A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details

Umehara, Hisanori; Okazaki, Kazuichi; Yasukawa, Masaki; Kawano, Mitsuhiro; Yamamoto, Motohisa; Saeki, Takako; Matsui, Shoko; Sumida, Takayuki; Mimori, Tsuneyo; Tanaka, Yoshiya; Tsubota, Kazuo; Yoshino, Tadashi; Kawa, Shigeyuki; Suzuki, Ritsuro; Takegami, Tsutomu; Tomosugi, Naohisa; Kurose, Nozomu; Ishigaki, Yasuhito; Azumi, Atsushi; Kojima, Masaru; Nakamura, Shigeo; Inoue, Dai

doi:10.3109/s10165-011-0508-6

Cited by 600 publications

(281 citation statements)

References 66 publications

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“…This is due to the prominent infiltration of lymphocytes and plasmacytes with fibrosis 3,5,14. The causes are still unclear; however, some abnormal immunological mechanisms are involved.…”

Section: Current Concepts Of Igg4-rd In the Hepato-bilio-pancreatic Smentioning

confidence: 99%

“…In 1995, Yoshida et al 2. proposed a novel concept of AIP, which has been accepted as type 1 AIP (IgG4-related pancreatitis), the pancreatic manifestation of IgG4-related disease (IgG4-RD) 3. IgG4-RD is recognized worldwide as a novel clinical entity following the epoch-making evidence of increased serum levels of IgG4 in the history of AIP 4.…”

Section: Introductionmentioning

confidence: 99%

“…Referring to the original concept of multifocal fibrosclerosis, recent studies led us to develop a novel concept of a systemic disease such as IgG4-related systemic sclerosing disease,14 systemic IgG4-related plasmacytic syndrome,15 or IgG4-positive multiorgan lymphoproliferative syndrome,16 all of which may refer to the same conditions. Based on these findings, although it is unclear whether the pathogenetic mechanisms in individual organs are same or not,3,17 the comprehensive term “IgG4-related disease IgG4-RD,” which was internationally endorsed with the proposal of nomenclatures for individual organ lesions as well as pathological consensus, and diagnostic criteria have been proposed from the Japanese investigators 17. In this review, we discussed the current concepts of hepato-bilio-pancreatic lesions and recent advances in our understanding of the pathogenesis of IgG4-RD.…”

Section: Introductionmentioning

confidence: 99%

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Recent Advances in the Concept and Pathogenesis of IgG4-Related Disease in the Hepato-Bilio-Pancreatic System

et al. 2014

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Recent studies have proposed nomenclatures of type 1 autoimmune pancreatitis (AIP) (IgG4-related pancreatitis), IgG4-related sclerosing cholangitis (IgG4-SC), IgG4-related cholecystitis, and IgG4-related hepatopathy as IgG4-related disease (IgG4-RD) in the hepato-bilio-pancreatic system. In IgG4-related hepatopathy, a novel concept of IgG4-related autoimmune hepatitis (AIH) with the same histopathological features as AIH has been proposed. Among organs involved in IgG4-RD, associations with pancreatic and biliary lesions are most frequently observed, supporting the novel concept of “biliary diseases with pancreatic counterparts.” Targets of type 1 AIP and IgG4-SC may be periductal glands around the bile and pancreatic ducts. Based on genetic backgrounds, innate and acquired immunity, Th2-dominant immune status, regulatory T (Treg) or B cells, and complement activation via a classical pathway may be involved in the development of IgG4-RD. Although the role of IgG4 remains unclear in IgG4-RD, IgG4-production is upregulated by interleukin 10 from Treg cells and by B cell activating factor from monocytes/basophils with stimulation of toll-like receptors/nucleotide-binding oligomerization domain-like receptors. Based on these findings, we have proposed a hypothesis for the development of IgG4-RD in the hepato-bilio-pancreatic system. Further studies are necessary to clarify the pathogenic mechanism of IgG4-RD.

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“…This is due to the prominent infiltration of lymphocytes and plasmacytes with fibrosis 3,5,14. The causes are still unclear; however, some abnormal immunological mechanisms are involved.…”

Section: Current Concepts Of Igg4-rd In the Hepato-bilio-pancreatic Smentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Recent Advances in the Concept and Pathogenesis of IgG4-Related Disease in the Hepato-Bilio-Pancreatic System

et al. 2014

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“…10,11 Récemment, un certain nombre d'auteurs ont laissé entendre que la FPR idiopathique est en partie due à une affection inflammatoire systémique connue sous le nom de maladie à IgG4. 12 Celle-ci a été identifiée dans 47-58 % des patients ayant déjà reçu un diagnostic de FPR idiopathique. 13,14 De même, environ 50 % des anévrismes inflammatoires de l'aorte abdominale sont liés aux IgG4.…”

Section: Fibrose Rétropéritonéaleunclassified

“…Deux groupes japonais ont développé en 2011 des critères diagnostiques plus complets pour les maladies à IgG4 (tableau 2). 12 La maladie à IgG4 est responsable seulement d'une partie des cas de FRPI (50 %). 13,14 La possibilité d'un terrain prédisposé, voire de facteurs génétiques, a été envisagée.…”

Section: La Frpi Et La Maladie à Igg4unclassified

Fibrose rétropéritonéale : revue de littérature

Jakhlal

Elghazoui

Jabbour

et al. 2017

CUAJ

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RésuméLa fibrose rétropéritonéale (FRP) est une maladie rare, caractérisée par la présence d'un tissu fibro-inflammatoire aberrant qui se forme souvent autour de la portion sous-rénale de l'aorte abdominale et des artères iliaques. Une obstruction urétérale est souvent observée. Récemment, la FPR idiopathique est rapportée parmi les manifestations de la maladie à IgG4. L'imagerie joue un rôle important dans le diagnostic de la FRP. Les glucocorticoïdes sont le traitement de référence, utilisés en monothérapie ou en association avec d'autres agents médicamenteux. En cas d'échec des mesures conservatrices, la chirurgie peut être proposée. Récemment, des techniques minimalement invasives ont été utilisées.

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