Published in:
01-04-2017 | Original Article—Liver, Pancreas, and Biliary Tract
Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis
Authors:
Sung-Hoon Moon, Myung-Hwan Kim, Jong Kyun Lee, Seunghee Baek, Young Sik Woo, Dong Hui Cho, Dongwook Oh, Tae Jun Song, Do Hyun Park, Sang Soo Lee, Dong Wan Seo, Sung Koo Lee
Published in:
Journal of Gastroenterology
|
Issue 4/2017
Login to get access
Abstract
Background
Recent research has shown that a substantial number of patients with primary sclerosing cholangitis (PSC) can also have elevated serum/tissue IgG4. The aim of our study was to develop a simple scoring system for the discrimination of IgG4-related sclerosing cholangits (IgG4-SC) from PSC.
Methods
Patients with IgG4-SC (n = 39) and PSC (n = 76) who had intrahepatic/hilar strictures were included. Candidate-differentiating variables included patient age, other organ involvement (OOI), inflammatory bowel disease, serum IgG4, and cholangiographic features. A scoring system was developed on the basis of these variables, and its performance was internally validated using a bootstrapping-based method.
Results
The scoring system in the final model included age (<30 years, 0 points; 30–39 years, 1 point; 40–49 years, 2 points; 50–59 years, 3 points; ≥60 years, 4 points), OOI (no, 0 points; yes, 3 points), and beaded appearance (yes, 0 points; no, 2 points). The patients were classified according to their total score into three categories: 0–4 points, probable PSC; 5–6 points, indicating diagnostic steroid trial; 7–9 points, probable IgG4-SC. The discrimination between IgG4-SC and PSC using the scoring system was excellent (area under the receiver operating characteristic curve, 0.986).
Conclusions
A reliable differentiation of IgG4-SC from PSC can be made using the scoring system presented here. We suggest the diagnosis of IgG4-SC at a cutoff of 7 points or higher and the indication of diagnostic steroid trial at 5 or 6 points. External validation of our scoring system is warranted.