Clinical characteristics of immunoglobulin IgG4-related sclerosing cholangitis: Comparison of cases with and without autoimmune pancreatitis in a large cohort.
Dig Liver Dis
; 53(10): 1308-1314, 2021 Oct.
Article
in En
| MEDLINE
| ID: mdl-33664004
ABSTRACT
BACKGROUND:
The clinical characteristics of IgG4-related sclerosing cholangitis (IgG4-SC) especially without autoimmune pancreatitis (AIP) have not been investigated in a large cohort.AIMS:
To clarify the clinical characteristics of IgG4-SC and IgG4-SC without AIP.METHODS:
We retrospectively reviewed imaging, serology, other organ involvement (OOI) and histology of 872 patients with IgG4-SC who participated in a Japanese nationwide survey in 2019, and compared these items between IgG4-SC with and without AIP.RESULTS:
AIP was present in 83.7% (730/872) of IgG4-SC. In IgG4-SC, bile duct wall thickening was observed on ultrasound (528/650; 81.2%), computed tomography (375/525; 71.4%) and magnetic resonance imaging or cholangiopancreatography (290/440; 65.9%). An elevated serum IgG4 level (≥ 135â¯mg/dL) was found in 88.0% (322/366). IgG4-related OOI other than AIP was observed in 25.2% (211/836). The proportion of females was significantly higher in IgG4-SC without AIP (28.9% vs. 20.1%; pâ¯=â¯0.025). Hilar stricture was the most common cholangiographic type in IgG4-SC without AIP (39/107; 36.4%).There were no significant differences between IgG4-SC with and without AIP in the rates of bile duct wall thickening, elevated serum IgG4 level, or IgG4-related OOI.CONCLUSIONS:
The clinical characteristics of IgG4-SC was similar between IgG4-SC with and without AIP in a large cohort.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Bile Ducts
/
Cholangitis, Sclerosing
/
Immunoglobulin G4-Related Disease
/
Autoimmune Pancreatitis
Type of study:
Diagnostic_studies
/
Observational_studies
/
Risk_factors_studies
Limits:
Female
/
Humans
/
Male
Country/Region as subject:
Asia
Language:
En
Journal:
Dig Liver Dis
Journal subject:
GASTROENTEROLOGIA
Year:
2021
Document type:
Article