BACKGROUND AND AIMS: Information about the natural history of small duct primary sclerosing cholangitis (SDPSC) remains scant despite literature suggesting that it constitutes 6-16% of all cases of primary sclerosing cholangitis (PSC). We combined clinical data on SDPSC cases from two tertiary care institutions with liver transplantation programs with the aim of studying the natural history of SDPSC. METHODS: Medical records of 25 individuals with SDPSC were reviewed. Diagnosis of SDPSC was based on liver biopsy findings consistent with PSC, a normal cholangiogram, and elimination of known causes of secondary sclerosing cholangitis. Demographic information, symptoms, past medical history, laboratory values, and histologic data were evaluated. Our primary outcome measure was liver transplantation or death. Secondary outcome measures included evidence of end-stage liver disease, development of cholangiocarcinoma, and/or the development of classic PSC on a repeat cholangiogram. RESULTS: Data on 25 individuals (13 males, 12 females; mean age 40 ± 15 years) diagnosed with SDPSC were analyzed. Upon presentation, 11 patients had symptoms including abdominal pain, fatigue, and pruritus. Inflammatory bowel disease was present in 14 patients (56%) at diagnosis. On initial liver biopsy, 60% had early-stage disease (I or II) and none had cirrhosis. On follow-up (1-168 months, median 17 months), malignancy or progression to classic large duct PSC was not noted. Two (8%) patients had evidence of varices and one of the two also developed ascites; one of these patients underwent liver transplantation and the other one died due to sepsis. CONCLUSIONS: SDPSC, a mild disease at presentation typically runs a benign course and likely is not an early stage of classic PSC. Further studies with a control group of classic PSC and longer follow-up are needed to study the natural history of SDPSC.
BACKGROUND AND AIMS: Information about the natural history of small duct primary sclerosing cholangitis (SDPSC) remains scant despite literature suggesting that it constitutes 6-16% of all cases of primary sclerosing cholangitis (PSC). We combined clinical data on SDPSC cases from two tertiary care institutions with liver transplantation programs with the aim of studying the natural history of SDPSC. METHODS: Medical records of 25 individuals with SDPSC were reviewed. Diagnosis of SDPSC was based on liver biopsy findings consistent with PSC, a normal cholangiogram, and elimination of known causes of secondary sclerosing cholangitis. Demographic information, symptoms, past medical history, laboratory values, and histologic data were evaluated. Our primary outcome measure was liver transplantation or death. Secondary outcome measures included evidence of end-stage liver disease, development of cholangiocarcinoma, and/or the development of classic PSC on a repeat cholangiogram. RESULTS: Data on 25 individuals (13 males, 12 females; mean age 40 ± 15 years) diagnosed with SDPSC were analyzed. Upon presentation, 11 patients had symptoms including abdominal pain, fatigue, and pruritus. Inflammatory bowel disease was present in 14 patients (56%) at diagnosis. On initial liver biopsy, 60% had early-stage disease (I or II) and none had cirrhosis. On follow-up (1-168 months, median 17 months), malignancy or progression to classic large duct PSC was not noted. Two (8%) patients had evidence of varices and one of the two also developed ascites; one of these patients underwent liver transplantation and the other one died due to sepsis. CONCLUSIONS: SDPSC, a mild disease at presentation typically runs a benign course and likely is not an early stage of classic PSC. Further studies with a control group of classic PSC and longer follow-up are needed to study the natural history of SDPSC.
Authors: Ulrika Broomé; Hans Glaumann; Eva Lindstöm; Lars Lööf; Sven Almer; Hanne Prytz; Hanna Sandberg-Gertzén; Stefan Lindgren; Frans-Thomas Fork; Gunnar Järnerot; Rolf Olsson Journal: J Hepatol Date: 2002-05 Impact factor: 25.083
Authors: Annika Bergquist; Anders Ekbom; Rolf Olsson; Dan Kornfeldt; Lars Lööf; Ake Danielsson; Rolf Hultcrantz; Stefan Lindgren; Hanne Prytz; Hanna Sandberg-Gertzén; Sven Almer; Fredrik Granath; Ulrika Broomé Journal: J Hepatol Date: 2002-03 Impact factor: 25.083
Authors: Kiran Bambha; W Ray Kim; Jayant Talwalkar; Heidi Torgerson; Joanne T Benson; Terry M Therneau; Edward V Loftus; Barbara P Yawn; E Rolland Dickson; L Joseph Melton Journal: Gastroenterology Date: 2003-11 Impact factor: 22.682
Authors: Einar Björnsson; Rolf Olsson; Annika Bergquist; Stefan Lindgren; Barbara Braden; Roger W Chapman; Kirsten M Boberg; Paul Angulo Journal: Gastroenterology Date: 2008-01-17 Impact factor: 22.682
Authors: Tobias J Weismüller; Palak J Trivedi; Annika Bergquist; Mohamad Imam; Henrike Lenzen; Cyriel Y Ponsioen; Kristian Holm; Daniel Gotthardt; Martti A Färkkilä; Hanns-Ulrich Marschall; Douglas Thorburn; Rinse K Weersma; Johan Fevery; Tobias Mueller; Olivier Chazouillères; Kornelius Schulze; Konstantinos N Lazaridis; Sven Almer; Stephen P Pereira; Cynthia Levy; Andrew Mason; Sigrid Naess; Christopher L Bowlus; Annarosa Floreani; Emina Halilbasic; Kidist K Yimam; Piotr Milkiewicz; Ulrich Beuers; Dep K Huynh; Albert Pares; Christine N Manser; George N Dalekos; Bertus Eksteen; Pietro Invernizzi; Christoph P Berg; Gabi I Kirchner; Christoph Sarrazin; Vincent Zimmer; Luca Fabris; Felix Braun; Marco Marzioni; Brian D Juran; Karouk Said; Christian Rupp; Kalle Jokelainen; Maria Benito de Valle; Francesca Saffioti; Angela Cheung; Michael Trauner; Christoph Schramm; Roger W Chapman; Tom H Karlsen; Erik Schrumpf; Christian P Strassburg; Michael P Manns; Keith D Lindor; Gideon M Hirschfield; Bettina E Hansen; Kirsten M Boberg Journal: Gastroenterology Date: 2017-03-06 Impact factor: 22.682