Retroperitoneal fibrosis associated with immunoglobulin G4-related disease

Nao Fujimori, Tetsuhide Ito, Hisato Igarashi, takamasa ono, Taichi Nakamura, Yusuke Niina, Masayuki Hijioka, Lingaku Lee, Masahiko Uchida, Ryoichi Takayanagi

Research output: Contribution to journalReview article

31 Citations (Scopus)

Abstract

Retroperitoneal fibrosis is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs. Retroperitoneal fibrosis can be of 2 types: idiopathic and secondary. The recently advocated concept and diagnostic criteria of immunoglobulin G4 (IgG4)-related disease, derived from research on autoimmune pancreatitis (AIP), has led to widespread recognition of retroperitoneal fibrosis as a condition caused by IgG4-related disease. We now know that previously diagnosed idiopathic retroperitoneal fibrosis includes IgG4-related disease; however, the actual prevalence is unclear. Conversely, some reports on AIP suggest that retroperitoneal fibrosis is concurrently found in about 10% of IgG4-related disease. Because retroperitoneal fibrosis has no specific symptoms, diagnosis is primarily based on diagnostic imaging (computed tomography and magnetic resonance imaging), which is also useful in evaluating the effect of therapy. Idiopathic retroperitoneal fibrosis can occur at different times with other lesions of IgG4-related disease including AIP. Thus, the IgG4 assay is recommended to diagnose idiopathic retroperitoneal fibrosis. High serum IgG4 levels should be treated and monitored as a symptom of IgG4-related disease. The first line of treatment for retroperitoneal fibrosis is steroid therapy regardless of its cause. For patients with concurrent AIP, i.e., IgG4-related retroperitoneal fibrosis, the starting dose of steroid is usually 30-40 mg/d. The response to steroid therapy is generally favorable. In most cases, the pancreatic lesion and retroperitoneal fibrosis improve after the initial treatment. However, the epidemiology, treatment for recurring retroperitoneal fibrosis, and long-term prognosis are still largely unknown. Further analysis of such cases and research are necessary.

Original languageEnglish
Pages (from-to)35-41
Number of pages7
JournalWorld Journal of Gastroenterology
Volume19
Issue number1
DOIs
Publication statusPublished - Feb 18 2013

Fingerprint

Retroperitoneal Fibrosis
Immunoglobulins
Pancreatitis
Steroids
Therapeutics
Diagnostic Imaging
Rare Diseases
Research
Autoimmune Diseases

All Science Journal Classification (ASJC) codes

  • Gastroenterology

Cite this

Retroperitoneal fibrosis associated with immunoglobulin G4-related disease. / Fujimori, Nao; Ito, Tetsuhide; Igarashi, Hisato; ono, takamasa; Nakamura, Taichi; Niina, Yusuke; Hijioka, Masayuki; Lee, Lingaku; Uchida, Masahiko; Takayanagi, Ryoichi.

In: World Journal of Gastroenterology, Vol. 19, No. 1, 18.02.2013, p. 35-41.

Research output: Contribution to journalReview article

Fujimori, N, Ito, T, Igarashi, H, ono, T, Nakamura, T, Niina, Y, Hijioka, M, Lee, L, Uchida, M & Takayanagi, R 2013, 'Retroperitoneal fibrosis associated with immunoglobulin G4-related disease', World Journal of Gastroenterology, vol. 19, no. 1, pp. 35-41. https://doi.org/10.3748/wjg.v19.i1.35
Fujimori, Nao ; Ito, Tetsuhide ; Igarashi, Hisato ; ono, takamasa ; Nakamura, Taichi ; Niina, Yusuke ; Hijioka, Masayuki ; Lee, Lingaku ; Uchida, Masahiko ; Takayanagi, Ryoichi. / Retroperitoneal fibrosis associated with immunoglobulin G4-related disease. In: World Journal of Gastroenterology. 2013 ; Vol. 19, No. 1. pp. 35-41.
@article{970adc89aa1242f090f6a923ebe7dbed,
title = "Retroperitoneal fibrosis associated with immunoglobulin G4-related disease",
abstract = "Retroperitoneal fibrosis is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs. Retroperitoneal fibrosis can be of 2 types: idiopathic and secondary. The recently advocated concept and diagnostic criteria of immunoglobulin G4 (IgG4)-related disease, derived from research on autoimmune pancreatitis (AIP), has led to widespread recognition of retroperitoneal fibrosis as a condition caused by IgG4-related disease. We now know that previously diagnosed idiopathic retroperitoneal fibrosis includes IgG4-related disease; however, the actual prevalence is unclear. Conversely, some reports on AIP suggest that retroperitoneal fibrosis is concurrently found in about 10{\%} of IgG4-related disease. Because retroperitoneal fibrosis has no specific symptoms, diagnosis is primarily based on diagnostic imaging (computed tomography and magnetic resonance imaging), which is also useful in evaluating the effect of therapy. Idiopathic retroperitoneal fibrosis can occur at different times with other lesions of IgG4-related disease including AIP. Thus, the IgG4 assay is recommended to diagnose idiopathic retroperitoneal fibrosis. High serum IgG4 levels should be treated and monitored as a symptom of IgG4-related disease. The first line of treatment for retroperitoneal fibrosis is steroid therapy regardless of its cause. For patients with concurrent AIP, i.e., IgG4-related retroperitoneal fibrosis, the starting dose of steroid is usually 30-40 mg/d. The response to steroid therapy is generally favorable. In most cases, the pancreatic lesion and retroperitoneal fibrosis improve after the initial treatment. However, the epidemiology, treatment for recurring retroperitoneal fibrosis, and long-term prognosis are still largely unknown. Further analysis of such cases and research are necessary.",
author = "Nao Fujimori and Tetsuhide Ito and Hisato Igarashi and takamasa ono and Taichi Nakamura and Yusuke Niina and Masayuki Hijioka and Lingaku Lee and Masahiko Uchida and Ryoichi Takayanagi",
year = "2013",
month = "2",
day = "18",
doi = "10.3748/wjg.v19.i1.35",
language = "English",
volume = "19",
pages = "35--41",
journal = "World Journal of Gastroenterology",
issn = "1007-9327",
publisher = "WJG Press",
number = "1",

}

TY - JOUR

T1 - Retroperitoneal fibrosis associated with immunoglobulin G4-related disease

AU - Fujimori, Nao

AU - Ito, Tetsuhide

AU - Igarashi, Hisato

AU - ono, takamasa

AU - Nakamura, Taichi

AU - Niina, Yusuke

AU - Hijioka, Masayuki

AU - Lee, Lingaku

AU - Uchida, Masahiko

AU - Takayanagi, Ryoichi

PY - 2013/2/18

Y1 - 2013/2/18

N2 - Retroperitoneal fibrosis is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs. Retroperitoneal fibrosis can be of 2 types: idiopathic and secondary. The recently advocated concept and diagnostic criteria of immunoglobulin G4 (IgG4)-related disease, derived from research on autoimmune pancreatitis (AIP), has led to widespread recognition of retroperitoneal fibrosis as a condition caused by IgG4-related disease. We now know that previously diagnosed idiopathic retroperitoneal fibrosis includes IgG4-related disease; however, the actual prevalence is unclear. Conversely, some reports on AIP suggest that retroperitoneal fibrosis is concurrently found in about 10% of IgG4-related disease. Because retroperitoneal fibrosis has no specific symptoms, diagnosis is primarily based on diagnostic imaging (computed tomography and magnetic resonance imaging), which is also useful in evaluating the effect of therapy. Idiopathic retroperitoneal fibrosis can occur at different times with other lesions of IgG4-related disease including AIP. Thus, the IgG4 assay is recommended to diagnose idiopathic retroperitoneal fibrosis. High serum IgG4 levels should be treated and monitored as a symptom of IgG4-related disease. The first line of treatment for retroperitoneal fibrosis is steroid therapy regardless of its cause. For patients with concurrent AIP, i.e., IgG4-related retroperitoneal fibrosis, the starting dose of steroid is usually 30-40 mg/d. The response to steroid therapy is generally favorable. In most cases, the pancreatic lesion and retroperitoneal fibrosis improve after the initial treatment. However, the epidemiology, treatment for recurring retroperitoneal fibrosis, and long-term prognosis are still largely unknown. Further analysis of such cases and research are necessary.

AB - Retroperitoneal fibrosis is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs. Retroperitoneal fibrosis can be of 2 types: idiopathic and secondary. The recently advocated concept and diagnostic criteria of immunoglobulin G4 (IgG4)-related disease, derived from research on autoimmune pancreatitis (AIP), has led to widespread recognition of retroperitoneal fibrosis as a condition caused by IgG4-related disease. We now know that previously diagnosed idiopathic retroperitoneal fibrosis includes IgG4-related disease; however, the actual prevalence is unclear. Conversely, some reports on AIP suggest that retroperitoneal fibrosis is concurrently found in about 10% of IgG4-related disease. Because retroperitoneal fibrosis has no specific symptoms, diagnosis is primarily based on diagnostic imaging (computed tomography and magnetic resonance imaging), which is also useful in evaluating the effect of therapy. Idiopathic retroperitoneal fibrosis can occur at different times with other lesions of IgG4-related disease including AIP. Thus, the IgG4 assay is recommended to diagnose idiopathic retroperitoneal fibrosis. High serum IgG4 levels should be treated and monitored as a symptom of IgG4-related disease. The first line of treatment for retroperitoneal fibrosis is steroid therapy regardless of its cause. For patients with concurrent AIP, i.e., IgG4-related retroperitoneal fibrosis, the starting dose of steroid is usually 30-40 mg/d. The response to steroid therapy is generally favorable. In most cases, the pancreatic lesion and retroperitoneal fibrosis improve after the initial treatment. However, the epidemiology, treatment for recurring retroperitoneal fibrosis, and long-term prognosis are still largely unknown. Further analysis of such cases and research are necessary.

UR - http://www.scopus.com/inward/record.url?scp=84873652135&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84873652135&partnerID=8YFLogxK

U2 - 10.3748/wjg.v19.i1.35

DO - 10.3748/wjg.v19.i1.35

M3 - Review article

C2 - 23326160

AN - SCOPUS:84873652135

VL - 19

SP - 35

EP - 41

JO - World Journal of Gastroenterology

JF - World Journal of Gastroenterology

SN - 1007-9327

IS - 1

ER -