A new clinicopathological entity of IgG4-related autoimmune disease

Published on Oct 1, 2003in Journal of Gastroenterology6.132
· DOI :10.1007/S00535-003-1175-Y
Terumi Kamisawa60
Estimated H-index: 60
,
Nobuaki Funata34
Estimated H-index: 34
+ 6 AuthorsHitoshi Nakajima5
Estimated H-index: 5
Sources
Abstract
Background. Autoimmune pancreatitis (AIP) is occasionally associated with other autoimmune diseases. Methods. To investigate the pathophysiology of AIP, we immunohistochemically examined the pancreas and other organs in eight patients with AIP, and in controls, using anti-CD4-T and CD8-T cell subsets, as well as IgG4 antibodies. Results. In AIP patients, severe or moderate infiltration of IgG4-positive plasma cells associated with CD4- or CD8-positive T lymphocytes was detected in the peripancreatic tissue (6/6), bile duct (8/ 8), gallbladder (8/8), portal area of the liver (3/3), gastric mucosa (5/7), colonic mucosa (2/2), salivary glands (1/ 2), lymph nodes (6/6), and bone marrow (2/2), as well as in the pancreas (8/8). There were few IgG4-positive plasma cells at the same sites in controls. Conclusions. These results suggest that AIP is not simply pancreatitis but that it is a pancreatic lesion involved in IgG4-related systemic disease with extensive organ involvement. We propose a new clinicopathological entity, of a systemic IgG4-related autoimmune disease in which AIP and its associated diseases might be involved.
📖 Papers frequently viewed together
2,772 Citations
1,118 Citations
493 Citations
References5
Newest
#1Terumi KamisawaH-Index: 60
#2Nobuaki FunataH-Index: 34
Last. H. NakajimaH-Index: 6
view all 8 authors...
Background: Autoimmune pancreatitis is a unique clinical entity proposed recently, and is sometimes associated with inflammation of other organs. Aims: To examine the pathophysiology of the pancreas and other organs in patients with autoimmune pancreatitis. Patients and methods: We evaluated clinicopathological findings in six resected and one autopsied patient with autoimmune pancreatitis. The pancreas, peripancreatic tissue, bile duct, and gall bladder were examined histologically and immunohi...
550 CitationsSource
#1Kazuichi Okazaki (Kyoto University)H-Index: 68
#2Tsutomu ChibaH-Index: 81
Since the first documented case of a particular form of pancreatitis with hypergammaglobulinaemia, similar cases have been reported, leading to the concept of an autoimmune related pancreatitis or so-called “autoimmune pancreatitis”. Although it has not yet been widely accepted as a new clinical entity, the present article discusses the recent concept of autoimmune pancreatitis.
558 CitationsSource
#1Hideaki HamanouH-Index: 1
#2Shigeyuki KawaH-Index: 55
Last. Kendo KiyosawaH-Index: 6
view all 9 authors...
Summary Sclerosing pancreatitis is associated with raised concentrations of lgG4. We treated 22 patients with sclerosing pancreatitis, and identified and followed-up three with concomitant hydronephrosis caused by ureteral mass, later diagnosed as retroperitoneal fibrosis. We histologically examined the ureteral and pancreatic lesions of these patients and noted abundant infiltration of lgG4-bearing plasma cells in both tissues. Treatment with corticosteroids lowered serum concentrations of lgG4...
640 CitationsSource
#1Hideaki Hamano (Shinshu University)H-Index: 38
#2Shigeyuki KawaH-Index: 55
Last. Kendo KiyosawaH-Index: 6
view all 11 authors...
Background Sclerosing pancreatitis is a unique form of pancreatitis that is characterized by irregular narrowing of the main pancreatic duct, lymphoplasmacytic inflammation of the pancreas, and hypergammaglobulinemia and that responds to glucocorticoid treatment. Preliminary studies suggested that serum IgG4 concentrations are elevated in this disease but not in other diseases of the pancreas or biliary tract. Methods We measured serum IgG4 concentrations using single radial immunodiffusion and ...
2,772 CitationsSource
Abstract The disease complex of chronic pancreatitis, sclerosing cholangitis, and Sjogren's syndrome seems to be a well-defined entity with an autoimmune cause similar to that which occurs in primary biliary cirrhosis. Treatment depends on the component of primary sclerosing cholangitis and, more particularly, on the degree of extrahepatic involvement.
140 CitationsSource
Cited By1433
Newest
#1Cecilia NapodanoH-Index: 6
#2Maria Paola MarinoH-Index: 1
Last. Umberto Basile (UCSC: Catholic University of the Sacred Heart)H-Index: 8
view all 11 authors...
The loss of tolerance to self-antigens is the unequivocal "red line" of autoimmunity: both development of autoreactive T and B cells and production of polyclonal autoantibodies represent seminal keys to the pathogenesis of protean autoimmune diseases. Most of these autoantibodies are immunoglobulins G (IgG), functionally distinguished in four subclasses named IgG1, IgG2, IgG3, and IgG4, due to structural differences in the hinge and heavy chain constant regions. Different studies analyzed serum ...
3 CitationsSource
#2A. Pérez GómezH-Index: 2
Last. M. Álvarez-Mon SotoH-Index: 3
view all 4 authors...
Source
#1Nikhil N. Tarte (Baton Rouge General Medical Center – Mid-City Campus)H-Index: 1
#1Nikhil N Tarte (UIC: University of Illinois at Chicago)
Last. Nirupa J. Patel (LSU Health Sciences Center New Orleans)H-Index: 7
view all 5 authors...
IgG4-related disease (IgG4-RD) is an immune-mediated multi-organ inflammatory disorder caused by tissue infiltration of lymphocytes with IgG4-secreting plasma cells. Herein, we discuss a case of a patient with IgG4-RD who had involvement of multiple organs: the kidneys, lymph nodes, bone marrow (biopsy performed), lungs, liver, and small intestine (imaging abnormalities). Although several case reports and series of IgG4-RD involving different organ involvement are in the literature, our patient ...
Source
#2Fatemeh Esfahanian (Tehran University of Medical Sciences)H-Index: 10
Last. Mehdi Zeinalizadeh (Tehran University of Medical Sciences)H-Index: 6
view all 5 authors...
Hypophysitis is a rare pituitary inflammatory disorder classified in different ways. Immunoglobulin G4-related disease (IgG4-RD), also a rare disease is a systemic fibro-inflammatory condition characterized by infiltration of tissue with IgG4-positive plasma cells; however prevalence of both of them probably is underestimated. In this paper, we present an Iranian patient with biopsy-proven IgG4-related hypophysitis and then review the clinical characteristics, laboratory, imaging, pathologic fin...
Source
#1Yoshihito IijimaH-Index: 6
#2Shun IwaiH-Index: 3
Last. Hidetaka UramotoH-Index: 35
view all 10 authors...
BACKGROUND Immunoglobulin G4 (IgG4)-related diseases are characterized by abnormal IgG4 levels, swelling, and marked infiltration and fibrosis of the lymphocytes and IgG4-positive plasma cells, causing hypertrophic lesions or nodules. The cause is currently not well understood. IgG4-related diseases involving lesions limited to the pleura are extremely rare. Herein, we report an IgG4-related disease presenting with multiple pleural nodules confirmed by thoracoscopic surgical biopsy. CASE PRESENT...
Source
#1Byung-Woo Yoo (Yonsei University)H-Index: 2
#2Sang-Won Lee (Yonsei University)H-Index: 23
Last. Seung Min Jung (Catholic University of Korea)H-Index: 12
view all 5 authors...
2 CitationsSource
#1Y J Ji (Shanxi Medical University)
#1Yongjin Ji (Shanxi Medical University)
view all 3 authors...
Source
#1Zhen Zhao (PKU: Peking University)H-Index: 1
#2Dapeng Mou (Capital Medical University)
Last. Yin Su (PKU: Peking University)H-Index: 16
view all 9 authors...
Background IgG4-related ophthalmic disease (IgG4-ROD) is one of the phenotypes of IgG4-related disease (IgG4-RD), and its lesions are mainly located in the ocular. Currently, there are few studies on IgG4-ROD and no study has compared the phenotypic differences between IgG4-ROD and non IgG4-ROD (nIgG4-ROD). Thus, it is difficult to establish the optimal treatment strategy for IgG4-ROD. The aim of this study was to identify the disparities between the two groups and to clarify the risk factors fo...
Source
#1G. Saitakis (MEE: Massachusetts Eye and Ear Infirmary)
#2Bart K Chwalisz (Harvard University)H-Index: 5
Abstract Purpose of review IgG4-related disease (IgG4-RD) is emerging as a fibro-inflammatory entity affecting multiple organs, including manifold neurologic manifestations. This review discusses general characteristics of IgG4-RD neurologic disease including epidemiology, histology, clinical picture and treatment approaches. Recent findings IgG4-RD is increasingly recognized as an important underlying pathophysiology in multiple disorders of neurologic interest, including orbital inflammation, ...
Source
#1Takahiro Nakazawa (Nagoya City University)H-Index: 29
#2Terumi KamisawaH-Index: 60
Last. Michiaki Unno (Tohoku University)H-Index: 54
view all 16 authors...
Background Several years have passed since the clinical diagnostic criteria for IgG4-related sclerosing cholangitis 2012 were published. New findings and knowledge have accumulated since then. Methods The Research Committees for IgG4-related Diseases and for Intractable Diseases of the Liver and Biliary Tract in association with the Ministry of Health, Labor and Welfare of Japan and the Japan Biliary Association, have established a working group consisting of researchers specializing in IgG4-SC ...
Source