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Consensus statement on the pathology of IgG4-related disease.

Published on Sep 1, 2012in Modern Pathology 6.37
· DOI :10.1038/modpathol.2012.72
Vikram V. Deshpande85
Estimated H-index: 85
(Harvard University),
Yoh Zen62
Estimated H-index: 62
(University of Cambridge)
+ 38 AuthorsJohn H. Stone67
Estimated H-index: 67
(Harvard University)
Cite
Abstract
IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; and-often but not always-elevated serum IgG4 concentrations. An international symposium on IgG4-related disease was held in Boston, MA, on 4-7 October 2011. The organizing committee comprising 35 IgG4-related disease experts from Japan, Korea, Hong Kong, the United Kingdom, Germany, Italy, Holland, Canada, and the United States, including the clinicians, pathologists, radiologists, and basic scientists. This group represents broad subspecialty expertise in pathology, rheumatology, gastroenterology, allergy, immunology, nephrology, pulmonary medicine, oncology, ophthalmology, and surgery. The histopathology of IgG4-related disease was a specific focus of the international symposium. The primary purpose of this statement is to provide practicing pathologists with a set of guidelines for the diagnosis of IgG4-related disease. The diagnosis of IgG4-related disease rests on the combined presence of the characteristic histopathological appearance and increased numbers of IgG4? plasma cells. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. We propose a terminology scheme for the diagnosis of IgG4-related disease that is based primarily on the morphological appearance on biopsy. Tissue IgG4 counts and IgG4:IgG ratios are secondary in importance. The guidelines proposed in this statement do not supplant careful clinicopathological correlation and sound clinical judgment. As the spectrum of this disease continues to expand, we advocate the use of strict criteria for accepting newly proposed entities or sites as components of the IgG4-related disease spectrum.
  • References (84)
  • Citations (1048)
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References84
Newest
Published on Mar 1, 2012in Journal of Surgical Research 1.87
Seth B. Krantz12
Estimated H-index: 12
(NU: Northwestern University),
Mario Anthonio Shields11
Estimated H-index: 11
(NU: Northwestern University)
+ 2 AuthorsDavid J. Bentrem49
Estimated H-index: 49
(NU: Northwestern University)
Pancreatic adenocarcinoma remains among the most lethal of human malignancies. Overall 5-y survival is less than 5%, and only 20% of patients presenting with localized disease amenable to surgical resection. Even in patients who undergo resection, long-term survival remains extremely poor. A major contributor to the aggressiveness of multiple cancers, and pancreatic cancer in particular, is the process of epithelial-to-mesenchymal transition (EMT). This review highlights the growing evidence of ...
Published on Feb 9, 2012in The New England Journal of Medicine 70.67
John H. Stone67
Estimated H-index: 67
,
Yoh Zen62
Estimated H-index: 62
,
Deshpande5
Estimated H-index: 5
Published on Feb 1, 2012in Modern Rheumatology 1.97
Hisanori Umehara23
Estimated H-index: 23
(Kanazawa Medical University),
Kazuichi Okazaki62
Estimated H-index: 62
(Kansai Medical University)
+ 23 AuthorsShigeyuki Kawa50
Estimated H-index: 50
(Shinshu University)
Background IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4+ plasma cells. Although IgG4-RD is not rare and is clinically important, its clinical diagnostic criteria have not been established. Comprehensive diagnostic criteria for IgG4-RD, including the involvement of various organs, are intended for the practical use of general physicians and nonspecialists.
Published on Feb 1, 2012in Modern Rheumatology 1.97
Hisanori Umehara23
Estimated H-index: 23
(Kanazawa Medical University),
Kazuichi Okazaki62
Estimated H-index: 62
(Kansai Medical University)
+ 19 AuthorsYoshiya Tanaka51
Estimated H-index: 51
IgG4-related disease (IgG4RD) is a novel clinical disease entity characterized by elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4RD may be present in a certain proportion of patients with a wide variety of diseases, including Mikulicz’s disease, autoimmune pancreatitis, hypophysitis, Riedel thyroiditis, interstitial pneumonitis, interstitial nephritis, prostatitis, lymphadenopathy, retroperitoneal fibrosis, inflammatory aortic aneurysm...
Published on Jan 1, 2012in Journal of The American College of Surgeons 4.45
Skye C. Mayo23
Estimated H-index: 23
(Johns Hopkins University),
Marta M. Gilson15
Estimated H-index: 15
(Johns Hopkins University)
+ 7 AuthorsTimothy M. Pawlik80
Estimated H-index: 80
(Johns Hopkins University)
Background Surgical resection remains the only potentially curative option for patients with pancreatic adenocarcinoma (PAC). Advances in surgical technique and perioperative care have reduced perioperative mortality; however, temporal trends in perioperative morbidity and the use of adjuvant therapy on a population basis remain ill-defined. Study Design Using Surveillance, Epidemiology, and End Results–Medicare data, 2,461 patients with resected PAC were identified from 1991 to 2005. We examine...
Published on Oct 1, 2011in Clinical and Experimental Nephrology 1.97
Mitsuhiro Kawano29
Estimated H-index: 29
(Kanazawa University),
Takako Saeki24
Estimated H-index: 24
+ 12 AuthorsHiroki Takahashi45
Estimated H-index: 45
(Sapporo Medical University)
Background IgG4-related disease has attracted wide attention recently. It is characterized by a high level of serum IgG4 and dense infiltration of IgG4-positive plasma cells into multiple organs, with the kidney being one representative target. Although several sets of diagnostic criteria for autoimmune pancreatitis (AIP) are available and renal lesion is recognized as an extra-pancreatic manifestation of AIP, it is difficult to differentiate IgG4-related tubulointerstitial nephritis (TIN) witho...
Published on Oct 1, 2011in The American Journal of Medicine 4.76
Arezou Khosroshahi25
Estimated H-index: 25
,
Mollie D. Carruthers2
Estimated H-index: 2
+ 3 AuthorsJohn H. Stone67
Estimated H-index: 67
Published on Jul 1, 2011in Journal of The American Society of Nephrology 8.55
Yassaman Raissian5
Estimated H-index: 5
(Mayo Clinic),
Samih H. Nasr45
Estimated H-index: 45
+ 10 AuthorsSuresh T. Chari73
Estimated H-index: 73
IgG4-related systemic disease is an autoimmune disease that was first recognized in the pancreas but also affects other organs. This disease may manifest as tubulointerstitial nephritis (IgG4-TIN), but its clinicopathologic features in the kidney are not well described. Of the 35 patients with IgG4-TIN whose renal tissue specimens we examined, 27 (77%) had acute or progressive chronic renal failure, 29 (83%) had involvement of other organ systems, and 18 of 23 (78%) had radiographic abnormalitie...
Published on May 1, 2011in Annals of Surgical Oncology 3.68
William F. Regine44
Estimated H-index: 44
(UMD: University of Maryland, College Park),
Kathryn Winter47
Estimated H-index: 47
+ 7 AuthorsChristopher G. Willett78
Estimated H-index: 78
(Duke University)
Background The impact of the addition of gemcitabine to 5-fluorouracil (5-FU) chemoradiation (CRT) on 5-year overall survival (OS) in resected pancreatic adenocarcinoma are presented with updated results of a phase III trial.
Published on May 1, 2011in Journal of Hepato-biliary-pancreatic Sciences 2.35
Kensuke Kubota29
Estimated H-index: 29
(YCU: Yokohama City University),
Shingo Kato16
Estimated H-index: 16
(YCU: Yokohama City University)
+ 11 AuthorsNoritoshi Kobayashi22
Estimated H-index: 22
(YCU: Yokohama City University)
Background and study aims Expression of the forkhead/winged helix family of transcription factor P3+ regulatory T cells (FOXP3+ Treg), a master gene of regulatory T cells (Treg) is observed in patients with autoimmune pancreatitis (AIP). We investigated the usefulness of detection of FOXP3+ Treg in the main duodenal papilla for differential diagnosis between AIP and pancreatic cancer (Pca).
Cited By1048
Newest
Published on Jan 24, 2019in Scientific Reports 4.01
Yuka Gion8
Estimated H-index: 8
(RMIT: RMIT University),
Mai Takeuchi (Kurume University)+ 6 AuthorsYasuharu Sato26
Estimated H-index: 26
(RMIT: RMIT University)
Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a systemic disorder involving benign mass formation due to fibrosis and intense lymphoplasmacytosis; the chronic inflammation associated with the disease might also contribute to oncogenesis. Activation-induced cytidine deaminase (AID), normally expressed in germinal centre activated B-cells, is an enzyme that edits DNA/RNA and induces somatic hypermutation and Ig class switching. AID expression is strictly controlled under physiological condit...
Published in Journal of Neuroimmunology 2.83
Jingfang Lin (Sichuan University), Linmao Zheng (Sichuan University)+ -3 AuthorsZhen Hong (Sichuan University)
Abstract IgG4-related disease (IgG4-RD) is an immune-mediated condition that affects multiple organ systems with tumorlike swelling. Here, for the first time, we report a rare case of IgG4-RD that involves the cerebral parenchyma, intramedullary spinal cord and lymph nodes. The diagnosis of IgG4-RD was made based on histological features, multi organ involvement and high serum IgG4 levels. The patient responded well to glucocorticoid treatment. Our case broadens the phenotypic presentation for t...
Published on Jun 1, 2019in World Neurosurgery 1.72
Stephen J. Slade (Virginia Mason Medical Center), Erin M. Bauer (Virginia Mason Medical Center)+ 1 AuthorsAmish J. Dave (Virginia Mason Medical Center)
Abstract Background Back pain is a leading reason patients seek medical attention. While musculoskeletal causes are common, rarer etiologies can present. Case Description A 50-year-old male presented with 2 months of isolated upper back pain initially suspected to be secondary to overuse muscular strain. Over the next 3 months, his pain worsened, and he developed lower extremity dysesthesias and subjective weakness despite a normal neurological exam. Non-revealing laboratory workup included norm...
Published on May 31, 2018in Head and Neck Pathology
Zitong Zhao (SGH: Singapore General Hospital), Lee Yu Jin (SGH: Singapore General Hospital)+ 2 AuthorsKok Hing Lim2
Estimated H-index: 2
(SGH: Singapore General Hospital)
IgG4-related disease of the thyroid gland is a recently recognized subtype of thyroiditis, often with rapid progression requiring surgical treatment. It is considered as a spectrum of disease varying from early IgG4-related Hashimoto’s thyroiditis (HT) pattern to late fibrosing HT or Riedel’s thyroiditis patterns. Here, we report a 47-year-old Malay woman presenting with progressively painless neck swelling over 3 years and subclinical hypothyroidism. Computed tomography (CT) scan revealed diffu...
Published on Sep 1, 2019in Urology case reports
Jerasit Surintrspanont (King Chulalongkorn Memorial Hospital), Anapat Sanpawat (King Chulalongkorn Memorial Hospital)+ 1 AuthorsPiyada Sitthideatphaiboon (King Chulalongkorn Memorial Hospital)
Abstract Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized systemic condition characterized by particular clinical, serologic, and pathologic features that are consistent across a wide range of organ systems. Herein, we present a rare case of IgG4-RD presenting as multiple inflammatory pseudotumors involving the kidney and other organs involvement mimicking urothelial cell carcinoma with liver, lymph node and lung metastasis. The final diagnosis was made based on characte...
Published on Mar 25, 2019in Virchows Archiv 2.87
Silvia Uccella22
Estimated H-index: 22
(University of Insubria),
Cristina Amaglio (University of Insubria)+ 7 AuthorsStefano La Rosa30
Estimated H-index: 30
(UNIL: University of Lausanne)
IgG4-related hypophysitis (IgG4-RH) is a rare disease, which can occur singularly or as manifestation of a systemic IgG4-related disease (IgG4-RD). Less than one hundred cases have been reported in the literature, very few of which were histopathologically documented. We analyzed the clinical, radiological, and histopathological features of two cases of IgG4-RH, the former observed in a 66-year-old man in the context of an IgG4-RD, and the latter affecting a 21-year-old woman, as an isolated les...
Published in Digestive Diseases and Sciences 2.94
Kazuichi Okazaki62
Estimated H-index: 62
(Kansai Medical University)
Autoimmune pancreatitis (AIP) is an entity that has been recognized since 1961. Prior to the discovery of elevated serum IgG4 as a useful biomarker for its diagnosis, Dr. Yoshida in 1995 first described the entity of AIP, which in retrospect closely resembles the current concept of type 1 AIP. Since the discovery of IgG4 as a biomarker (the IgG4-era), a novel concept of IgG4-related disease (IgG4-RD) has been accepted as being comprised of two subtypes of AIP: type 1 defined as the pancreatic ma...
Published on Jun 22, 2019in Clinical and Experimental Nephrology 1.97
Diego Moriconi3
Estimated H-index: 3
(UniPi: University of Pisa),
Domenico Giannese2
Estimated H-index: 2
+ 6 AuthorsMaria Francesca Egidi5
Estimated H-index: 5
Background Retroperitoneal fibrosis (RF) is a rare disease of unclear etiology characterized by the presence of fibroinflammatory tissue in the retroperitoneal space, which can entrap and obstruct retroperitoneal structures, notably the ureters. The disease responds well to steroid therapy, but tends to recur even after years. The aim of our study was to evaluate the long-term renal outcome of patients affected by idiopathic retroperitoneal fibrosis looking for predictive risk factors for recurr...
Published on Jun 15, 2019in European Archives of Oto-rhino-laryngology 1.75
Gwanghui Ryu (SCH: Soonchunhyang University), Hyun-Jin Cho1
Estimated H-index: 1
(Gyeongsang National University)
+ 5 AuthorsHun-Jong Dhong16
Estimated H-index: 16
(SMC: Samsung Medical Center)
Introduction Inflammatory pseudotumor (IPT) in the sinonasal cavity and skull base region is benign non-neoplastic inflammatory process. However, IPT can mimic malignant tumor or infectious disease and there are difficulties in confirmation of diagnosis. The aim of study is to evaluate the clinical significance of immunoglobulin G4 (IgG4) in IPT in terms of steroid response and differential diagnosis with other skull base infiltrative lesions.
Published in Frontiers in Immunology 4.72
Debby van den Elshout-den Uyl (UU: Utrecht University), Clothaire P.E. Spoto1
Estimated H-index: 1
(UU: Utrecht University)
+ -3 AuthorsRoos J. Leguit7
Estimated H-index: 7
(UU: Utrecht University)
IgG4 related disease is a fibro-inflammatory disorder characterized by swelling of tissues and affected organs accompanied by the development of scar tissue (fibrosis) and infiltration by IgG4 positive plasma cells. Almost any organ can be affected, but so far no reports of bone marrow involvement have been published. Here we present a case of a 76-year-old male with IgG4 related disease presenting primarily with vertebral bone marrow lesions. Histopathology showed the typical features of storif...