The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD.

Published on Jan 28, 2021in Modern Rheumatology2.113
· DOI :10.1080/14397595.2020.1859710
Hisanori Umehara56
Estimated H-index: 56
,
Kazuichi Okazaki85
Estimated H-index: 85
(Kansai Medical University)
+ 7 AuthorsMitsuhiro Kawano34
Estimated H-index: 34
(Kanazawa University)
Sources
Abstract
IgG4-related disease (IgG4-RD) is a fascinating clinical entity first reported in this century in Japan, and includes a wide variety of diseases, such as formerly named Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis and retroperitoneal fibrosis. The Japanese IgG4 team organized by the Ministry of Health, Labor and Welfare (MHLW) of Japan has published the first criteria, comprehensive diagnostic (CD) criteria for IgG-RD 2011. Thereafter, IgG4-RD has been accepted widely and many cases have been reported from all over the world. Several problems have arisen in clinical practice, however, including the difficulty obtaining biopsy samples, and the sensitivity and specificity in cut off level of serum IgG4 and impaired immunostaining of IgG4. Given these situations, the Japanese IgG4 team has updated the 2011 comprehensive diagnostic criteria for IgG4-RD and propose the 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD, which consists of 3 domains; 1) Clinical and radiological features, 2) Serological diagnosis and 3) Pathological diagnosis. In addition, the new pathological diagnosis is composed by three sub-items including storiform fibrosis and obliterative phlebitis.
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#1Kenji NotoharaH-Index: 41
#2Terumi KamisawaH-Index: 77
Last. Kazuichi Okazaki (Kansai Medical University)H-Index: 85
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The biopsy-based diagnosis of autoimmune pancreatitis (AIP) is difficult but is becoming imperative for pathologists due to the increased amount of endoscopic ultrasound-guided biopsy tissue. To cope with this challenge, we propose guidance for the biopsy diagnosis of type 1 AIP. This guidance is for pathologists and comprises three main parts. The first part includes basic issues on tissue acquisition, staining, and final diagnosis, and is intended for gastroenterologists as well. The second pa...
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#1Yuichi Sumii (Okayama University)H-Index: 2
#2Noboru Asada (Okayama University)H-Index: 15
Last. Yoshinobu Maeda (Okayama University)H-Index: 7
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IgG4-producing marginal zone B-cell lymphomas (MZLs) have been recently proposed as a subtype of MZLs. Despite the abundant literature on pathophysiological features of this type of lymphoma, only a few retrospective studies pertaining to the treatment outcomes have been reported, and its prognosis remains unclear. We retrospectively analyzed seven patients with IgG4-producing MZLs diagnosed at our institute, with specific reference to treatment and outcomes. The median age was 69.0 years (55–79...
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#1Samiullah Khan (Tianjin Medical University General Hospital)H-Index: 4
#2Lanping Zhu (Tianjin Medical University General Hospital)H-Index: 3
Last. Bangmao Wang (Tianjin Medical University General Hospital)H-Index: 21
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BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune inflammatory and fibrotic condition. The disease is characterized by tissue infiltration with dense lymphoplasmacytes and IgG4-positive plasma cells. SUMMARY: The aim of this study was to provide gastroenterologists with novel insights into evaluating the gastroesophageal involvement with IgG4-RD or mimickers of this condition and to give special attention to clinicopathological features. A literature review was performed u...
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#1Zachary S. Wallace (Harvard University)H-Index: 27
#2Ray NadenH-Index: 7
Last. John H. Stone (Harvard University)H-Index: 94
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OBJECTIVE: IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. METHODS: An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators use...
54 CitationsSource
#1Zachary S. Wallace (Harvard University)H-Index: 27
#2Ray Naden (McMaster University)H-Index: 7
Last. John H. Stone (Harvard University)H-Index: 94
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IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serological, radiological and pathological data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exe...
109 CitationsSource
#1Ichiro Mizushima (Kanazawa University)H-Index: 13
#2Satomi Kasashima (Kanazawa University)H-Index: 22
Last. Nobukazu Ishizaka (Osaka Medical College)H-Index: 37
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: IgG4-related disease is a systemic disease, characterized by elevation of serum IgG4 and, histopathologically, massive infiltration of IgG4+ lymphocyte and plasma cell infiltration, storiform fibrosis, causing enlargement, nodules or thickening. It may affect various organs simultaneously or metachronously. Here we analyzed the clinical and pathological characteristics of 99 patients diagnosed with IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis. Of 99 patients (women/men,...
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#1Kazunori Yamada (Kanazawa University)H-Index: 18
#2Motohisa Yamamoto (Sapporo Medical University)H-Index: 41
Last. Mitsuhiro Kawano (Kanazawa University)H-Index: 34
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Background The aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of IgG4-RD in Japan.
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#1Hisanori UmeharaH-Index: 56
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 85
Last. Tsutomu Chiba (Kyoto University)H-Index: 105
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We read with great interest the editorial by Fox and Fox1 describing the use of serum immunoglobulin G4 (IgG4) concentrations as a marker for IgG4-related disease (IgG4-RD). IgG4-RD is a fascinating clinical entity including a wide variety of diseases, formerly diagnosed as Mikulicz’s disease, autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis and retroperitoneal fibrosis.2 ,3 However, universal criteria for IgG4-RD have not yet been established at present, making its diagnosis i...
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#1Hisanori UmeharaH-Index: 56
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 85
Last. Tsutomu Chiba (Kyoto University)H-Index: 105
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AbstractIgG4-related disease (IgG4-RD) is a fascinating clinical entity proposed by Japanese investigators, and includes a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis, retroperitoneal fibrosis, etc. Although all clinicians in every field of medicine may encounter this new disease, a unifying diagnostic criterion has not been established. In 2011, the Japanese IgG4 team, organized by the Ministry of He...
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Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a chronic inflammatory disease that simultaneously or consecutively involves multiple organs of the body. It is characterized by elevated serum IgG4 levels and massive infiltration of IgG4+ plasma cells in the damaged tissues. IgG4-related autoimmune hepatitis (IgG4-AIH) and IgG4-hepatopathy are relatively new entities that have been proposed as a phenotype of IgG4-RD in the liver. Immunoglobulin G4-AIH is defined as a disorder with serologic...
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