scholarly journals Primary sclerosing cholangitis with moderately elevated serum‐IgG4 – characterization and outcome of a distinct variant phenotype

2021 ◽  
Author(s):  
Taotao Zhou ◽  
Henrike Lenzen ◽  
Leona Dold ◽  
Bennet Bündgens ◽  
Heiner Wedemeyer ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Elevated Serum ◽  
Variant Phenotype ◽  
Serum Igg4

Elevated Serum IgG4 Concentration in Patients with Primary Sclerosing Cholangitis

2006 ◽  
Vol 101 (9) ◽  
pp. 2070-2075 ◽  
Author(s):  
Flavia D Mendes ◽  
Roberta Jorgensen ◽  
Jill Keach ◽  
Jerry A Katzmann ◽  
Thomas Smyrk ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Elevated Serum ◽  
Serum Igg4

652 Prevalence and Significance of Elevated Serum IgG4 Levels in Primary Sclerosing Cholangitis

2015 ◽  
Vol 148 (4) ◽  
pp. S-990
Author(s):  
Nazir Ahmed ◽  
Raghuwansh P. Sah ◽  
Naoki Takahashi ◽  
Keith Lindor ◽  
Suresh T. Chari
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Elevated Serum ◽  
Serum Igg4

Primary sclerosing cholangitis with elevated serum IgG4 levels and/or infiltration of abundant IgG4-positive plasma cells

2009 ◽  
Vol 45 (1) ◽  
pp. 122-129 ◽  
Author(s):  
Masanori Koyabu ◽  
Kazushige Uchida ◽  
Norimasa Fukata ◽  
Takeo Kusuda ◽  
Tsukasa Ikeura ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Serum Igg4

The impact of elevated serum IgG4 levels in patients with primary sclerosing cholangitis

2014 ◽  
Vol 46 (10) ◽  
pp. 903-908 ◽  
Author(s):  
Maria Benito de Valle ◽  
Tobias Müller ◽  
Einar Björnsson ◽  
Morgane Otten ◽  
Martin Volkmann ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Elevated Serum ◽  
Serum Igg4 ◽  
The Impact

scholarly journals Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052095921
Author(s):  
Cheng Xu ◽  
Yongmei Han
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
History Of ◽  
The Common

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.

Diagnosis, Differential Diagnosis, and Epidemiology of Primary Sclerosing Cholangitis

2015 ◽  
Vol 33 (Suppl. 2) ◽  
pp. 134-139 ◽  
Author(s):  
Cyriel Y. Ponsioen
Keyword(s):  
Bile Duct ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Large Population ◽  
Cumulative Risk ◽  
Population Based ◽  
Small Duct ◽  
Serum Igg4 ◽  
Profile Characteristic ◽  
Secondary Causes

According to recent guidelines, primary sclerosing cholangitis (PSC) is diagnosed when a patient has a cholestatic liver enzyme profile, characteristic bile duct changes on imaging, and when secondary causes of sclerosing cholangitis are excluded. In patients with a clinical suspicion but normal cholangiography, a liver biopsy is indicated to establish a diagnosis of small duct PSC. Several other disease entities such as IgG4-associated cholangitis (IAC), cholangiocarcinoma (CCA), and secondary causes of sclerosing cholangitis such as choledocholithiasis, AIDS-cholangiopathy, ischemia, surgical bile duct trauma, or mast cell cholangiopathy can mimic PSC. IAC can be differentiated from PSC by applying the HISORt criteria including the serum IgG4 level. In cases where serum IgG4 is less than 2 × ULN, the ratio of IgG4/IgG1 >0.24 is indicative for IAC. Choledocholithiasis with recurrent cholangitis as a cause of sclerosing cholangitis can pose a conundrum, since PSC itself is associated with an increased prevalence of gallstones. The epidemiology of PSC worldwide has been poorly described. Incidence and prevalence rates vary from 0-1.3 and 0-16.2 per 100,000 inhabitants respectively. However, these figures are not based on population-based cohorts. A recent large population-based cohort from the Netherlands reported an incidence of 0.5 and a prevalence of 6/100,000. Approximately 10% fulfil the criteria for small duct PSC. At diagnosis of PSC, concurrent inflammatory bowel disease (IBD), primarily ulcerative colitis or Crohn's colitis is present in 50%, but increasing to 80%, 10 years or more after diagnosis. Conversely, 3% of IBD patients will develop PSC. PSC predisposes to malignancy. The estimated cumulative risk of developing CCA after 30 years is 20%. For colorectal carcinoma in PSC/colitis patients, the estimated cumulative risk at 30 years is 13%.

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