IgG4-related sclerosing disease (IgG4-RSD) is a systemic one in which IgG4-positive plasma cells and T lymphocytes extensively infiltrate various organs. The clinical manifestations of the disease include autoimmune pancreatitis, sclerosing cholangitis, cholecystitis, sialodenitis, retroperitoneal fibrosis, tubulointestitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumors and lymphadenopathy, all related with significantly elevated serum IgG4 levels. Tissue fibrosis with obliterative phlebitis of the affected organs is pathologically induced. The disease occurs predominantly in elderly men and responds well to steroid therapy. Since malignant tumors are frequently suspected on initial presentation, IgG4-RSD should be considered in the differential diagnosis to avoid unnecessary surgery.
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Immunoglobulin G4 related sclerosing disease mimicking a lytic lesion of the mandible: a case report and review of literature.
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Department of Oral and Maxillofacial Surgery, Goa Dental College and Hospital, Bambolim, Goa 403202, India.
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Peter Medawar Building for Pathogen Research, Nuffield Department of Medicine, University of Oxford, South Parks Road, Oxford OX1 3SY, UK.
Immune-mediated liver and biliary conditions, such as IgG4-related pancreatobiliary disease (IgG4-PB) and a subset of primary sclerosing cholangitis (PSC- high(h)IgG4), exhibit increased IgG4 levels in the blood. The relative expression of IgG4+ and IgG1+ B cells in the blood and the expression of complement and Fc receptors on these IgG1+ and IgG4+ B cells in IgG4-PB and PSC have not been previously described. We hypothesised that the patterns of expression of these cells and their receptors would differ, are relevant to disease pathogenesis and may represent therapeutic targets.
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Division of Gastroenterology and Hepatology, Department of Internal Medicine, Faculty of Medicine, University of Miyazaki, Miyazaki 889-1692, Japan.
Article Synopsis
- The case report discusses a 64-year-old woman with IgG4-related sclerosing cholangitis who developed multiple intrahepatic artery aneurysms while on treatment.
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- The report highlights that hepatic artery aneurysms can be serious complications in patients with IgG4-SC, indicating a need for vigilant monitoring.
IgG4-related disease for the hematologist.
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Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disease with many important manifestations in hematopoietic and lymphoid tissue. IgG4 is the least naturally abundant IgG subclass, and the hallmark feature of IgG4-RD is markedly increased IgG4-positive plasma cells (with an IgG4 to IgG ratio >40%) in affected tissue, along with elevated polyclonal serum IgG and IgG4 in most patients. Histological diagnosis is essential, and other key features include storiform fibrosis, lymphoplasmacytic infiltrate, tissue eosinophilia, and obliterative phlebitis.
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Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Japan. Electronic address:
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