IgG4-Related Diseases-Continues To Be a Cancer Mimicker.

Published on Jan 9, 2020in Cureus
· DOI :10.7759/CUREUS.6610
Soujanya Sodavarapu1
Estimated H-index: 1
(San Joaquin General Hospital),
Gurinder S Ghotra1
Estimated H-index: 1
(San Joaquin General Hospital)
+ 2 AuthorsAmandeep Singh Gill2
Estimated H-index: 2
(San Joaquin General Hospital)
Immunoglobulin G4-related disease (IgG4-RD) is a chronic multisystem immune-mediated disease. Histologically, it presents as infiltration with IgG4-secreting plasma cells affecting many organs such as the pancreas, lacrimal glands, salivary glands, kidneys, and arteries, resulting in chronic fibrosis and scarring within the tissue which over time forms into a mass. It is a slow-growing process that usually remains indolent until it causes a mass effect or tissue infiltration. Our patient was found to have a pancreatic mass on imaging concerning pancreatic neoplastic lesions. He subsequently underwent biopsy for histology/pathology and IgG4 levels, which led to our diagnosis of IgG4-RD. Imaging, blood tests, and, most importantly, histopathological features of the involved organ are important in determining the diagnosis. This is important as a treatment plan will be based on the given diagnosis, and patients with IgG4-RD respond very well to systemic steroid therapy.
: A 78-year-old woman was admitted to our hospital with a pancreatic tumor, incidentally discovered in an abdominal ultrasound exam. She was asymptomatic and without any previous personal pathological condition. The computed tomography (CT) and the magnetic resonance imaging (MRI) scan showed a mass lesion of 4 cm in diameter, located in the pancreatic body, conditioning the invasion of the splenic vein. The patient was admitted to surgery. During the laparotomy, we found a tumoral lesion highly...
3 CitationsSource
#1Atsushi Kanno (Tohoku University)H-Index: 39
#1Atsushi Kanno (Tohoku University)H-Index: 18
Last. Tooru Shimosegawa (Tohoku University)H-Index: 93
view all 17 authors...
Background and Aims Histopathologic examination is critical for diagnosing autoimmune pancreatitis (AIP). However, specimens obtained using EUS-guided FNA (EUS-FNA) are not recommended for histopathologic diagnosis because of inadequate sample size volume. We evaluated EUS-FNA efficacy for AIP diagnosis using a 22G needle. Methods Seventy-eight patients exhibiting the imaging characteristics indicative of AIP in the pancreatic parenchyma and pancreatic duct underwent EUS-FNA with a 22G needle at...
57 CitationsSource
#1Cory A. Perugino (Harvard University)H-Index: 14
#2John H. Stone (Harvard University)H-Index: 94
IgG4-related disease (IgG4-RD) is capable of causing great morbidity and even mortality if the condition remains undiagnosed or poorly treated, yet is typically a treatment-responsive disorder. Glucocorticoids have not been studied rigorously and practices with regard to dosing and duration of treatment remain largely empiric. In addition, IgG4-RD patients are often particularly susceptible to and intolerant of the deleterious effects of glucocorticoid therapy. B cell depletion with anti-CD20 mo...
20 CitationsSource
#1Terumi Kamisawa (Tokyo Metropolitan Komagome Hospital)H-Index: 77
#2Kazuichi Okazaki (Kansai Medical University)H-Index: 83
It is critical to differentiate IgG4-related disease (IgG4-RD) from malignant tumor and similar disease of the affected organ to apply appropriate therapy and avoid unnecessary surgery. IgG4-RD is diagnosed on combination of typical radiological findings; elevation of serum IgG4 levels; histopathological findings of abundant infiltration of IgG4-positive plasma cells and lymphocytes, storiform fibrosis , and obliterative phlebitis ; association with other IgG4-related diseases; and response to s...
27 CitationsSource
#1Arezou Khosroshahi (Emory University)H-Index: 31
#2Zachary S. Wallace (Harvard University)H-Index: 27
Last. John H. Stone (Harvard University)H-Index: 94
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A. Khosroshahi, Z. S. Wallace, J. L. Crowe, T. Akamizu, A. Azumi, M. N. Carruthers, S. T. Chari, E. Della-Torre, L. Frulloni, H. Goto, P. A. Hart, T. Kamisawa, S. Kawa, M. Kawano, M. H. Kim, Y. Kodama, K. Kubota, M. M. Lerch, M. L€ ohr, Y. Masaki, S. Matsui, T. Mimori, S. Nakamura, T. Nakazawa, H. Ohara, K. Okazaki, J. H. Ryu, T. Saeki, N. Schleinitz, A. Shimatsu, T. Shimosegawa, H. Takahashi, M. Takahira, A. Tanaka, M. Topazian, H. Umehara, G. J. Webster, T. E. Witzig, M. Yamamoto, W. Zhang, T....
445 CitationsSource
#1Dai Inoue (Kanazawa University)H-Index: 5
#1Dai Inoue (Kanazawa University)H-Index: 20
Last. Yoh ZenH-Index: 86
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AbstractImmunoglobulin G4-related disease (IgG4-RD) is a recently discovered systemic condition, in which various organ manifestations are linked by a similar histological appearance. Our knowledge of this condition is still fragmented, as most studies have examined only a few dozen patients or focu
204 CitationsSource
#1Minerva Lazos Ochoa (HGM: Hospital General de México)H-Index: 1
Last. Raúl Romero FeregrinoH-Index: 4
view all 4 authors...
IgG4-related disease (IgG4RD) is a chronic recurring fibro-inflammatory pathology that is considered to be of autoimmune origin. Histopathology is considered to be the gold standard method for diagnosis. IgG4RD affects multiple organs. IgG4RD was first identified in the pancreas and was called autoimmune pancreatitis (AIP). During the following years, the disease spectrum was expanded and it was realised that the extrapancreatic lesions can precede, coexist or appear after the diagnosis of AIP. ...
7 CitationsSource
#1John H. Stone (Harvard University)H-Index: 94
#2Arezou Khosroshahi (Harvard University)H-Index: 31
Last. Suresh T. Chari (Mayo Clinic)H-Index: 103
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John H. Stone, Arezou Khosroshahi, Vikram Deshpande, John K. C. Chan, J. Godfrey Heathcote, Rob Aalberse, Atsushi Azumi, Donald B. Bloch, William R. Brugge, Mollie N. Carruthers, Wah Cheuk, Lynn Cornell, Carlos Fernandez-Del Castillo, Judith A. Ferry, David Forcione, Gunter Kloppel, Daniel L. Hamilos, Terumi Kamisawa, Satomi Kasashima, Shigeyuki Kawa, Mitsuhiro Kawano, Yasufumi Masaki, Kenji Notohara, Kazuichi Okazaki, Ji Kon Ryu, Takako Saeki, Dushyant Sahani, Yasuharu Sato, Thomas Smyrk, James...
447 CitationsSource
#1Vikram Deshpande (Harvard University)H-Index: 121
#2Yoh Zen (University of Cambridge)H-Index: 86
Last. John H. Stone (Harvard University)H-Index: 94
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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, Ital...
1,443 CitationsSource
#1Kazuichi Okazaki (Kansai Medical University)H-Index: 83
#2Shigeyuki Kawa (Shinshu University)H-Index: 67
Last. Makoto Otsuki (University of Occupational and Environmental Health Japan)H-Index: 9
view all 18 authors...
In 1961, Sarles et al.1 asked the following question regarding the particular cases of pancreatitis with hypergammaglobulinemia: “Chronic inflammatory sclerosis of the pancreas—an autoimmune pancreatic disease?” As similar cases were rarely observed, a relationship between such pancreatitis and autoimmunity was viewed skeptically during the following several decades. In 1992, Toki et al.2 have reported 4 cases with unusual diffuse irregular narrowing of the main pancreatic duct and diffuse enlar...
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