Immunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice—Case Report and Literature Review
Joint Authors
Karmacharya, Paras
Shrestha, Pragya
Le, Brian
Wagner, Brent
Pompella, William
Source
Issue
Vol. 2018, Issue 2018 (31 Dec. 2018), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2018-08-23
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
IgG4-related sclerosing cholangitis (IgG4-SC) is one of the most common extra-pancreatic manifestation of IgG4-related disease (IgG4-RD) and is clinically distinct from primary sclerosing cholangitis (PSC).
IgG4-RD is an increasingly recognized immune-mediated fibroinflammatory systemic disease, mostly affecting middle-aged and older male populations that can affect multiple organs.
The presence of extra-biliary clinical manifestations of IgG4-RD, such as parotid and lacrimal swelling, lymphadenopathy, autoimmune pancreatitis, and retroperitoneal fibrosis, if present could provide important clues to diagnosis.
High serum IgG4 levels, characteristic radiological (e.g., sausage-shaped pancreas or periaortitis) or biopsy findings (high percentage of IgG4+ plasma cells, lymphoplasmacytic infiltrate, storiform fibrosis, or obliterative phlebitis) in the setting of these features is diagnostic of this disease process.
However, isolated IgG4-SC might be a diagnostic challenge, and the distinction is important as management of this disorder is vastly different from other causes of cholangitis such as PSC.
Systemic corticosteroid therapy is the mainstay of therapy.
American Psychological Association (APA)
Shrestha, Pragya& Le, Brian& Wagner, Brent& Pompella, William& Karmacharya, Paras. 2018. Immunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice—Case Report and Literature Review. Case Reports in Rheumatology،Vol. 2018, no. 2018, pp.1-5.
https://search.emarefa.net/detail/BIM-1149906
Modern Language Association (MLA)
Shrestha, Pragya…[et al.]. Immunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice—Case Report and Literature Review. Case Reports in Rheumatology No. 2018 (2018), pp.1-5.
https://search.emarefa.net/detail/BIM-1149906
American Medical Association (AMA)
Shrestha, Pragya& Le, Brian& Wagner, Brent& Pompella, William& Karmacharya, Paras. Immunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice—Case Report and Literature Review. Case Reports in Rheumatology. 2018. Vol. 2018, no. 2018, pp.1-5.
https://search.emarefa.net/detail/BIM-1149906
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1149906