IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry.

Published on Jan 1, 2012in Medicine1.552
· DOI :10.1097/MD.0B013E3182433D77
Mikael Ebbo23
Estimated H-index: 23
,
Laurent Daniel31
Estimated H-index: 31
+ 33 AuthorsNicolas Schleinitz36
Estimated H-index: 36
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Abstract
AbstractIgG4-related systemic disease is now recognized as a systemic disease that may affect various organs. The diagnosis is usually made in patients who present with elevated IgG4 in serum and tissue infiltration of diseased organs by numerous IgG4+ plasma cells, in the absence of validated diagn
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#1Arezou Khosroshahi (Harvard University)H-Index: 30
Last. John H. StoneH-Index: 76
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AbstractPatients with IgG4-related disease (IgG4-RD) typically have elevated serum concentrations of IgG4 and share histopathologic features that are similar across affected organ(s). IgG4-RD patients frequently require prolonged treatment with glucocorticoids and are often unable to taper these med
346 CitationsSource
#1Kazuichi Okazaki (Kansai Medical University)H-Index: 68
#2Kazushige Uchida (Kansai Medical University)H-Index: 29
Last. Makoto Takaoka (Kansai Medical University)H-Index: 24
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Recent studies have suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 AIP, related to IgG4 (lymphoplasmacytic sclerosing pancreatitis); and type 2 AIP, related to a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis). Compared with type 2 AIP, the clinicopathological features of type 1 AIP, with increased serum IgG4/IgE levels, abundant infiltration of IgG4 + plasmacytes and lymphocytes, autoantibodies, and steroid responsiveness, are more...
223 CitationsSource
Purpose of review To review studies that have examined underlying genetic and immunological aspects of IgG4-related disease. Recent findings Genetic studies have suggested that several human leukocyte antigen (HLA) and non-HLA haplotypes/genotypes are associated with susceptibility to IgG4-related disease or to disease relapse after steroid therapy. Among several autoantibodies identified so far, autoantibodies against lactoferrin and carbonic anhydrase II are most frequently detected in serum o...
108 CitationsSource
#1Satomi KasashimaH-Index: 21
#2Yoh Zen (University of Cambridge)H-Index: 69
Last. Yasuni Nakanuma (Kanazawa University)H-Index: 87
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Objective Immunoglobulin G4-related sclerosing disease (IgG4-SD) has recently been reported to occur in the cardiovascular system and manifest as inflammatory abdominal aortic aneurysm. Thoracic aortic lesions are often associated with aortitis in several divergent etiologies. Thus, this study was performed to review thoracic aortic lesions from the aspect of IgG4-SD and to elucidate the clinicopathologic characteristics of this subgroup in the thoracic aorta. Methods The study comprised 125 pat...
118 CitationsSource
#1Takako SaekiH-Index: 29
#2Shinichi Nishi (Niigata University)H-Index: 38
Last. Ichiei Narita (Niigata University)H-Index: 39
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IgG4-related disease is a recently recognized multi-organ disorder characterized by high levels of serum IgG4 and dense infiltration of IgG4-positive cells into several organs. Although the pancreas was the first organ recognized to be affected by IgG4-related disorder in the syndrome of autoimmune pancreatitis, we present here clinico-pathological features of 23 patients diagnosed as having renal parenchymal lesions. These injuries were associated with a high level of serum IgG4 and abundant Ig...
394 CitationsSource
#1Reiko Akitake (Kyoto University)H-Index: 6
#2Tomohiro Watanabe (Kyoto University)H-Index: 35
Last. Tsutomu ChibaH-Index: 81
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We report a case of immunoglobulin G4 (IgG4)-related sclerosing disease involving the pancreas, liver and salivary glands. Massive infiltration of IgG4-expressing plasma cells was seen in the liver and submandibular lymph nodes. Interestingly, accumulation of IgG4-expressing plasma cells was also seen in the colon and terminal ileum. Peripheral blood mononuclear cells (PBMCs) isolated from this patient exhibited enhanced production of IgG4 and interleukin-10 upon stimulation with Toll-like recep...
105 CitationsSource
#1John H. Stone (Harvard University)H-Index: 76
#2Arezou Khosroshahi (Harvard University)H-Index: 30
Last. James R. Stone (Harvard University)H-Index: 52
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Objective IgG4-related systemic disease, a disorder recognized only recently, can cause lymphoplasmacytic inflammation in the thoracic aorta. The percentage of cases caused by IgG4-related systemic disease is not known. We aimed to determine the percentage of noninfectious thoracic aortitis cases that are associated with IgG4-related systemic disease and to establish pathologic criteria for identifying involvement of the thoracic aorta by this disorder. Methods We searched our Pathology Service ...
164 CitationsSource
#1John H. Stone (Harvard University)H-Index: 76
#2Arezou Khosroshahi (Harvard University)H-Index: 30
Last. James R. Stone (Harvard University)H-Index: 52
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We describe herein a patient who developed a dissection of the ascending aorta in the setting of IgG4-related systemic disease, linking IgG4-related systemic disease with a newly-recognized subset of noninfectious aortitis. At the time of aortic surgery, a transmural lymphoplasmacytic infiltrate was detected in the patient's aorta, with a principal focus of inflammation within the media. Immunohistochemical studies demonstrated that >50% of the plasma cells in the lesion stained for IgG4. By in ...
223 CitationsSource
#1Yasufumi Masaki (Kanazawa Medical University)H-Index: 28
#2Lingli Dong (Kanazawa Medical University)H-Index: 16
Last. Hisanori Umehara (Kanazawa Medical University)H-Index: 43
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Background: Mikulicz’s disease (MD) has been considered as one manifestation of Sjogren’s syndrome (SS). Recently, it has also been considered as an IgG 4 -related disorder. Objective: To determine the differences between IgG 4 -related disorders including MD and SS. Methods: A study was undertaken to investigate patients with MD and IgG 4 -related disorders registered in Japan and to set up provisional criteria for the new clinical entity IgG 4 -positive multiorgan lymphoproliferative syndrome ...
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#1Rob C. Aalberse (UvA: University of Amsterdam)H-Index: 53
#2S. O. Stapel (UvA: University of Amsterdam)H-Index: 2
Last. Theo Rispens (UvA: University of Amsterdam)H-Index: 42
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Summary Despite its well-known association with IgE-mediated allergy, IgG4 antibodies still have several poorly understood characteristics. IgG4 is a very dynamic antibody: the antibody is involved in a continuous process of half-molecules (i.e. a heavy and attached light-chain) exchange. This process, also referred to as ‘Fab-arm exchange’, results usually in asymmetric antibodies with two different antigen-combining sites. While these antibodies are hetero- bivalent, they will behave as monova...
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#1Marco Lanzillotta (UniSR: Vita-Salute San Raffaele University)H-Index: 14
#2Andreu Fernández-Codina (UWO: University of Western Ontario)H-Index: 9
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INTRODUCTION Awareness of IgG4-related disease (IgG4-RD) is increasing worldwide and specialists are now familiar with most of its clinical manifestations and mimickers. IgG4-RD promptly responds to glucocorticoids and repeated courses are typically used to induce and maintain remission because the disease relapses in most patients. If left untreated it can lead to organ dysfunction, organ failure and death. Advancement in our understanding of IgG4-RD pathogenesis is leading to the identificatio...
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#1Chien‐Ting Hsu (NTU: National Taiwan University)
#2Yung‐Ming Jeng (NTU: National Taiwan University)
Last. Jia-Feng Wu (NTU: National Taiwan University)H-Index: 22
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#1Yanying Liu (PKU: Peking University)H-Index: 12
#2Lijuan Zhu (Zhengzhou University)H-Index: 1
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#2Keishi Mizuguchi (Kanazawa University)H-Index: 3
Last. Mitsuhiro KawanoH-Index: 20
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BACKGROUND IgG4-related kidney disease causes renal impairment of unknown pathogenesis that may progress to kidney failure. Although ectopic germinal centers contribute to the pathogenesis of the head and neck lesions of IgG4-related disease, the presence of tertiary lymphoid tissue (TLT) containing germinal centers in IgG4-RKD has rarely been reported. CASE PRESENTATION We report a 72-year-old Japanese man who had IgG4-related tubulointerstitial nephritis (TIN) with TLT formation incidentally d...
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#1Andreu Fernández-Codina (UWO: University of Western Ontario)H-Index: 9
#2Blanca Pinilla (Hospital General Universitario Gregorio Marañón)H-Index: 8
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OBJECTIVES Several IgG4-related disease (IgG4-RD) phenotypes have been proposed and the first set of classification criteria have been recently created. Our objectives were to assess the phenotype distribution and the performance of the classification criteria in Spanish patients as genetic and geographical differences may exist. METHODS We performed a cross-sectional multicentre study (Registro Espanol de Enfermedad Relacionada con la IgG4, REERIGG4) with nine participating centres from Spain. ...
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#1Su Jin Kim (PNU: Pusan National University)H-Index: 2
#2Seung Uk Lee (Kosin University)H-Index: 5
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#1Zaizhu Zhang (Peking Union Medical College Hospital)
#2Wenmin Guan (Peking Union Medical College Hospital)
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OBJECTIVES To retrospectively evaluate CT and magnetic resonance (MR) imaging thoracic paravertebral findings at baseline and follow-up in patients with IgG4-related disease. METHODS The study consisted of 36 patients with IgG4-related disease involving thoracic paravertebral regions (32 men and four women; mean age, 58 years; range, 25-78 years). A total of 36 patients underwent CT or MR imaging at baseline; 20 patients underwent follow-up. CT and MR images were reviewed and analysed in consens...
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#1Renqin Lin (Fujian Medical University)H-Index: 2
#2Shenglin Wang (Fujian Medical University)H-Index: 2
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