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dc.contributor.authorBjörnsson, Einar
dc.contributor.authorChari, Suresh
dc.contributor.authorSilveira, Marina
dc.contributor.authorGossard, Andrea
dc.contributor.authorTakahashi, Naoki
dc.contributor.authorSmyrk, Thomas
dc.contributor.authorLindor, Keith
dc.date.accessioned2012-05-14T09:21:09Z
dc.date.available2012-05-14T09:21:09Z
dc.date.issued2011-05
dc.date.submitted2012-05-14
dc.identifier.citationAm. J. Ther. 2011, 18(3):198-205en_GB
dc.identifier.issn1536-3686
dc.identifier.pmid20228674
dc.identifier.doi10.1097/MJT.0b013e3181c9dac6
dc.identifier.urihttp://hdl.handle.net/2336/223496
dc.descriptionTo access publisher full text version of this article. Please click on the hyperlink in Additional Links field.en_GB
dc.description.abstractSteroid responsive biliary strictures in patients fulfilling criteria for primary sclerosing cholangitis (PSC) have been reported. The clinical course and response to therapy in patients with PSC with elevated immunoglobulin G4 (IgG4) levels has not been investigated previously. Patients with PSC were screened for IgG4-related biliary disease during 2006 to 2008 and data were collected prospectively. A total of 33 out of 285 (12%) patients with PSC (18 males) had elevated IgG4 (>140mg/dL) with a median age of 46 years (interquartile range 29-60); 24 could be evaluated. All patients had both intrahepatic and extrahepatic biliary strictures. Pancreatic disorders were found in 4 (17%), and 11 of 24 (46%) presented with jaundice; 8 of 24 (33%) received biliary stenting for a median time of 4 months (0-6). Liver cirrhosis was diagnosed in 12 of the 24 (50%). Overall, 18 patients were treated with corticosteroids and 6 patients managed conservatively. Nine of 10 patients with elevated bilirubin had improvement. Alkaline phosphatase decreased significantly at 2 months and at last follow-up. IgG4 levels at baseline were 242 (216-357) mg/dL and decreased to 109 (80-236) at 2 months (P < 0.05) and 174 (115-269) at last follow-up (P < 0.05). A total of 39% had adverse effects of steroids, mostly hyperglycemia. Relapses occurred in 7 of the 14 (50%), but biliary stents could be removed in all. Elevated IgG4 levels were observed in 12% of typical patients with PSC. Prevalence of cirrhosis was high, suggesting a severe liver disease course. Most patients had a good biochemical response to steroids, but adverse effects were common. Future work should be directed at finding therapy that is more effective, better tolerated, and of more lasting benefit.
dc.language.isoenen
dc.publisherLippincott Williams & Wilkinsen_GB
dc.relation.urlhttp://dx.doi.org/10.1097/MJT.0b013e3181c9dac6en_GB
dc.rightsArchived with thanks to American journal of therapeuticsen_GB
dc.subject.meshAdrenal Cortex Hormonesen_GB
dc.subject.meshAdulten_GB
dc.subject.meshAlkaline Phosphataseen_GB
dc.subject.meshBiliary Tract Surgical Proceduresen_GB
dc.subject.meshBilirubinen_GB
dc.subject.meshCholangiocarcinomaen_GB
dc.subject.meshCholangitis, Sclerosingen_GB
dc.subject.meshFemaleen_GB
dc.subject.meshHumansen_GB
dc.subject.meshImmunoglobulin Gen_GB
dc.subject.meshLiver Cirrhosisen_GB
dc.subject.meshLiver Diseasesen_GB
dc.subject.meshMaleen_GB
dc.subject.meshMiddle Ageden_GB
dc.subject.meshPancreatitisen_GB
dc.subject.meshRecurrenceen_GB
dc.titlePrimary sclerosing cholangitis associated with elevated immunoglobulin G4: clinical characteristics and response to therapy.en
dc.typeArticleen
dc.contributor.departmentLandspitali The National University Hospital, Reykjavík, Iceland. Division of Gastroenterology and Hepatology, Sahlgrenska University Hospital, Gothenburg, Sweden.en_GB
dc.identifier.journalAmerican journal of therapeuticsen_GB
html.description.abstractSteroid responsive biliary strictures in patients fulfilling criteria for primary sclerosing cholangitis (PSC) have been reported. The clinical course and response to therapy in patients with PSC with elevated immunoglobulin G4 (IgG4) levels has not been investigated previously. Patients with PSC were screened for IgG4-related biliary disease during 2006 to 2008 and data were collected prospectively. A total of 33 out of 285 (12%) patients with PSC (18 males) had elevated IgG4 (>140mg/dL) with a median age of 46 years (interquartile range 29-60); 24 could be evaluated. All patients had both intrahepatic and extrahepatic biliary strictures. Pancreatic disorders were found in 4 (17%), and 11 of 24 (46%) presented with jaundice; 8 of 24 (33%) received biliary stenting for a median time of 4 months (0-6). Liver cirrhosis was diagnosed in 12 of the 24 (50%). Overall, 18 patients were treated with corticosteroids and 6 patients managed conservatively. Nine of 10 patients with elevated bilirubin had improvement. Alkaline phosphatase decreased significantly at 2 months and at last follow-up. IgG4 levels at baseline were 242 (216-357) mg/dL and decreased to 109 (80-236) at 2 months (P < 0.05) and 174 (115-269) at last follow-up (P < 0.05). A total of 39% had adverse effects of steroids, mostly hyperglycemia. Relapses occurred in 7 of the 14 (50%), but biliary stents could be removed in all. Elevated IgG4 levels were observed in 12% of typical patients with PSC. Prevalence of cirrhosis was high, suggesting a severe liver disease course. Most patients had a good biochemical response to steroids, but adverse effects were common. Future work should be directed at finding therapy that is more effective, better tolerated, and of more lasting benefit.


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