DOI: 10.1016/j.ijscr.2013.10.017] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/20/2013] [Revised: 10/29/2013] [Accepted: 10/29/2013] [Indexed: 01/03/2023] Open
Abstract
INTRODUCTION Biliary inflammatory pseudotumors (IPTs) represent an exceptional benign cause of obstructive jaundice. These lesions are often mistaken for cholangiocarcinomas and are treated with major resections, because their final diagnosis can be achieved only after formal pathological examination of the resected specimen. Consequently, biliary IPTs are usually managed with unnecessary major resections. PRESENTATION OF CASE A 71-year-old female patient underwent an extra-hepatic bile duct resection en-bloc with the gallbladder and regional lymph nodes for an obstructing intraluminal growing tumor of the mid common bile duct (CBD). Limited resection was decided intraoperatively because of negative for malignancy fast frozen sections analysis in addition to the benign macroscopic features of the lesion. Histologically the tumor proved an IPT, arising from the bile duct epithelium, composed of inflammatory cells and reactive mesenchymal tissues. DISCUSSION The present case underlines the value of intraoperative reassessment of patients undergoing surgical resection for histopathologically undiagnosed biliary occupying lesions, in order to optimize their surgical management. CONCLUSION The probability of benign lesions mimicking cholangiocarcinoma should always be considered to avoid unnecessary major surgical resections, especially in fragile and/or elderly patients.
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12
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Abstract
OBJECTIVE IgG4-related disease was not recognized as a specific clinical entity until 2003 when extrapancreatic lesions were reported in patients with autoimmune pancreatitis. IgG4-related disease is characterized by elevated serum IgG4 levels and infiltration of the target organ by IgG4-positive plasma cells. The complete gamut of visceral involvement is still being outlined. The purpose of this article is to highlight the plethora of lesions under the spectrum of IgG4-related disease of the abdomen and pelvis, describe their imaging appearances on multimodality cross-sectional imaging, and discuss the differential diagnoses. CONCLUSION It is important for radiologists to recognize the multiorgan involvement and few classic features of IgG4-related disease that often tend to simulate malignancy.
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13
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Horiguchi S, Ikeda F, Shiraha H, Yamamoto N, Sakakihara I, Noma Y, Tsutsumi K, Kato H, Hagihara H, Yasunaka T, Nakamura S, Kobashi H, Kawamoto H, Yamamoto K. Diagnostic usefulness of precise examinations with intraductal ultrasonography, peroral cholangioscopy and laparoscopy of immunoglobulin G4-related sclerosing cholangitis. Dig Endosc 2012; 24:370-3. [PMID: 22925292 DOI: 10.1111/j.1443-1661.2012.01300.x] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Herein, a case of immunoglobulin G4 (IgG4)-related sclerosing cholangitis is reported. IgG4 was diagnosed based on observations from peroral cholangioscopy and laparoscopy, and these methods are proposed for definitive and precise diagnosis of this disease. A 76-year-old male patient with inguinal Paget's disease had intrahepatic bile duct dilatations detected with computed tomography at his periodic check-up. Magnetic resonance cholangiography showed stenosis of the upper common bile duct and poststenotic dilatation of left intrahepatic bile ducts. The portal tract and bilateral intrahepatic bile ducts were surrounded by a low-density area, facing a tumor-like lesion at segment 2. Cytological examinations of the stenotic and dilated lesions revealed no cellular atypia. Histological examination of the tumor showed normal liver tissue with infiltration of lymphocytes, indicating an inflammatory pseudotumor. Peroral cholangioscopy excluded the possibility of biliary cancer and indicated that the stenotic legion was of submucosal, not mucosal, origin. Laparoscopic observations showed discoloration with wide yellowish-white lobular markings and wide depressed lesions at segments 2 and 7. Liver histology showed mild cholangitis with infiltration of IgG4-positive plasma cells around the bile ducts. Serum IgG4 levels were elevated. From these findings, the patient was diagnosed with IgG4-related sclerosing cholangitis. After treatment with prednisolone, blood liver enzymes and IgG4 rapidly normalized, bile duct dilatations improved, and the hepatic pseudotumor disappeared. The cholangitis did not recur. In this case, biliary cancer was ruled out by observation with peroral cholangioscopy, and the spread of cholangitis in the liver periphery was verified with laparoscopy; this information could not be obtained with other modalities.
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Affiliation(s)
- Shigeru Horiguchi
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
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14
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Kim DH, Kim J, Park DH, Lee JH, Choi KD, Lee GH, Jung HY, Kim JH. Immunoglobulin G4-related inflammatory pseudotumor of the stomach. Gastrointest Endosc 2012; 76:451-2. [PMID: 21981816 DOI: 10.1016/j.gie.2011.07.061] [Citation(s) in RCA: 28] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/02/2011] [Accepted: 07/27/2011] [Indexed: 02/08/2023]
Affiliation(s)
- Do Hoon Kim
- Department of Gastroenterology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea
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15
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Abu-Wasel B, Eltawil KM, Molinari M. Benign inflammatory pseudotumour mimicking extrahepatic bile duct cholangiocarcinoma in an adult man presenting with painless obstructive jaundice. BMJ Case Rep 2012; 2012:006514. [PMID: 22739336 DOI: 10.1136/bcr-2012-006514] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022] Open
Abstract
Inflammatory pseudotumours (IPTs) of the biliary tract are extremely rare and heterogeneous by aetiology and clinical presentation. They might cause biliary obstruction and mimic cholangiocarcinomas and their final diagnosis is usually achieved only after surgical excision. The most characteristic feature of IPT is the presence of chronic inflammatory cell infiltrates with variable degree of proliferating fibrous tissue. IPTs have the potential for recurrence even after resection and if untreated they can grow causing a variety of symptoms due to compression of the surrounding structures and organs. Despite the significant improvement of modern imaging techniques, preoperative distinction between IPTs of the biliary system and malignancies is extremely difficult. Histological diagnosis poses a clinical challenge because sampling is often suboptimal. Although rare, IPTs should be in the differential diagnosis of patients who present with painless jaundice and no other clinical symptoms or signs characteristics of cholangiocarcinomas.
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Affiliation(s)
- Bassam Abu-Wasel
- Department of Surgery, Dalhousie University, Halifax, Nova Scotia, Canada
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Abstract
Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recently defined emerging entity characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis. IgG4-RSD usually affects middle aged and elderly patients, with a male predominance. It is associated with an elevated serum titer of IgG4, which acts as a marker for this recently characterized entity. The prototype is IgG4-related sclerosing pancreatitis or autoimmune pancreatitis (AIP). Other common sites of involvement are the hepatobiliary tract, salivary gland, orbit, and lymph node, however practically any organ can be involved, including upper aerodigestive tract, lung, aorta, mediastinum, retroperitoneum, soft tissue, skin, central nervous system, breast, kidney, and prostate. Fever or constitutional symptoms usually do not comprise part of the clinical picture. Laboratory findings detected include raised serum globulin, IgG and IgG4. An association with autoantibody detection (such as antinuclear antibodies and rheumatoid factor) is seen in some cases. Steroid therapy comprises the mainstay of treatment. Disease progression with involvement of multiple organ-sites may be encountered in a subset of cases and may follow a relapsing-remitting course. The principal histopathologic findings in several extranodal sites include lymphoplasmacytic infiltration, lymphoid follicle formation, sclerosis and obliterative phlebitis, along with atrophy and destruction of tissues. Immunohistochemical staining shows increased IgG4+ cells in the involved tissues (>50 per high-power field, with IgG4/IgG ratio >40%). IgG4-RSD may potentially be rarely associated with the development of lymphoma and carcinoma. However, the nature and pathogenesis of IgG4-RSD are yet to be fully elucidated and provide immense scope for further studies.
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Affiliation(s)
- Mukul Divatia
- Department of Pathology, The Methodist Hospital, Weill Medical College of Cornell University, Houston, TX, USA
| | - Sun A Kim
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Jae Y. Ro
- Department of Pathology, The Methodist Hospital, Weill Medical College of Cornell University, Houston, TX, USA
- Department of Pathology, Yonsei University College of Medicine, Seoul, Korea
- National Cancer Center, Goyang, Korea
- The University of Texas, MD Anderson Cancer Center, Houston, TX, USA
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Li SY, Huang XY, Chen YT, Liu Y, Zhao S. Autoimmune pancreatitis characterized by predominant CD8+ T lymphocyte infiltration. World J Gastroenterol 2011; 17:4635-9. [PMID: 22147972 PMCID: PMC3225101 DOI: 10.3748/wjg.v17.i41.4635] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/22/2011] [Revised: 06/02/2011] [Accepted: 06/09/2011] [Indexed: 02/06/2023] Open
Abstract
Autoimmune pancreatitis (AIP) is a rare form of pancreatitis characterized by prominent lymphocyte infiltration and pancreatic fibrosis resulting in organ dysfunction. The pathogenesis and pathology of AIP remain unknown. A 64-year-old Chinese man presented with symptoms and signs of bile duct obstruction diffuse enlargement of the head of pancreas, elevated IgG levels, and negative autoimmune antibody responses. A pylorus-preserving pancreatoduodenectomy was performed and a pancreatic tumor was suspected. However, periductal lymphoplasmacytic infiltration and fibrosis were found in the head of pancreas and nearby organs instead of tumor cells. Four months after surgery, the patient was readmitted because of reoccurrence of severe jaundice and sustained abdominal distension. Prednisone 30 mg/d was administered orally as an AIP was suspected. One and a half months later, the symptoms of the patient disappeared, and globulin, aminotransferase and bilirubin levels decreased significantly. Over a 9-mo follow-up period, the dose of prednisone was gradually decreased to 10 mg/d and the patient remained in good condition. We further demonstrated dominant CD3+/CD8+ populations, CD20+ cells and a few CD4+ cells in the pancreatic parenchyma, duodenum and gallbladder wall by immunohistochemical assay. This AIP case presented with significant CD8+ T lymphocyte infiltration in the pancreas and extra-pancreatic lesions, indicating that this cell population may be more important in mediating AIP pathogenesis than previously known and that AIP might be a poorly defined autoimmune disease with heterogeneous pathogenesis.
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Lindstrom KM, Cousar JB, Lopes MBS. IgG4-related meningeal disease: clinico-pathological features and proposal for diagnostic criteria. Acta Neuropathol 2010; 120:765-76. [PMID: 20844883 DOI: 10.1007/s00401-010-0746-2] [Citation(s) in RCA: 132] [Impact Index Per Article: 8.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/25/2010] [Revised: 09/03/2010] [Accepted: 09/04/2010] [Indexed: 12/16/2022]
Abstract
IgG4-related disease has evolved from originally being recognized as a form of pancreatitis to encompass diseases of numerous organs including the hypophysis and one reported case of dural involvement. A search of the University of Virginia, Division of Neuropathology files for 10 years identified ten cases of unexplained lymphoplasmacytic meningeal inflammation that we then evaluated using immunohistochemical stains for IgG4 and IgG. Ten control cases including sarcoidosis (4), tuberculosis (1), bacterial abscess (2), Langerhans cell histiocytosis (2), and foreign body reaction (1) were also examined. The number of IgG4-positive plasma cells was counted in five high power fields (HPFs) and an average per HPF was calculated. Cases that contained greater than ten IgG4-positive cells/HPF were considered to be IgG4-related. Five of the study cases met these criteria, including one case of leptomeningeal inflammation. All cases exhibited the typical histological features of IgG4-related disease including lymphoplasmacytic inflammation, fibrosis, and phlebitis. The dural-based lesions appear to represent a subset of the cases historically diagnosed as idiopathic hypertrophic pachymeningitis. While the leptomeningeal process most closely resembles non-vasculitic autoimmune inflammatory meningoencephalitis. Given these findings, IgG4-related meningitis should be considered in the differential diagnosis of meningeal inflammatory lesions after stringent clinical and histologic criteria are used to rule out other possible diagnoses.
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Uehara T, Hamano H, Kawa S, Sano K, Oki K, Kobayashi Y, Nagaya T, Akamatsu T, Kurozumi M, Fujinaga Y, Tanaka E, Honda T, Ota H. Chronic gastritis in the setting of autoimmune pancreatitis. Am J Surg Pathol 2010; 34:1241-9. [PMID: 20697253 DOI: 10.1097/pas.0b013e3181ec07ee] [Citation(s) in RCA: 35] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
Autoimmune pancreatitis (AIP) is a recently recognized disease entity. In some patients, this disease is associated with other inflammatory diseases. In this study, we aimed to elucidate the pathologic characteristics of AIP-associated gastritis (AIP-G). We evaluated and compared the pathologic findings and immunohistochemical expressions of immunoglobulin G (IgG)4 and IgG in gastric biopsy specimens from 13 AIP-G patients with those from patients of 2 control groups. We divided the AIP-G patients who did not receive steroid therapy [AIP-G-ST(-)] into the following 2 groups: without Helicobacter pylori (HP) infection [AIP-G-HP(-)] and with HP infection [AIP-G-HP(+)]. The control groups comprised 19 patients who were diagnosed with chronic active gastritis associated with HP infection and 7 patients with nonsteroidal anti-inflammatory drug-induced gastritis. We classified the findings for the gastric mucosa into those for the upper and the lower lamina propria. The characteristic finding of AIP-G groups was diffusely lymphoplasmacytic infiltration in the lamina propria. The IgG4-positive plasma cell/IgG-positive plasma cell ratios (IgG4/IgG ratios) in both the upper and lower lamina propria in the AIP-G-ST(-) groups were predominantly higher than the corresponding values in the other groups. In the AIP-G-ST(-) groups, the IgG4/IgG ratio in the lower lamina propria was predominantly higher than that in the upper lamina propria, irrespective of the HP status. In conclusion, diffuse lymphoplasmacytic infiltration in the lamina propria and increased IgG4/IgG ratio in the gastric mucosa (notably in the lower lamina propria) may be the characteristic findings of AIP-G.
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Affiliation(s)
- Takeshi Uehara
- Department of Laboratory Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
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IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol 2010; 17:303-32. [PMID: 20733352 DOI: 10.1097/pap.0b013e3181ee63ce] [Citation(s) in RCA: 291] [Impact Index Per Article: 19.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
An elevated serum titer of immunoglobulin G4 (IgG4), the least common (3% to 6%) of the 4 subclasses of IgG, is a surrogate marker for the recently characterized IgG4-related sclerosing disease. The syndrome affects predominantly middle-aged and elderly patients, with male predominance. The patients present with symptoms referable to the involvement of 1 or more sites, usually in the form of mass lesions. The prototype is IgG4-related sclerosing pancreatitis (also known as autoimmune pancreatitis), most commonly presenting as painless obstructive jaundice with or without a pancreatic mass. Other common sites of involvement are the hepatobiliary tract, salivary gland, orbit, and lymph node, but practically any organ-site can be affected, such as retroperitoneum, aorta, mediastinum, soft tissue, skin, central nervous system, breast, kidney, prostate, upper aerodigestive tract, and lung. The patients usually have a good general condition, with no fever or constitutional symptoms. Common laboratory findings include raised serum globulin, IgG, IgG4, and IgE, whereas lactate dehydrogenase is usually not raised. Some patients have low titers of autoantibodies (such as antinuclear antibodies and rheumatoid factor). The disease often shows excellent response to steroid therapy. The natural history is characterized by the development of multiple sites of involvement with time, sometimes after many years. However, the disease can remain localized to 1 site in occasional patients. The main pathologic findings in various extranodal sites include lymphoplasmacytic infiltration, lymphoid follicle formation, sclerosis and obliterative phlebitis, accompanied by atrophy and loss of the specialized structures of the involved tissue (such as secretory acini in pancreas, salivary gland, or lacrimal gland). The relative predominance of the lymphoplasmacytic and sclerotic components results in 3 histologic patterns: pseudolymphomatous, mixed, and sclerosing. Immunostaining shows increased IgG4+ cells in the involved tissues (>50 per high-power field, with IgG4/IgG ratio >40%). The lymph nodes show multicentric Castleman disease-like features, reactive follicular hyperplasia, interfollicular expansion, or progressive transformation of germinal centers, with the unifying feature being an increase in IgG4+ plasma cells on immunostaining. The nature and pathogenesis of IgG4-related sclerosing disease are still elusive. Occasionally, the disease can be complicated by the development of malignant lymphoma and possibly carcinoma.
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21
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Pitman MB. Liver. Diagn Cytopathol 2010. [DOI: 10.1016/b978-0-7020-3154-0.00008-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
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Kamisawa T, Takuma K, Anjiki H, Egawa N, Fujiwara J, Koizumi K, Sasaki T. 5. Extra-pancreatic Lesions and IgG4-related Sclerosing Disease. ACTA ACUST UNITED AC 2010; 99:97-101. [DOI: 10.2169/naika.99.97] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
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Matsubayashi H, Furukawa H, Maeda A, Matsunaga K, Kanemoto H, Uesaka K, Fukutomi A, Ono H. Usefulness of positron emission tomography in the evaluation of distribution and activity of systemic lesions associated with autoimmune pancreatitis. Pancreatology 2009; 9:694-9. [PMID: 19684434 DOI: 10.1159/000199439] [Citation(s) in RCA: 61] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/30/2008] [Accepted: 01/23/2009] [Indexed: 12/11/2022]
Abstract
BACKGROUND/AIMS Autoimmune pancreatitis (AIP) is an IgG4-related systemic disease often accompanied with a variety of lesions outside of the pancreas and is treated with steroid therapy. The aim of this study is to analyze the usefulness of positron emission tomography with (18)F-fluorodeoxyglucose (FDG-PET) in the evaluation of distribution and activity of systemic lesions of AIP during steroid therapy. METHODS Eleven cases of AIP had their FDG-PET images evaluated before and 3 months after steroid therapy and another 2 cases only before therapy. AIP activity was determined by the level of serum markers, IgG and IgG4, and compared with findings of PET. RESULTS In all 13 cases of AIP, a moderate to intense level of FDG accumulation was recognized in the pancreatic lesion before steroid therapy. Of 13 patients, 11 (84.6%) showed FDG accumulation in the multiple organs, such as mediastinal and other lymph nodes, salivary gland, biliary tract, prostate, and aortic wall. In 11 patients who underwent PET before and after steroid therapy, FDG accumulation was diminished in almost all systemic lesions, with a mean of maximum standardized uptake value (SUV(max)) in the pancreatic lesion from 5.12 to 2.69. Similar to the SUV level, serum IgG and IgG4 were decreased in most of the cases after steroid therapy. CONCLUSIONS FDG-PET is an effective modality to evaluate the response of steroid therapy and the distribution and activity of various systemic lesions of AIP.
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Shimosegawa T, Kanno A. Autoimmune pancreatitis in Japan: overview and perspective. J Gastroenterol 2009; 44:503-17. [PMID: 19377842 DOI: 10.1007/s00535-009-0054-6] [Citation(s) in RCA: 56] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2009] [Accepted: 02/26/2009] [Indexed: 02/06/2023]
Abstract
Since the rediscovery and definition of autoimmune pancreatitis (AIP) by Yoshida et al. in 1995, the disease has been attracting attention because of its unique clinical features and practical issues. This disease shows very impressive imaging findings, serological changes, and characteristic histopathology. It occurs most commonly in elderly males with painless jaundice or mild abdominal pain; resemblance in imaging findings between AIP and pancreatobiliary cancers poses an important practical issue of differentiation. With increasing recognition of AIP and accumulation of cases, another important feature of this disease has been revealed, i.e., association of extrapancreatic organ involvements. Initially misunderstood because it can be accompanied by other autoimmune disorders, such as Sjögren's syndrome or primary sclerosing cholangitis (PSC), AIP is now known to be associated with unique types of sialadenitis and cholangitis distinct from Sjögren's syndrome or PSC. Now the concept of "IgG4-related sclerosing disease" has become widely accepted and the list of organs involved continues to increase. With worldwide recognition, an emerging issue is the clinical definition of other possible types of autoimmune-related pancreatitis called "idiopathic duct-centric chronic pancreatitis (IDCP)" and "AIP with granulocyte epithelial lesion (GEL)" and their relation to AIP with lymphoplasmacytic sclerosing pancreatitis (LPSP). The time has arrived to establish clinical diagnostic criteria of AIP based on international consensus and to discuss regional and racial differences in the clinicopathological features of AIP. Consensus guidelines are also required for the ideal use of steroids in the treatment of AIP to suppress recurrence efficiently with minimal side effects. There are many issues to be settled in AIP; international collaboration of experts in the pancreas field is necessary to clarify the entire picture of this unique and important disease.
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Affiliation(s)
- Tooru Shimosegawa
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Japan.
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Possible association between IgG4-associated systemic disease with or without autoimmune pancreatitis and non-Hodgkin lymphoma. Pancreas 2009; 38:523-6. [PMID: 19258916 DOI: 10.1097/mpa.0b013e31819d73ca] [Citation(s) in RCA: 91] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
OBJECTIVES IgG4-associated systemic disease (ISD) is a multiorgan fibroinflammatory disorder whose pancreatic manifestation is called autoimmune pancreatitis (AIP). We describe 3 patients who developed non-Hodgkin lymphoma during the follow-up of ISD. METHODS At our institution's pancreas clinic, we have prospectively and retrospectively examined patients with ISD with (n = 101) or without (n = 10) AIP (mean age, 59 years; 90 males and 21 females). We reviewed the medical records of all 111 patients to identify patients who developed non-Hodgkin lymphoma during the follow-up since their first presentation of ISD. Standardized incidence rate was calculated. RESULTS The 111 patients with ISD with or without AIP had 331 patient-years of observation during which 3 patients had a diagnosis of non-Hodgkin lymphoma 3 to 5 years after the diagnosis of ISD. In these patients who later developed lymphoma, ISD involved the pancreas (AIP) in 2 and salivary gland in 1. Non-Hodgkin lymphoma had extranodal involvement in all patients (liver [n = 2], adrenal glands [n=1], kidney [n= 1], and lung [n = 1]). Standardized incidence rate was 16.0 (95% confidence interval, 3.3-45.5). CONCLUSIONS We report 3 cases of non-Hodgkin lymphoma that developed during the follow-up of ISD suggesting that patients with ISD may be at an increased risk of developing non-Hodgkin lymphoma.
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IgG4-related hepatic inflammatory pseudotumor with sclerosing cholangitis: a case report and review of the literature. CASES JOURNAL 2009; 2:7029. [PMID: 19829899 PMCID: PMC2740269 DOI: 10.4076/1757-1626-2-7029] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 10/21/2008] [Accepted: 04/02/2009] [Indexed: 12/24/2022]
Abstract
Introduction Inflammatory pseudotumor is rare benign mass composed of chronic inflammatory cell infiltration and proliferating fibrous tissue. Some cases of inflammatory pseudotumor show abundant infiltrating IgG4-positive plasma cells and obliterative phlebitis, which are the pathologic hallmarks of autoimmune pancreatitis. Case presentation A 77-year-old Japanese man was admitted to our hospital because of epigastric pain. A solitary mass with delayed enhancement by dynamic computed tomography was present in the left hepatic lobe. Endoscopic retrograde cholangiography showed only segmental stenosis of the left intrahepatic bile duct. No abnormal findings were detected in the pancreas. The patient was clinically diagnosed as having intrahepatic cholangiocarcinoma and underwent surgery. Histological examination of the hepatic mass and bile duct wall showed abundant IgG4-positive plasma cell infiltration with obliterative phlebitis. The final diagnosis was IgG4-related hepatic inflammatory pseudotumor with sclerosing cholangitis. Delayed enhancement by computed tomography is a characteristic feature of IgG4-related inflammatory pseudotumor similar to that of autoimmune pancreatitis. Conclusion IgG4-related hepatic inflammatory pseudotumor unassociated with autoimmune pancreatitis should be one of the entities considered for differential diagnosis of liver tumors. Delayed enhancement on computed tomography might be useful finding for diagnosing IgG4-related hepatic inflammatory pseudotumor.
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Morselli-Labate AM, Pezzilli R. Usefulness of serum IgG4 in the diagnosis and follow up of autoimmune pancreatitis: A systematic literature review and meta-analysis. J Gastroenterol Hepatol 2009; 24:15-36. [PMID: 19067780 DOI: 10.1111/j.1440-1746.2008.05676.x] [Citation(s) in RCA: 92] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
High circulating serum immunoglobulin G4 (IgG4) levels have been proposed as a marker of autoimmune pancreatitis (AIP). The aim of the present study was to review the data existing in the English literature on the usefulness of the IgG4 serum levels in the diagnosis and follow up of patients with AIP. A total of 159 patients with AIP and 1099 controls were described in seven selected papers reporting the usefulness of serum IgG4 in diagnosing AIP. In total, 304 controls had pancreatic cancer, 96 had autoimmune diseases, and the remaining 699 had other conditions. The summary receiver-operating characteristic curve analysis was carried out by means of Meta-DiSc open-access software. Serum IgG4 showed good accuracy in distinguishing between AIP and the overall controls, pancreatic cancer and other autoimmune diseases (area under the curve [+/- SE]: 0.920 +/- 0.073, 0.914 +/- 0.191, and 0.949 +/- 0.024, respectively). The studies analyzed showed significantly heterogeneous specificity values in each of the three analyses performed. The analysis of the four studies comparing AIP and pancreatic cancers also showed significantly heterogeneous values of sensitivities and odds ratios. Regarding the usefulness of IgG4 as a marker of efficacy of steroid treatment, a decrease in the serum concentrations of IgG4 was found in the four available studies. The serum IgG4 subclass is a good marker of AIP, and its determination should be included in the diagnostic workup of this disease. However, the heterogeneity of the studies published until now means that more studies are necessary in order to better evaluate the true accuracy of IgG4 in discriminating AIP versus other autoimmune diseases.
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Abstract
We report a case of autoimmune pancreatitis involving the colonic mucosa. Although serum level of IgG4 was normal, computed tomography and endoscopic retrograde cholangiopancreatography showed diffuse enlargement of the pancreas and irregular narrowing of the pancreatic ducts, respectively. Colonoscopy revealed a polypoidal lesion in the ascending colon. A lymphoplasmacytic infiltration was seen both in the pancreas and in the polypoidal lesion of the colon. Furthermore, immunohistochemical analysis showed abundant IgG4-positive plasma cells in these lesions. This is the first case report of a simultaneous occurrence of autoimmune pancreatitis and a colonic polypoidal lesion, both of which are characterized with increased IgG4 responses.
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Abstract
Based on histological and immunohistochemical examination of various organs of patients with autoimmune pancreatitis (AIP), a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AIP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4-related sclerosing diseases. This disease includes AIP, sclerosing cholangitis, cholecystitis, sialadenitis, retroperitoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AIP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunostaining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.
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Kobayashi G, Fujita N, Noda Y, Ito K, Horaguchi J. Autoimmune pancreatitis: with special reference to a localized variant. J Med Ultrason (2001) 2008; 35:41-50. [PMID: 27278690 DOI: 10.1007/s10396-008-0177-z] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/26/2007] [Accepted: 03/07/2008] [Indexed: 12/24/2022]
Abstract
In 2006, the Japan Pancreas Society revised the diagnostic criteria for autoimmune pancreatitis (AIP) so as to more clearly define its morphological, pathological, and immunological features, as follows: (1) diffuse or segmental narrowing of the main pancreatic duct with an irregular wall and diffuse or localized enlargement of the pancreas recognized by imaging studies; (2) high serum gamma globulin, IgG, or IgG4 levels, or the presence of autoantibodies; and (3) marked interlobular fibrosis and prominent infiltration of lymphocytes and plasma cells in the periductal area, occasionally with lymphoid follicles in the pancreas. Establishing a diagnosis of AIP has become easier with knowledge of its immunological abnormalities, including serum IgG4 levels. However, the localized form of AIP sometimes mimics pancreatic cancer. The rate of focal mass formation in patients with AIP is reportedly 24%-43%; however, there have been few reports on the histological findings of localized AIP, in contrast to mass-forming pancreatitis (MFP). Our review of patients who had undergone resection due to a preoperative diagnosis of MFP with possible cancer revealed 72% to be patients with localized AIP. For the discrimination of these conditions, it is important to recognize the characteristic ultrasonographic findings of AIP, i.e., (1) diffuse or localized enlargement and hypoechogenicity of the pancreas; (2) rarity of calcification, cystic lesions, and peripancreatic fluid collection; (3) thickened layer structure of the bile duct wall; (4) iso/hypervascularity in the swollen portion of the pancreas; (5) attenuation of pancreatic swelling and bile duct wall thickening after steroid therapy; and (6) multiple hypoechoic masses in various organs, including the pancreas. Contrast-enhanced endoscopic ultrasonography is potentially a useful tool in the differential diagnosis and for assessment of the efficacy of steroid therapy by enabling evaluation of the vascularity of the lesions. Along with the presence of IgG4-positive plasma cells, verification of obliterative phlebitis is highly specific for the histological diagnosis of AIP.
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Affiliation(s)
- Go Kobayashi
- Department of Gastroenterology, Sendai City Medical Center, 5-22-1 Tsurugaya, Miyagino-ku, Sendai, 983-0824, Japan.
| | - Naotaka Fujita
- Department of Gastroenterology, Sendai City Medical Center, 5-22-1 Tsurugaya, Miyagino-ku, Sendai, 983-0824, Japan
| | - Yutaka Noda
- Department of Gastroenterology, Sendai City Medical Center, 5-22-1 Tsurugaya, Miyagino-ku, Sendai, 983-0824, Japan
| | - Kei Ito
- Department of Gastroenterology, Sendai City Medical Center, 5-22-1 Tsurugaya, Miyagino-ku, Sendai, 983-0824, Japan
| | - Jun Horaguchi
- Department of Gastroenterology, Sendai City Medical Center, 5-22-1 Tsurugaya, Miyagino-ku, Sendai, 983-0824, Japan
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Matsubayashi H, Furukawa H, Uesaka K, Sasaki K, Ono H, Hruban RH. Autoimmune pancreatitis accompanied by cholecystitis, periaortitis and pseudotumors of the liver. Case Rep Gastroenterol 2008; 2:155-61. [PMID: 21490882 PMCID: PMC3075136 DOI: 10.1159/000125456] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/17/2022] Open
Abstract
A variety of extrapancreatic lesions have been associated with autoimmune pancreatitis (AIP), and these lesions can be difficult to diagnose. We report a patient referred to Shizuoka Cancer Center with the diagnosis of a possible biliary carcinoma with liver metastasis who was shown to have AIP accompanied by pseudotumors of liver. Clinical imaging revealed diffuse enlargement of the head of the pancreas with irregular narrowing of the main pancreatic duct and inferior common bile duct, multiple liver masses, mediastinal lymphadenopathy, and thickening of the wall of the gallbladder and abdominal aorta. Cytology and biopsy from the pancreaticobiliary tract was negative for malignancy. Serum carcinoembryonic antigen (CEA) and carbohydrate antigen (CA19-9) levels were in the normal range, but soluble interleukin 2 receptor (sIL2R), IgG4 and antinuclear antibody were abnormally high (sIL2R: 2,550 U/ml; IgG4: 764 mg/dl). Corticosteroid therapy was effective and these abnormal findings all improved. This case demonstrates the clinical importance of AIP accompanied by other systemic disorders in the differential diagnosis of patients with a pancreatic mass lesion.
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Extrapancreatic F-18 FDG accumulation in autoimmune pancreatitis. Ann Nucl Med 2008; 22:215-9. [PMID: 18498037 DOI: 10.1007/s12149-007-0107-y] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/04/2007] [Accepted: 09/11/2007] [Indexed: 12/16/2022]
Abstract
We report two cases of autoimmune pancreatitis (AIP) in which fluorine-18 fluorodeoxyglucose (FDG) showed moderate accumulation in the pancreas, as well as in bilateral submandibular glands and in multifocal lymph nodes. FDG positron emission tomography (PET)/computed tomography (CT) is a useful diagnostic tool to assess the extrapancreatic lesions of AIP, which is a recently proposed new clinicopathological entity named immunoglobulin G4 (IgG4)-related systemic disease. Recognition of the FDG-PET/CT findings of IgG4-related sclerosing disease is crucial to avoid unnecessary surgery or other intervention because of similarities to malignant lymphoma or malignant tumor with multiple lymph node metastases.
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Abstract
In this review article, we will briefly describe the main characteristics of autoimmune pancreatitis and then we will concentrate on our aim, namely, evaluating the clinical characteristics of patients having recurrence of pain from the disease. In fact, the open question is to evaluate the possible presence of autoimmune pancreatitis in patients with an undefined etiology of acute pancreatitis and for this reason we carried out a search in the literature in order to explore this issue. In cases of recurrent attacks of pain in patients with “diopathic”pancreatitis, we need to keep in mind the possibility that our patients may have autoimmune pancreatitis. Even though the frequency of this disease seems to be quite low, we believe that in the future, by increasing our knowledge on the subject, we will be able to diagnose an ever-increasing number of patients having acute recurrence of pain from autoimmune pancreatitis.
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Uehara T, Hamano H, Kawakami M, Koyama M, Kawa S, Sano K, Honda T, Oki K, Ota H. Autoimmune pancreatitis-associated prostatitis: Distinct clinicopathological entity. Pathol Int 2008; 58:118-25. [DOI: 10.1111/j.1440-1827.2007.02199.x] [Citation(s) in RCA: 82] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
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Cornell LD, Chicano SL, Deshpande V, Collins AB, Selig MK, Lauwers GY, Barisoni L, Colvin RB. Pseudotumors due to IgG4 immune-complex tubulointerstitial nephritis associated with autoimmune pancreatocentric disease. Am J Surg Pathol 2007; 31:1586-97. [PMID: 17895762 DOI: 10.1097/pas.0b013e318059b87c] [Citation(s) in RCA: 151] [Impact Index Per Article: 8.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Autoimmune pancreatitis (AIP) is a mass-forming chronic fibroinflammatory condition centered on the pancreatobiliary system and characterized by predominant immunoglobulin G4 (IgG4)-positive plasma cells. Recent reports have brought to light the multiorgan involvement of this disease. We describe a series of 5 cases of tubulointerstitial nephritis (TIN) associated with AIP and characterize the clinical, pathologic, ultrastructural, and immunopathologic features of TIN. The specimens consisted of 4 biopsies and 1 nephrectomy. The average patient age was 64 years (range 45 to 78) and the male to female ratio was 4:1. All had histologic and/or clinical and radiographic evidence of AIP, mass-forming sclerosing cholangitis, or both. The clinical impression in 4 patients was a renal mass or vasculitis. Two patients had renal insufficiency. Histologic preparations revealed a dense tubulointerstitial lymphoplasmacytic infiltrate. Eosinophils were often numerous. Tubulitis and tubular injury were present, along with tubular atrophy with focally thickened tubular basement membranes (TBMs). The histologic appearance ranged from a cellular, inflammatory pattern without tubular atrophy to a striking expansive interstitial fibrosis with tubular destruction. The nephrectomy specimen demonstrated a masslike nodular pattern of inflammation with normal renal tissue elsewhere. Glomeruli were uninvolved. By immunohistochemistry or immunofluorescence, numerous plasma cells in the infiltrate were positive for IgG4. TBM granular IgG deposits, predominantly of the IgG4 subclass, were detected in 4 of 5 cases by either immunofluorescence or immunohistochemistry. By electron microscopy, corresponding amorphous electron-dense deposits were present in the TBM in these cases. This type of TIN, typically characterized by a masslike lesion consisting of a lymphoplasmacytic infiltrate with eosinophils and prominent IgG4-positive plasma cells and immune-complex deposits in the TBM, may be part of a systemic IgG4-related disease, which we term "IgG4-associated immune complex Multiorgan Autoimmune Disease" (IMAD).
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Affiliation(s)
- Lynn D Cornell
- Departments of Pathology, Massachusetts General Hospital, 55 Fruit Street, Boston, MA 02114, USA.
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Díaz-Torné C, Narváez J, De Lama E, Diez-García M, Narváez JA, Bernad B, Llatjos R, Nolla JM, Valverde J. Inflammatory pseudotumor of the liver associated with rheumatoid arthritis. ACTA ACUST UNITED AC 2007; 57:1102-6. [PMID: 17665472 DOI: 10.1002/art.22904] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
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Kobayashi G, Fujita N, Noda Y, Ito K, Horaguchi J, Takasawa O, Obana T, Nakahara K, Uzuki M, Sawai T. Lymphoplasmacytic sclerosing pancreatitis forming a localized mass: a variant form of autoimmune pancreatitis. J Gastroenterol 2007; 42:650-6. [PMID: 17701128 DOI: 10.1007/s00535-007-2068-2] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/25/2006] [Accepted: 04/22/2007] [Indexed: 02/04/2023]
Abstract
BACKGROUND To elucidate the correlation of autoimmune pancreatitis (AIP) and mass-forming pancreatitis, in which a localized mass is formed in the pancreas. METHODS Nine cases of mass-forming pancreatitis were divided into two groups, one consisting of cases that met the histological diagnostic criteria for AIP by the Japan Pancreas Society (JPS) and the other consisting of cases which did not. Histological findings, immunohistological findings, and pancreatograms were compared between these groups. RESULTS Six cases met the histological criteria of JPS (group A) and the other three did not (group B). In the mass-forming portion in group A, the wall of the pancreatic duct showed marked thickening. However, the epithelium was well preserved, and dilatation of the branch ducts or protein plugs were rarely observed. All cases showed marked obliterative phlebitis. The IgG4 labeling index (LI) was 25% or more in all but one case. In the portion other than the mass, the lobular structure was well preserved and the IgG4 LI was less than 10%. The pancreatogram showed localized stenosis or obstruction at the site of the mass associated with a normal-appearing main pancreatic duct in the remaining portion. In group B, histological findings typical of chronic pancreatitis with dilated branch ducts and protein plugs were observed. Obliterative phlebitis was not confirmed. The IgG4 LI in the mass-forming portion was low (2.3%-11.1%). CONCLUSIONS There exists a subgroup of AIP showing localized mass formation and stenosis or obstruction of the main pancreatic duct with prominent obliterative phlebitis associated with a normal ductal segment.
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MESH Headings
- Adult
- Aged
- Antibodies, Anti-Idiotypic/immunology
- Autoimmune Diseases/diagnosis
- Autoimmune Diseases/etiology
- Autoimmune Diseases/immunology
- CD4-CD8 Ratio
- CD4-Positive T-Lymphocytes/immunology
- CD8-Positive T-Lymphocytes/immunology
- Cholangiopancreatography, Endoscopic Retrograde
- Diagnosis, Differential
- Female
- Humans
- Immunoglobulin G/immunology
- Leukemia, Lymphocytic, Chronic, B-Cell/complications
- Leukemia, Lymphocytic, Chronic, B-Cell/diagnosis
- Leukemia, Lymphocytic, Chronic, B-Cell/immunology
- Magnetic Resonance Imaging
- Male
- Middle Aged
- Pancreatitis/diagnosis
- Pancreatitis/etiology
- Pancreatitis/immunology
- Prognosis
- Retrospective Studies
- Tomography, X-Ray Computed
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Affiliation(s)
- Go Kobayashi
- Department of Gastroenterology, Sendai City Medical Center, 5-22-1 Tsurugaya, Miyagino-ku, Sendai 983-0824, Japan
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Shimosegawa T. 1. Diagnosis and Treatment of Autoimmune Pancreatitis. ACTA ACUST UNITED AC 2007; 96:1946-52. [DOI: 10.2169/naika.96.1946] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
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Uchida K, Satoi S, Miyoshi H, Hachimine D, Ikeura T, Shimatani M, Matsushita M, Takaoka M, Takai S, Ashida K, Okazaki K. Inflammatory pseudotumors of the pancreas and liver with infiltration of IgG4-positive plasma cells. Intern Med 2007; 46:1409-12. [PMID: 17827840 DOI: 10.2169/internalmedicine.46.6430] [Citation(s) in RCA: 44] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/26/2023] Open
Abstract
Recently, it has been reported that autoimmune pancreatitis (AIP) can be complicated with various extrapancreatic lesions. Here, we report a very rare case of pancreatic and hepatic inflammatory pseudotumor (IPT) with the infiltration of IgG4-positive plasmacytes. The patient showed pancreatic and hepatic masses with elevated levels of serum IgG4. Endoscopic retrograde cholangiopancreatography revealed narrowing of the intrapancreatic bile duct. Fluorine-18fluorodeoxyglucose positron emission tomography suggested pancreatic cancer with hepatic metastasis. Histopathologic findings showed fibrosis and infiltration of IgG4-positive plasmacytes, suggesting IPT. The present case suggests a possible common mechanism in the development of AIP and IPT of the liver.
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Affiliation(s)
- Kazushige Uchida
- Third Department of Internal Medicine, Kansai Medical University, Moriguchi.
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Abstract
PURPOSE OF REVIEW As in our previous reviews, we endeavor to review important new observations in chronic pancreatitis made in the past year. Topics recently reviewed were truncated to accommodate a surge in publications on clinical aspects of chronic pancreatitis, which contained new observations or insights into new or old concepts. RECENT FINDINGS Cystic fibrosis carriers have been found to be at increased risk of pancreatitis. Autoimmune pancreatitis may belong to a multiorgan immunoglobulin G4-related autoimmune disease, and the natural history of chronic pancreatitis differs among the etiologies. Diffusion-weighted magnetic resonance imaging improves upon previous methodologies for diagnosing reduced pancreatic exocrine secretion, and fecal elastase-1 has been found to be a poor test for diagnosing pancreatic malabsorption. Visceral hyperalgesia or heightened central pain perception may contribute to pain in chronic pancreatitis. Instruments are evolving to assess quality of life in chronic pancreatitis, and fibrolytic agents have been found to have therapeutic promise. SUMMARY Researchers this past year have further characterized genetic, molecular and clinical aspects of chronic pancreatitis. Advancing the understanding of fibrogenesis, mechanisms of exocrine insufficiency, calcification, and pain and continuing development/modification of diagnostic tests should lead to improved prevention, detection and treatment of the condition. More accurate quantification of outcomes is critical for translating potential therapies from bench to bedside.
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Affiliation(s)
- Matthew J DiMagno
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, Michigan, USA.
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Kamisawa T, Okamoto A. Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol 2006; 41:613-25. [PMID: 16932997 PMCID: PMC2780632 DOI: 10.1007/s00535-006-1862-6] [Citation(s) in RCA: 376] [Impact Index Per Article: 19.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/19/2006] [Accepted: 06/20/2006] [Indexed: 02/06/2023]
Abstract
Autoimmune pancreatitis (AIP) is a peculiar type of pancreatitis of presumed autoimmune etiology. Many new clinical aspects of AIP have been clarified during the past 10 years, and AIP has become a distinct entity recognized worldwide. However, its precise pathogenesis or pathophysiology remains unclear. As AIP dramatically responds to steroid therapy, accurate diagnosis of AIP is necessary to avoid unnecessary surgery. Characteristic dense lymphoplasmacytic infiltration and fibrosis in the pancreas may prove to be the gold standard for diagnosis of AIP. However, since it is difficult to obtain sufficient pancreatic tissue, AIP should be diagnosed currently based on the characteristic radiological findings (irregular narrowing of the main pancreatic duct and enlargement of the pancreas) in combination with serological findings (elevation of serum gamma-globulin, IgG, or IgG4, along with the presence of autoantibodies), clinical findings (elderly male preponderance, fluctuating obstructive jaundice without pain, occasional extrapancreatic lesions, and favorable response to steroid therapy), and histopathological findings (dense infiltration of IgG4-positive plasma cells and T lymphocytes with fibrosis and obliterative phlebitis in various organs). It is apparent that elevation of serum IgG4 levels and infiltration of abundant IgG4-positive plasma cells into various organs are rather specific to AIP patients. We propose a new clinicopathological entity, "IgG4-related sclerosing disease", and suggest that AIP is a pancreatic lesion reflecting this systemic disease.
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Affiliation(s)
- Terumi Kamisawa
- Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, 113-8677, Japan
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Lopez-Tomassetti Fernandez EM, Luis HD, Malagon AM, Gonzalez IA, Pallares AC. Recurrence of inflammatory pseudotumor in the distal bile duct: Lessons learned from a single case and reported cases. World J Gastroenterol 2006; 12:3938-43. [PMID: 16804988 PMCID: PMC4087951 DOI: 10.3748/wjg.v12.i24.3938] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Inflammatory myofibroblastic tumors (IMTs) or inflammatory pseudotumors (IPs) have been extensively discussed in the literature. They are usually found in the lung and upper respiratory tract. However, reporting of cases involving the biliopancreatic region has increased over recent years. Immunohistochemical study of these lesions limited to the pancreatic head or distal bile duct seems to be compatible with those observed in a new entity called autoimmune pancreatitis, but usually intense fibrotic reaction (zonation) predominates producing a mass. When this condition is limited to the pancreatic head, the common bile duct might be involved by the inflammatory process and jaundice may occur often resembling adenocarcinoma of the pancreas. We have previously reported a case of IMT arising from the bile duct associated with autoimmune pancreatitis which is an extremely rare entity. Four years after Kaush-Whipple resection, radiological examination on routine follow-up revealed a tumor mass, suggesting local recurrence. Ultrasound-guided FNA confirmed our suspicious diagnosis. This present case, as others, suggests that persistent follow-up is necessary in order to prevent irreversible liver damage at this specific location.
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Affiliation(s)
- E M Lopez-Tomassetti Fernandez
- Department of Gastrointestinal Surgery, University Hospital of Canary Islands, Ofra s/n. La Cuesta, La Laguna, Santa Cruz de Tenerife, Spain.
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Hamano H, Kawa S. Are there any other organs in which autoimmune pancreatitis-associated lesions remain to be identified? Intern Med 2006; 45:883-4. [PMID: 16946568 DOI: 10.2169/internalmedicine.45.0162] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
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