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VOLUME 8 , ISSUE 1 ( January-April, 2021 ) > List of Articles


Diagnosing Small Duct Primary Sclerosing Cholangitis—A Rarer Variant of a Rare Disease: Challenges and Role of Liver Biopsy

Merina Rahman, Abdullah A Mukit, Faysal Ahmed, Dulal C Das, Sheikh M Noor-e-Alam

Keywords : Liver biopsy, Primary sclerosing cholangitis, Rare, Small duct, Ursodeoxycholic acid

Citation Information : Rahman M, Mukit AA, Ahmed F, Das DC, Noor-e-Alam SM. Diagnosing Small Duct Primary Sclerosing Cholangitis—A Rarer Variant of a Rare Disease: Challenges and Role of Liver Biopsy. Bengal Physician Journal 2021; 8 (1):24-28.

DOI: 10.5005/jp-journals-10070-7049

License: CC BY-NC 4.0

Published Online: 17-08-2021

Copyright Statement:  Copyright © 2021; The Author(s).


Primary sclerosing cholangitis (PSC), a disease of unknown etiology, is diagnosed on the clinical and biochemical findings of cholestasis combined with the characteristic findings seen on cholangiography. Small duct cholangitis is a variant of the PSC spectrum with distinct clinical course and outcome. We present two cases of small duct PSC, which are presented with the clinical and biochemical features of cholestasis but without typical positive findings on cholangiography. A liver biopsy was attempted, which confirmed sclerosing cholangitis. Upon diagnosis, both the patients were prescribed ursodeoxycholic acid at a dose of 15 mg/kg body weight/day and liver biochemistry was followed up after 3 months, which revealed biochemical improvement. Being an uncommon variant of this “not so common” disease spectrum, small duct PSC often poses a diagnostic dilemma with its presentation due to the clinical and cholangiographic mismatch. The cases remind that a negative cholangiography does not rule out the PSC spectrum.

  1. Deliwala S, Sundus S, Haykal T, et al. Small duct primary sclerosing cholangitis: an underdiagnosed cause of chronic liver disease and cirrhosis. Cureus 2020;12(3):e7298. DOI: 10.7759/cureus.7298 [Epub ahead of print].
  2. Angulo P, Maor-Kendler Y, Lindor K. Small-duct primary sclerosing cholangitis: a long term follow up study. Hepatology 2002;35(6): 1494–1500. DOI: 10.1053/jhep.2002.33202.
  3. Bjornsson E, Boberg K, Cullen S, et al. Patients with small duct primary sclerosing cholangitis have a favourable long term prognosis. Gut 2002;51(5):731–735. DOI: 10.1136/gut.51.5.731.
  4. Broome U, Glaumann H, Lindstom E, et al. Natural history and outcome in 32 Swedish patients with small duct primary sclerosing cholangitis (PSC). J Hepatol 2002;36(5):586–589. DOI: 10.1016/s0168-8278(02)00036-3.
  5. Bjornsson E, Olsson R, Bergquist A, et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology 2008;134(4):975–980. DOI: 10.1053/j.gastro.2008.01.042.
  6. Lindkvist B, Gullberg B, Bjornsson E, et al. Incidence and prevalence of primary sclerosing cholangitis in a defined adult population in Sweden. Hepatology 2010;52(2):571–577. DOI: 10.1002/hep.23678.
  7. Saarinen S, Olerup O, Broome U. Increased frequency of autoimmune diseases in patients with primary sclerosing cholangitis. Am J Gastroenterol 2000;95(11):3195–3199. DOI: 10.1111/j.1572-0241.2000.03292.x.
  8. Yanai H, Metalon S, Rosenblatt A, et al. Prognosis of primary sclerosing cholangitis in Israel is independent of coexisting inflammatory bowel disease. J Crohns Colitis 2015;9(2):177–184. DOI: 10.1093/ecco-jcc/jju013.
  9. Boonstra K, Weersma RK, van Erpecum KJ, et al. Population-based epidemiology, malignacy risk and outcome of primary sclerosing cholangitis. Hepatology 2013;58(6):2045–2055. DOI: 10.1002/hep.26565.
  10. Kuo A, Gomel R, Safer R, et al. Characteristics and outcomes reported by patients with primary sclerosing cholangitis through an online registry. Clin Gastroenterol Hepatol 2019;17(7):1372–1378. DOI: 10.1016/j.cgh.2018.04.047.
  11. Charatcharoenwitthaya P, Angulo P, Enders FB, et al. Impact of inflammatory bowel disease and ursodeoxycholic acid therapy on small-duct primary sclerosing cholangitis. Hepatology 2008;47(1):133–142. DOI: 10.1002/hep.21960.
  12. Kerlsen TH, Boberg K. Sclerosing cholangitis. In: Sherlock’s disease of the liver and biliary system. 13th ed. 2018. p. 341–354.
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