Inflammatory Nodules Identify Steroid-Responsive Primary Sclerosing Cholangitis

International Journal of Surgical Pathology
Jonathan H Chen, Vikram Deshpande

Abstract

Primary sclerosing cholangitis (PSC) is a cholangiopathy-usually associated with inflammatory bowel disease-that leads to cirrhosis and liver failure. Based on a multitude of clinical trials, there is general consensus that PSC progression is unchanged by current therapies, including steroids. However, there are scattered reports in the literature of PSC patients responsive to steroids. Recently, several steroid-responsive PSC mimics have been described, most notably immunoglobulin G4-related sclerosing cholangitis. Following these discoveries, many assume that cases in the literature previously reported as steroid-responsive PSC would now be classified as one of these mimics. We reviewed liver biopsies and the medical histories of patients diagnosed with PSC with documented response to steroids. We identified 3 cases of steroid-responsive PSC in patients with inflammatory bowel disease that do not fit criteria of known PSC mimics. All 3 were adults (age range = 18-44 years) with inflammatory bowel disease, and included 2 males and 1 female. All 3 patients had abnormal liver function tests that normalized on prednisone. Histologically, these 3 cases share a common feature, hepatic fibroinflammatory nodules in a collagen-rich ba...Continue Reading

References

Feb 1, 1991·Seminars in Liver Disease·M M Kaplan
Apr 6, 1995·The New England Journal of Medicine·Y M Lee, M M Kaplan
Jul 16, 1999·Lancet·G W ErkelensS D van der Werf
Nov 24, 1999·The American Journal of Gastroenterology·P AnguloK D Lindor
May 29, 2000·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·A J CzajaS B Moore
Sep 28, 2000·The American Journal of Gastroenterology·P AnguloK D Lindor
Mar 7, 2001·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·G V GregorioG Mieli-Vergani
Oct 6, 2001·Journal of Clinical Gastroenterology·M ParkesA S Mee
Oct 9, 2003·Scandinavian Journal of Gastroenterology·K M BobergE Schrumpf
Mar 3, 2004·Digestive Diseases and Sciences·Mark P Epstein, Marshall M Kaplan
Sep 28, 2005·Digestive Diseases and Sciences·Jasjeet S SekhonMarshall M Kaplan
Oct 14, 2006·The American Journal of Gastroenterology·Jens J W TischendorfPeter N Meier
Oct 24, 2006·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Rupert Abdalian, E Jenny Heathcote
Apr 4, 2007·Liver International : Official Journal of the International Association for the Study of the Liver·Jayant A TalwalkarR N Keith D Lindor
Jan 29, 2008·Gastroenterology·Amaar GhazaleMichael B Farnell
Mar 18, 2008·Journal of Clinical Gastroenterology·Daan W HommesSander J van Deventer
Jul 9, 2009·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Keith D LindorFelicity Enders
Jul 28, 2009·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Vikram DeshpandeGregory Y Lauwers
Jan 27, 2010·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Roger ChapmanUNKNOWN American Association for the Study of Liver Diseases
Oct 13, 2010·Nature Reviews. Gastroenterology & Hepatology·Flavia Mendes, Keith D Lindor
Nov 12, 2010·Journal of Hepatology·Kirsten Muri BobergUNKNOWN International Autoimmune Hepatitis Group
Jun 16, 2011·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Abdul M OseiniLewis R Roberts
Nov 30, 2011·Current Gastroenterology Reports·Ashley Barnabas, Roger W Chapman
Dec 20, 2011·Clinical Gastroenterology and Hepatology : the Official Clinical Practice Journal of the American Gastroenterological Association·Elizabeth MiletiMarion G Peters
Jan 14, 2012·Journal of Pediatric Gastroenterology and Nutrition·Tassos GrammatikopoulosGiorgina Mieli-Vergani
May 19, 2012·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Vikram DeshpandeJohn H Stone
Sep 18, 2012·The American Journal of Surgical Pathology·Yoh ZenBernard C Portmann
Jul 3, 2013·Lancet·Gideon M HirschfieldDavid H Adams
Apr 15, 2015·The American Journal of Gastroenterology·Keith D LindorUNKNOWN American College of Gastroenterology
Jul 5, 2015·Mayo Clinic Proceedings·John H StoneManuel Ramos-Casals
Sep 23, 2016·The New England Journal of Medicine·Konstantinos N Lazaridis, Nicholas F LaRusso
Apr 9, 2017·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Mark R DeneauM Kyle Jensen
May 17, 2017·Gastroenterology Clinics of North America·Jonathan H Chen, Vikram Deshpande

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