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Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis

  • Original Article—Liver, Pancreas, and Biliary Tract
  • Published:
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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.

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    Authors and Affiliations

    1. Department of Internal Medicine, Hallym University College of Medicine, Hallym University Sacred Heart Hospital, Anyang, South Korea

      Sung-Hoon Moon

    2. Department of Internal Medicine, University of Ulsan College of Medicine, Asan Medical Center, Asanbyeongwon-Gil 86, Songpa-gu, Seoul, 138-736, South Korea

      Myung-Hwan Kim, Dong Hui Cho, Dongwook Oh, Tae Jun Song, Do Hyun Park, Sang Soo Lee, Dong Wan Seo & Sung Koo Lee

    3. Department of Medicine, Sungkyunkwan University School of Medicine, Samsung Medical Center, 81 Irwon-Ro, Gangnam-Gu, Seoul, South Korea

      Jong Kyun Lee & Young Sik Woo

    4. Department of Clinical Epidemiology and Biostatistics, University of Ulsan College of Medicine, Asan Medical Center, Seoul, South Korea

      Seunghee Baek

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    M.-H. Kim and J. K. Lee contributed equally to this work as corresponding authors.

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    535_2016_1246_MOESM1_ESM.tif

    Supplementary Fig. 1. Capability of the scoring system to differentiate between IgG4-related sclerosing cholangitis and primary sclerosing cholangitis. The area under the receiver operating characteristic curve of the scoring system was 0.986 (TIFF 74 kb)

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    Moon, SH., Kim, MH., Lee, J.K. et al. Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis. J Gastroenterol 52, 483–493 (2017). https://doi.org/10.1007/s00535-016-1246-5

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    • DOI: https://doi.org/10.1007/s00535-016-1246-5

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