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IgG4 sclerosing disease

Sophie Restellini, Olivier Chazouillères, Jean-Louis Frossard
Inflammatory bowel diseases (IBD) are associated with various hepatobiliary disorders, reported both in Crohn's disease (CD) and ulcerative colitis (UC). They may occur at any moment in the natural course of the disease. The prevalence of liver dysfunction rises from 3% to 50% accordingly to definitions. Fatty liver is considered as the most common hepatobiliary complication in IBD whilst primary sclerosing cholangitis (PSC) the most specific one. Less frequently, IBD-associated hepatobiliary disorders include: autoimmune hepatitis/PSC overlap syndrome, IgG4-associated cholangiopathy, primary biliary cholangitis (PBC), hepatic amyloidosis, granulomatous hepatitis, cholelithiasis, portal vein thrombosis, and liver abscess...
October 6, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
Melinda J Yeh, So Yeon Kim, Kartik S Jhaveri, Spencer C Behr, Nieun Seo, Benjamin M Yeh
Autoimmune biliary diseases are poorly understood but important to recognize. Initially, autoimmune biliary diseases are asymptomatic but may lead to progressive cholestasis with associated ductopenia, portal hypertension, cirrhosis, and eventually liver failure. The three main forms of autoimmune biliary disease are primary biliary cirrhosis, primary sclerosing cholangitis, and IgG4-associated cholangitis. Although some overlap may occur between the three main autoimmune diseases of the bile ducts, each disease typically affects a distinct demographic group and requires a disease-specific diagnostic workup...
September 17, 2016: Abdominal Radiology
Tzyy-Ling Chuang, Bao-Bao Hsu, Chen-Lin Chi, Yuh-Feng Wang
BACKGROUND: The clinical picture of IgG4-related sclerosing disease (IgG4-RSD) may mimic lymphoma, and should be in the differential diagnosis of patients with this clinical picture. CASE SUMMARY: A 32-year-old female had recurrent swelling of both eyelids for more than 15 years. Examination revealed elastic, firm, swollen lacrimal glands about 2-3 cm in diameter that was not painful. Head and orbits magnetic resonance imaging (MRI) showed mass lesions over the bilateral lacrimal glands, submandibular glands, and left foramen of ovale...
September 2016: Medicine (Baltimore)
Emma L Culver, Roger W Chapman
IgG4-related hepatobiliary diseases are part of a multiorgan fibroinflammatory condition termed IgG4-related disease, and include IgG4-related sclerosing cholangitis (IgG4-SC) and IgG4-related hepatopathy. These diseases can present with biliary strictures and/or mass lesions, making them difficult to differentiate from primary sclerosing cholangitis (PSC) or other hepatobiliary malignancies. Diagnosis is based on a combination of clinical, biochemical, radiological and histological findings. However, a gold standard diagnostic test is lacking, warranting the identification of more specific disease markers...
October 2016: Nature Reviews. Gastroenterology & Hepatology
Dun-Hao Chang, Pao-Shu Wu, Yu-Chen Wang, Chin-Hsuan Lin, Wing-Yin Li, Hsu Ma, Chern-Kang Perng
OBJECTIVES: To investigate the clinicopathologic characteristics of patients with immunoglobulin G4-related chronic sclerosing sialadenitis (IgG4-RCSS), a recently recognized disease. STUDY DESIGN: Case series with chart review and pathology study. SETTINGS: Tertiary care hospital. SUBJECTS AND METHODS: We evaluated chronic sialadenitis specimens obtained over 11 years using pathologic examination and IgG4 immunohistochemistry staining...
August 30, 2016: Otolaryngology—Head and Neck Surgery
T N K W Gunasekara, S Di Palma, I N Bagwan
BACKGROUND: IgG4 related disease rarely affects the salivary glands and clinically is often confused with salivary gland malignancy. METHOD: This is a retrospective study comprising 137 cases of chronic sialadenitis diagnosed in a histopathology department over 4 years. The morphology was assessed by reviewing the histology slides and the incidence of IgG4 related sclerosing sialadenitis was calculated. IgG and IgG4 immunohistochemistry was performed and mean IgG4 count/hpf and IgG4/IgG ratio were determined...
August 2016: Malaysian Journal of Pathology
Kung-Chao Chang, Jen-Chieh Lee, Yu-Chu Wang, Liang-Yi Hung, Yenlin Huang, Wan-Ting Huang, Ren-Ching Wang, Tse-Ching Chen, Yi-Shan Tsai, L Jeffrey Medeiros
Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a morphologically distinctive lesion. Although the clinical course of SANT is benign, its reactive or neoplastic nature remains to be clarified. Furthermore, some investigators have suggested that SANT is related to IgG4 sclerosing lesion or inflammatory pseudotumor with stromal cells positive for Epstein-Barr virus (EBV). In this study, we assessed 22 cases of SANT derived from adult women. Clinical data and follow-up information were obtained by chart review...
October 2016: American Journal of Surgical Pathology
Y P Chen, X N Jiang, J P Lu, H Zhang, X Q Li, G Chen
OBJECTIVE: To investigate the clinicopathologic features, diagnosis and differential diagnosis of extranodal Rosai-Dorfman disease(RDD)of the breast. METHODS: Twelve cases of extranodal RDD of the breast were analyzed using hematoxylin-eosin stain and immunohistochemical staining.The morphological features and immunophenotype were observed by light microscopy, accompanied with a review of the literature. RESULTS: Twelve cases of extranodal RDD of the breast are presented...
August 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Sung-Hoon Moon, Myung-Hwan Kim, Jong Kyun Lee, Seunghee Baek, Young Sik Woo, Dong Hui Cho, Dongwook Oh, Tae Jun Song, Do Hyun Park, Sang Soo Lee, Dong Wan Seo, Sung Koo Lee
BACKGROUND: Recent research has shown that a substantial number of patients with primary sclerosing cholangitis (PSC) can also have elevated serum/tissue IgG4. The aim of our study was to develop a simple scoring system for the discrimination of IgG4-related sclerosing cholangits (IgG4-SC) from PSC. METHODS: Patients with IgG4-SC (n = 39) and PSC (n = 76) who had intrahepatic/hilar strictures were included. Candidate-differentiating variables included patient age, other organ involvement (OOI), inflammatory bowel disease, serum IgG4, and cholangiographic features...
July 28, 2016: Journal of Gastroenterology
Terumi Kamisawa, Sawako Kuruma, Kazuro Chiba
An accurate diagnosis should be made before treatment of autoimmune pancreatitis and immunoglobulin G4-related sclerosing cholangitis. Once a diagnosis has been established, steroids are the standard therapy and symptoms are the major indications. Before steroid therapy, obstructive jaundice and hyperglycemia should be controlled. An initial dose of 0.6 mg/kg/d of oral prednisolone is administered for 2 to 4 weeks, and is gradually tapered over 2 to 3 months. After steroid therapy has begun, blood and imaging tests are performed periodically...
August 2016: Seminars in Liver Disease
Shigeyuki Kawa
Although now considered to be a member of the systemic entity of immunoglobulin G4- (IgG4-) related disease, IgG4-related pancreatitis is generally referred to as type 1 autoimmune pancreatitis (AIP). Type 1 AIP was established based on a pathological background of lymphoplasmacytic sclerosing pancreatitis, high serum IgG4 concentration, and abundant IgG4-bearing plasma cell infiltration. The characteristic clinical features of type 1 AIP, such as elderly male preponderance, obstructive jaundice, and mass-forming lesions in the pancreas, often mimic those of pancreatic cancer...
August 2016: Seminars in Liver Disease
Yoh Zen
Immunoglobulin G4-related disease (IgG4-RD) in the pancreatobiliary system manifests as sclerosing cholangitis (SC), hepatic inflammatory pseudotumors, and type 1 autoimmune pancreatitis (AIP). The pathology of IgG4-RD involves an inflammatory process and fibrogenic pathway, the combination of which damages the affected organs. Fibroinflammatory injury is characterized by three microscopic findings: a diffuse lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, obliterative phlebitis, and storiform fibrosis...
August 2016: Seminars in Liver Disease
Yasuni Nakanuma, Yoji Ishizu, Yoh Zen, Kenichi Harada, Takeji Umemura
Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease involving many organs; it includes IgG4-related sclerosing cholangitis and inflammatory pseudotumor in the hepatobiliary system. Two types of hepatic parenchymal involvement have been reported in IgG4-RD: IgG4-related autoimmune hepatitis (AIH) and IgG4-hepatopathy. Moreover, only three cases of IgG4-related AIH have been reported. Immunoglobulin G4-related AIH is clinicopathologically similar to AIH, except for an elevated serum IgG4 level and heavy infiltration of IgG4-positive plasma cells in the liver tissue...
August 2016: Seminars in Liver Disease
Haruko Tanji, Hiroaki Okada, Ryosuke Igari, Yoshitaka Yamaguchi, Hiroyasu Sato, Yoshimi Takahashi, Shingo Koyama, Shigeki Arawaka, Manabu Wada, Toru Kawanami, Koichi Wakabayashi, Takeo Kato
A 58-year-old woman with a 1-month history of right hand clumsiness and speaking difficulty was admitted to our hospital. A neurological examination revealed sensory aphasia and right hemiparesis. Her laboratory tests showed elevated serum levels of IgG and IgG4, pancytopenia, and liver dysfunction. The results of the imaging studies of her abdomen were compatible with sclerosing cholangitis. Brain MRI showed extensive signal abnormalities in the left hemisphere on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images, extending from left internal capsule to the cerebral peduncle with an irregularly enhancing lesion in the left parietal lobe...
2016: Internal Medicine
Sum P Lee, Joseph R Roberts, Rahul Kuver
A variety of diseases are included under the umbrella term 'cholangitis', including hepatobiliary diseases with an autoimmune pathogenesis (such as primary sclerosing cholangitis, primary biliary cholangitis, and IgG4-associated sclerosing cholangitis) and disease processes associated with intraductal stones and infectious etiologies (such as ascending bacterial cholangitis, recurrent pyogenic cholangitis, and liver fluke-associated cholangitis). Recent advances in the pathophysiologic bases of these disorders, particularly with respect to the autoimmune variety, are allowing improved diagnosis and prognostication as well as providing the opportunity to refine and re-imagine treatment modalities...
2016: F1000Research
Hilary C McCrary, Erynne A Faucett, Audrey B Erman
Immunoglobulin G4-related sclerosing disease (IgG4-RSD) is a fibroinflammatory condition that has the potential to affect nearly every organ system. Classic histological findings include storiform fibrosis and lymphoplasmacytic infiltrates of immunoglobulin G4 (IgG4)-positive plasma cells. The clinical features of IgG4-RSD may be an under-recognized disease process that can mimic other autoimmune disorders, including Sjogren's syndrome. We describe a rare case of IgG4-RSD involving the salivary glands, initially misdiagnosed as Sjogren's syndrome...
October 2016: Laryngoscope
Ayodeji Oluwarotimi Omiyale
Autoimmune pancreatitis (AIP) is a rare, distinct and increasingly recognized form of pancreatitis which has autoimmune features. The international consensus diagnostic criteria (ICDC) for AIP recently described two subtypes; type 1[lymphoplasmacytic sclerosing pancreatitis (LPSP)] and type 2 [idiopathic duct-centric pancreatitis (IDCP) or AIP with granulocytic epithelial lesion (GEL)]. Type 1 is the more common form of the disease worldwide and current understanding suggests that it is a pancreatic manifestation of immunoglobulin G4-related disease (IgG4-RD)...
June 2016: Gland Surgery
Kevin Grewal, Paul Cohen, Jeff S Kwon, David A Kaufman
We describe a case of IgG4-related lung disease presenting as a lung mass with associated weight loss. IgG4-related disease is a systemic sclerosing disorder that causes fibrotic, often tumor-like manifestations that variably effect different organ systems. The clinical presentation of IgG4-related disease is protean. Timely recognition and diagnosis requires awareness on the part of clinicians and pathologists to the variable manifestations of this newly recognized disorder. We offer a concise review of the pulmonary manifestations, diagnosis and treatment of IgG4-related lung disease...
2016: Respiratory Medicine Case Reports
Juan Putra, Deborah L Ornstein
Küttner tumor/chronic sclerosing sialadenitis is a fibroinflammatory process that characteristically involves the submandibular gland of patients with IgG4-related disease. Histologic examination is often important to make the diagnosis because of its nonspecific clinical and radiologic findings. Microscopically, Küttner tumor should be distinguished from other entities such as extranodal marginal zone lymphoma, Sjögren's syndrome, and lymphoepithelial sialadenitis. The lesion is histologically well-demarcated with lobular architecture, extensive fibrosis, marked lymphoplasmacytic inflammation, formation of lymphoid follicles, acinar atrophy, and obliterative phlebitis, without the presence of lymphoepithelial lesions...
May 18, 2016: Head and Neck Pathology
Antonino Tuttolomondo, Irene Simonetta, Domenico Di Raimondo, Roberta Conigliaro, Francesca Corpora, Valerio Vassallo, Antonio Pinto
BACKGROUND: IgG4-related disease is a rare, clinical and pathologic disease entity of unknown etiology. Its main features are increased serum concentrations of IgG4 > 1,35 g/l, lymphocyte and IgG4+plasma-cell infiltration within tissues, fibrosis or sclerosis. The classical presentation of IgG4-RSD is pancreatitis which is combined with the involvement of biliary ducts in 74 percent of patients. Extrapancreatic manifestations include: abdominal or mediastinal lymphadenopathy; the involvement of salivary glands and lacrimal glands, kidneys, lung, retroperitoneum...
May 10, 2016: Current Pharmaceutical Design
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