Journal of Gastroenterology

, Volume 52, Issue 4, pp 483–493

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

  • 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
Original Article—Liver, Pancreas, and Biliary Tract

DOI: 10.1007/s00535-016-1246-5

Cite this article as:
Moon, SH., Kim, MH., Lee, J.K. et al. J Gastroenterol (2017) 52: 483. doi:10.1007/s00535-016-1246-5
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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.

Keywords

IgG4-related sclerosing cholangitis Primary sclerosing cholangitis Diagnosis Scoring system 

Supplementary material

535_2016_1246_MOESM1_ESM.tif (74 kb)
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)
535_2016_1246_MOESM2_ESM.docx (16 kb)
Supplementary material 2 (DOCX 15 kb)
535_2016_1246_MOESM3_ESM.docx (14 kb)
Supplementary material 3 (DOCX 13 kb)
535_2016_1246_MOESM4_ESM.docx (15 kb)
Supplementary material 4 (DOCX 14 kb)

Copyright information

© Japanese Society of Gastroenterology 2016

Authors and Affiliations

  • Sung-Hoon Moon
    • 1
  • Myung-Hwan Kim
    • 2
  • Jong Kyun Lee
    • 3
  • Seunghee Baek
    • 4
  • Young Sik Woo
    • 3
  • Dong Hui Cho
    • 2
  • Dongwook Oh
    • 2
  • Tae Jun Song
    • 2
  • Do Hyun Park
    • 2
  • Sang Soo Lee
    • 2
  • Dong Wan Seo
    • 2
  • Sung Koo Lee
    • 2
  1. 1.Department of Internal Medicine, Hallym University College of MedicineHallym University Sacred Heart HospitalAnyangSouth Korea
  2. 2.Department of Internal Medicine, University of Ulsan College of MedicineAsan Medical CenterSeoulSouth Korea
  3. 3.Department of Medicine, Sungkyunkwan University School of MedicineSamsung Medical CenterSeoulSouth Korea
  4. 4.Department of Clinical Epidemiology and Biostatistics, University of Ulsan College of MedicineAsan Medical CenterSeoulSouth Korea

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