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Journey to diagnosis: An unfinished exploration of IgG4-related sclerosing cholangitis.
World J Clin Cases
November 2024
Department of Infectious Diseases, Affiliated Hospital of Zunyi Medical University, Zunyi 563000, Guizhou Province, China.
IgG4-related sclerosing cholangitis (IgG4-SC) is an inflammatory disease that leads to bile duct stricture, characterized by the infiltration of IgG4-positive plasma cells into the bile duct wall, thickening of the bile duct wall, and narrowing of the lumen. The differential diagnosis of IgG4-SC mainly includes primary sclerosing cholangitis, cholangiocarcinoma, and pancreatic cancer. IgG4-SC is often associated with autoimmune pancreatitis and can be accurately diagnosed based on clinical diagnostic criteria.
View Article and Find Full Text PDFPrimary sclerosing cholangitis with IgG4-positive plasma cells in bile duct biopsies - Frequency and characterization.
J Dig Dis
June 2024
Department of Internal Medicine I, University of Bonn, Bonn, Germany.
Objectives: Patients diagnosed with primary sclerosing cholangitis (PSC) but with characteristics of immunoglobulin G4 (IgG4)-associated cholangitis (IAC) have been described. IAC often presents with biliary IgG4-positive plasma cell (IgG4+ PC) infiltration and responds to corticosteroids. In PSC, the frequencies or implications of biliary IgG4+ PC are unknown.
View Article and Find Full Text PDF[Primary sclerosing cholangitis-Diagnosis and treatment 2024].
Inn Med (Heidelb)
April 2024
Klinische Abteilung für Gastroenterologie und Hepatologie, Universitätsklinik für Innere Medizin, Medizinische Universität Graz, Auenbruggerplatz 15, 8036, Graz, Österreich.
The etiology of primary sclerosing cholangitis (PSC) remains unclear, which explains in part the lack of a causal treatment. The differential diagnostic distinction from the even rarer immunoglobulin 4 (IgG4)-associated cholangitis (IAC) is becoming increasingly more successful. Advances in the understanding of different clinical courses, improvements in noninvasive diagnostics through modern magnetic resonance imaging (MRI) and the introduction of liver elastography have led to the development of improved prognostic models.
View Article and Find Full Text PDFA case report of Immunoglobulin-G4-related hypertrophic sclerosing pachymeningitis.
Radiol Case Rep
May 2024
Department of Radiology, Hainan General Hospital (Hainan Affiliated Hospital of Hainan Medical University), NO. 19, XIUHUA ST, XIUYING DIC, Haikou, Hainan 570311, P.R. China.
IgG4-related diseases (IgG4-RD) are a group of chronic progressive autoimmune diseases of unknown etiology that are increasingly recognized as an important pathophysiological basis for a variety of systemic diseases. It is thought to involve almost any organ of the body, but the involvement of the central nervous system is relatively rare. We report the case of a 56-year-old male patient admitted to the hospital d recurrent dizziness and nausea for more than 3 months.
View Article and Find Full Text PDFImmune-mediated cholangiopathies in children: the need to better understand the pathophysiology for finding the future possible treatment targets.
Front Immunol
November 2023
2ndPediatric Discipline, Department of Mother and Child, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
Cholangiopathies are defined as focal or extensive damage of the bile ducts. According to the pathogenetic mechanism, it may be immune-mediated or due to genetic, infectious, toxic, vascular, and obstructive causes. Their chronic evolution is characterized by inflammation, obstruction of bile flow, cholangiocyte proliferation, and progression toward fibrosis and cirrhosis.
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