Retroperitoneal fibrosis associated with immunoglobulin G4-related disease

Fujimori, Nao; Ito, Tetsuhide; Igarashi, Hisato; Oono, Takamasa; Nakamura, Taichi; Niina, Yusuke; Hijioka, Masayuki; Lee, Lingaku; Uchida, Masahiko; Takayanagi, Ryoichi

doi:10.3748/wjg.v19.i1.35

Cited by 55 publications

(48 citation statements)

References 39 publications

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“…It is reported that CT diagnosis of AIP has a positive rate up to 92%; however, our study shows that on the basis of pancreatic and bile duct lesions, the performances of ultrasound, CT, and MRI are similar. However, regarding the peripancreatic cystic ring and the specific performance of AIP, the highest rate is with CT (75%), which is also useful in evaluating the effect of therapy [21]. MRI has the highest rate in finding peripancreatic lymph nodes (53.6%) and also has a high rate in finding the capsule-like low-density rim around the pancreas.…”

Section: Discussionmentioning

confidence: 99%

Clinical Features and Comprehensive Diagnosis of Autoimmune Pancreatitis in China

Wen²,

Huang³

et al. 2013

Digestion

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Aim: To search for the key factors of autoimmune pancreatitis (AIP) in China and to improve the early identification of AIP in order to avoid misdiagnosing it as pancreatic carcinoma. Methods: Clinical, imaging, laboratory, and pathological data about AIP were collected and analyzed from 1996 to 2011 in The Chinese People's Liberation Army General Hospital. Results: A total of 33 patients (male 29, female 4) aged 35-76 years (52.3 ± 9.3 years) meeting the International Consensus Diagnostic Criteria for type 1 (n = 32) or type 2 (n = 1) AIP were included. With improved understanding of AIP, the misdiagnosis rate and unnecessary laparotomy rate was decreased from 95.7% (22/23) and 91.3% (21/23) before 2006, to 20.0% (2/10) (p = 0.001) and 0.0% (p = 0.001) respectively after 2006. The major symptoms were jaundice in (24/33, 72.7%) and abdominal pain (12/33, 36.4%). Half of the patients had other accompanied autoimmune disorders. Laboratory tests showed an elevated level of serum IgG in 9 out of 11 patients (81.8%) and an increased eosinophil count in 16 out of 33 patients (48.5%). The serum IgG4 levels were twice the upper limit of the normal value. CT scan showed a low-contrast margin characterized by a capsule-like rim in 24 of 32 patients (75.0%). Pathologic examinations showed fibrosis and infiltration of massive lymphocytes and plasma cells in the pancreas. Prednisone was given to the patients and proved to be quite effective for all of them (12/12, 100%). Conclusion: Type 1 AIP shows a sex predilection compared with type 2 AIP in China. In order to diagnose AIP exactly, a comprehensive strategy, especially including imaging and pathological examination, should be used. With growing awareness, fewer AIP patients with this diagnosis would be misdiagnosed and incorrectly treated.

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Section: Discussionmentioning

confidence: 99%

Clinical Features and Comprehensive Diagnosis of Autoimmune Pancreatitis in China

Wen²,

Huang³

et al. 2013

Digestion

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“…It is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs. There will be a series of clinical manifestions once the fibro‐inflammatory tissue encases the abdominal aorta, iliac arteries and urinary duct . RPF is generally divided into two types: idiopathic RPF (IRPF) and secondary RPF.…”

Section: Introductionmentioning

confidence: 99%

IgG4‐related retroperitoneal fibrosis: a newly characterized disease

2016

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Retroperitoneal fibrosis (RPF) is a rare disease characterized by chronic, nonspecific inflammatory and sclerotic or fibrotic tissue in the periaortic or periiliac retroperitoneum that encases adjacent structures. There will be a series of clinical manifestations once the proliferated fibrous tissues encase the abdominal aorta, iliac arteries and urinary duct. RPF is generally divided into two types: idiopathic retroperitoneal fibrosis (IRPF) without identified pathogenesis, making up about two-thirds of cases, and secondary retroperitoneal fibrosis. Recent studies on Immunoglobulin G4-related disease (IgG4-RD) reveal that abundant infiltration of IgG4 positive plasma cells is found in biopsies on the mass of RPF of some IRPF patients, which is identified as one spectrum of IgG4-RD and is named IgG4-related RPF. IgG4-related RPF is often misdiagnosed as retroperitoneal visceral malignancy and is treated with surgery. In addition, because of its good response to glucocorticoid, early detection and treatment is important. We review the definition, epidemiology, clinical features, diagnostic criteria, treatment and prognosis of IgG4-related RPF in this article to raise awareness of this newly characterized disease.

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“…IgG4-RD has been reported in 31%-59% of patients with idiopathic retroperitoneal fibrosis. 82 Many patients are asymptomatic and the diagnosis is made incidentally, during imaging. Systemic symptoms, back pain, or obstructive symptoms from involvement of ureters or inferior vena cava can occur.…”

Section: Non-gastrointestinal Tract Manifestationsmentioning

confidence: 99%

Gastrointestinal and Extra-Intestinal Manifestations of IgG4–Related Disease

et al. 2018

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IgG4-related disease (IgG4-RD) is a chronic relapsing multi-organ fibro-inflammatory syndrome of presumed autoimmune etiology. It is characterized by increased serum levels of IgG4 and tissue infiltration by IgG4 cells. Increased titers of autoantibodies against a spectrum of self-antigens and response to steroids have led to its characterization as an autoimmune disease. However, the pathognomonic antigens probably differ among manifestations, and different antigens or autoantibodies produce similar immune reactions in different organs. Little is known about the pathogenic effects, if any, of serum IgG4 or IgG4 plasma cells in tissues. Despite several animal models of the disease, none truly recapitulates human IgG4-RD. Histologic analyses of tissues from patients with IgG4-RD reveal a dense lymphoplasmacytic infiltrate rich in IgG4 plasma cells, storiform fibrosis, and obliterative phlebitis, although these features vary among organs. Typical presentation and imaging findings include mass-forming synchronous or metachronous lesions in almost any organ, but most commonly in the pancreas, bile duct, retroperitoneum, kidneys, lungs, salivary and lacrimal glands, orbit, and lymph nodes. In all organs, inflammation can be reduced by corticosteroids and drugs that deplete B cells, such as rituximab. Patients with IgG4-RD have relapses that respond to primary therapy. Intense fibrosis accompanies the inflammatory response, leading to permanent organ damage and insufficiency. Death from IgG4-RD is rare. IgG4-RD is a multi-organ disease with predominant pancreatico-biliary involvement. Despite its relapsing-remitting course, patients have an excellent prognosis.

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Retroperitoneal fibrosis associated with immunoglobulin G4-related disease

Cited by 55 publications

References 39 publications

Clinical Features and Comprehensive Diagnosis of Autoimmune Pancreatitis in China

Clinical Features and Comprehensive Diagnosis of Autoimmune Pancreatitis in China

IgG4‐related retroperitoneal fibrosis: a newly characterized disease

Gastrointestinal and Extra-Intestinal Manifestations of IgG4–Related Disease

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