IgG4-related disease: Changing epidemiology and new thoughts on a multisystem disease.

Published on Jan 1, 2021
· DOI :10.1016/J.JTAUTO.2020.100074
Annarosa Floreani59
Estimated H-index: 59
(UNIPD: University of Padua),
Kazuichi Okazaki85
Estimated H-index: 85
(Kansai Medical University)
+ 1 AuthorsM. Eric Gershwin139
Estimated H-index: 139
(UC Davis: University of California, Davis)
IgG4-related disease (IgG4-RD) represents an immune-mediated fibroinflammatory condition with peculiar histopathologic changes that can affect various organs. In 2012 its unified nomenclature was published, which allows to abandon other synonymous names. Up to now, only little is known about its epidemiology around the world. However, although it is generally considered a rare condition, the number of patients with IgG4-RD is increasing enormously. Likewise, the annual number of publications on this subject has increased progressively. The spectrum of clinical manifestations in IgG4-RD is highly variable, depending on the severity of the disease as well as the presence of organ(s) involvement. This review gives an overview on changing epidemiology of IgG4-RD focusing the attention on the large cohorts of patients published in the literature.
#1Atsushi Tanaka (Teikyo University)H-Index: 33
#2Mitsuru MoriH-Index: 36
Last. Kazuichi Okazaki (Kansai Medical University)H-Index: 85
view all 10 authors...
BACKGROUND/PURPOSE Epidemiological data on immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are scarce. METHOD We randomly selected healthcare facilities from a list of all facilities in Japan. The selection rate was determined according to a stratification based on the facility characteristics and scale. We sent questionnaires to the selected facilities enquiring about the number and sex of patients with IgG4-SC in 2018. Among responding facilities, we further inquired about the clini...
4 CitationsSource
#1Atsushi Masamune (Tohoku University)H-Index: 67
#2Kazuhiro Kikuta (Tohoku University)H-Index: 32
Last. Kazuichi Okazaki (Kansai Medical University)H-Index: 85
view all 7 authors...
Background To further clarify the clinico-epidemiological features of autoimmune pancreatitis (AIP) in Japan, we conducted the fourth nationwide epidemiological survey.
21 CitationsSource
#1Corrado Campochiaro (UniSR: Vita-Salute San Raffaele University)H-Index: 23
#2Emanuel Della-Torre (UniSR: Vita-Salute San Raffaele University)H-Index: 27
Last. Lorenzo Dagna (UniSR: Vita-Salute San Raffaele University)H-Index: 37
view all 10 authors...
Abstract Background IgG4-Related Disease (IgG4-RD) promptly responds to glucocorticoids but relapses in most patients. Rituximab (RTX) represents a promising strategy to avoid IgG4-RD flares but its administration for maintaining disease remission has never been assessed in terms of optimal timing of infusion, dosage, and duration of treatment. In the present study we aimed to evaluate the efficacy and safety of RTX for maintenance of IgG4-RD remission. Methods Fourteen patients with IgG4-RD wer...
15 CitationsSource
#1Zachary S. Wallace (Harvard University)H-Index: 27
#2Ray NadenH-Index: 7
Last. John H. Stone (Harvard University)H-Index: 94
view all 24 authors...
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
#1Pegah KhoshpouriH-Index: 9
Last. Ihab R. KamelH-Index: 73
view all 10 authors...
Recognizing the multimodality imaging features of the biliary system and liver parenchyma in primary sclerosing cholangitis can help the radiologist provide an accurate diagnosis, identify a differ...
9 CitationsSource
#1Chikashi Terao (Kyoto University)H-Index: 32
#2Masao Ota (Shinshu University)H-Index: 1
Last. Chiharu KawanamiH-Index: 21
view all 69 authors...
Summary Background IgG4-related disease is a newly recognised immunopathological entity that includes autoimmune pancreatitis, IgG4-related sialadenitis, and IgG4-related kidney disease. To understand the genetic landscape of IgG4-related disease, we did a genome-wide association study. Methods We did a genome-wide association study of Japanese individuals, initially screening 857 patients with IgG4-related disease at 50 Japanese research institutions and DNA samples from 2082 healthy control pa...
10 CitationsSource
#1Yuri Takagi (YCU: Yokohama City University)H-Index: 2
#2Kensuke Kubota (YCU: Yokohama City University)H-Index: 32
Last. Atsushi Nakajima (YCU: Yokohama City University)H-Index: 82
view all 19 authors...
BACKGROUND AND AIM: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) presents as isolated proximal-type sclerosing cholangitis (i-SC). The present study sought to clarify the imaging differences between i-SC and Klatskin tumor. Differences between i-SC and IgG4-SC associated with autoimmune pancreatitis (AIP-SC) were also studied. METHODS: Differentiating factors between i-SC and Klatskin tumor were studied. Serum IgG4 level, CA19-9 level, computed tomography (CT) findings, cholangiogr...
4 CitationsSource
#1Nicolò de Pretis (University of Verona)H-Index: 8
#2Filippo Vieceli (University of Verona)H-Index: 2
Last. Luca Frulloni (University of Verona)H-Index: 58
view all 6 authors...
Abstract Background Autoimmune pancreatitis (AIP) is a well-recognized fibroinflammatory disease of the pancreas. Despite the significant number of studies published on AIP type 1 and 2, no studies have been focused on AIP type not otherwise specified (NOS) and therefore very little is known about clinical features and long-term outcomes of these patients. The aim of this study was to investigate clinical and radiological features of AIP type NOS-patients. Methods Patients classified as AIP type...
2 CitationsSource
#1John H. Newman (Vandy: Vanderbilt University)H-Index: 69
#2Shaver A (Vandy: Vanderbilt University)H-Index: 1
Last. John A. Phillips (Vandy: Vanderbilt University)H-Index: 79
view all 22 authors...
BACKGROUND: Family screening of a 48-year-old male with recently diagnosed IgG4-related disease (IgG4-RD) revealed unanticipated elevations in plasma IgG4 in his two healthy teenaged sons. METHODS: We performed gene sequencing, immune cell studies, HLA typing, and analyses of circulating cytotoxic CD4+ T lymphocytes and plasmablasts to seek clues to pathogenesis. DNA from a separate cohort of 99 patients with known IgG4-RD was also sequenced for the presence of genetic variants in a specific gen...
3 CitationsSource
#1Kosuke Minaga (Kindai University)H-Index: 18
#2Tomohiro Watanabe (Kindai University)H-Index: 35
Last. Masatoshi Kudo (Kindai University)H-Index: 116
view all 4 authors...
: IgG4-related disease (IgG4-RD) is a chronic-fibroinflammatory disorder affecting a wide range of organs. Elevation of serum IgG4 concentrations and abundant infiltration of IgG4-expressing plasma cells are key diagnostic features of this autoimmune disease. Although common organ involvement of IgG4-RD includes the salivary glands, pancreas, and bile duct, hepatic involvement is less well established. Recently, five studies identified a subtype of autoimmune hepatitis (AIH), called IgG4-associa...
11 CitationsSource
Cited By0
#1Atsushi Tanaka (Teikyo University)H-Index: 33
#2Kenji NotoharaH-Index: 41
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...