A Case of Advanced-Stage Sclerosing Cholangitis with Autoimmune Pancreatitis not Responsive to Steroid Therapy
Abstract
Context Autoimmune pancreatitis and sclerosing cholangitis associated with autoimmune pancreatitis respond well to steroid therapy. Some criteria used for the differential diagnosis of autoimmune pancreatitis and sclerosing cholangitis with autoimmune pancreatitis include the response to a steroid trial. Case report A 68-year-old woman was diagnosed as having type 3 sclerosing cholangitis with autoimmune pancreatitis four years after clinical onset. Seven years after clinical onset, imaging findings revealed multiple pancreatic stones in an atrophic pancreas, stenosis of the main pancreatic duct in the head of the pancreas and upstream dilatation and a longer stretch of stenosis in the hilar hepatic region. We tried steroid therapy in an attempt to ameliorate stenosis of both the bile duct and the pancreatic duct and prevent further progression. Neither lesion responded to steroid therapy. Conclusion Advanced-stage sclerosing cholangitis with autoimmune pancreatitis may sometimes be unresponsive to steroid therapy, and this should be borne in mind when attempting a steroid trial for the diagnosis of sclerosing cholangitis with autoimmune pancreatitis. Early administration of steroid is important for the prevention of disease progression.
Image: ERCP by using a balloon catheter showing a longer stretch of stenosis in the hilar hepatic region.
Downloads
References
Nakazawa T, Ohara H, Sano H, Ando T, Aoki S, Kobayashi S, et al. Clinical differences between primary sclerosing cholangitis and sclerosing cholangitis with autoimmune pancreatitis. Pancreas 2005; 30:20-5. [PMID 15632695] (FULL TEXT: http://journals.lww.com/pancreasjournal/pages/articleviewer.aspx?year=2005&issue=01000&article=00003&type=abstract)
Nakazawa T, Ohara H, Sano H, Ando T, Joh T. Schematic classification of sclerosing cholangitis with autoimmune pancreatitis by cholangiography. Pancreas 2006; 32:229. [PMID 16552350] (FULL TEXT: http://journals.lww.com/pancreasjournal/Citation/2006/03000/Schematic_Classification_of_Sclerosing_Cholangitis.21.aspx)
Nakazawa T, Ohara H, Sano H, Aoki S, Kobayashi S, Okamoto T, et al. Cholangiography can discriminate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis. Gastrointest Endosc 2004; 60:937-44. [PMID 15605009] (FULL TEXT: http://www.giejournal.org/article/PIIS0016510704022291/fulltext)
Kim KP, Kim MH, Kim JC, Lee SS, Seo DW, Lee SK. Diagnostic criteria for autoimmune chronic pancreatitis revisited. World J Gastroenterol 2006; 12:2487-96. [PMID 16688792] (FULL TEXT: http://www.wjgnet.com/1007-9327/12/2487.asp)
Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, et al. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol 2006; 4:1010-6. [PMID 16843735] (FULL TEXT: http://www.cghjournal.org/article/PIIS1542356506004861/fulltext)
Ghazale A, Chari ST, Zhang L, Smyrk TC, Takahashi N, Levy MJ, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008; 134:706-15. [PMID 18222442] (FULL TEXT: http://www.sciencedirect.com/science?_ob=MImg&_imagekey=B6WFX-4R98K3S-2-9&_cdi=6806&_user=839424&_orig=search&_coverDate=03%2F31%2F2008&_sk=998659996&view=c&wchp=dGLbVzb-zSkWb&md5=0073e0aaeedd23079008740dbcad0b3c&ie=/sdarticle.pdf)
Hirano A, Nakazawa T, Ohara H, Ando T, Hayashi K, Tanaka H, et al. Liver atrophy and portal stenosis in two cases of sclerosing cholangitis associated with autoimmune pancreatitis. Intern Med 2008; 47:1689-94. [PMID 18827417] (FULL TEXT: http://www.jstage.jst.go.jp/article/internalmedicine/47/19/1689/_pdf)
Okazaki K, Kawa S, Kamisawa T, Naruse S, Tanaka S, Nishimori I, et al. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol 2006; 41:626-31. [PMID 16932998] (FULL TEXT: http://www.springerlink.com/content/j6737117583k6465/fulltext.html)
Mendes FD, Jorgensen R, Keach J, Katzmann JA, Smyrk T, Donlinger J, et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 2006; 101:2070-5. [PMID 16879434] (FULL TEXT: http://www.nature.com/ajg/journal/v101/n9/full/ajg2006381a.html)
Kamisawa T, Okamoto A. Prognosis of autoimmune pancreatitis. J Gastroenterol 2007; 42 Suppl 18:59-62. [PMID 17520225] (FULL TEXT: http://www.springerlink.com/content/j011u03720v78606/fulltext.pdf)
Takayama M, Hamano H, Ochi Y, Saegusa H, Komatsu K, Muraki T, et al. Recurrent attacks of autoimmune pancreatitis result in pancreatic stone formation. Am J Gastroenterol 2004; 99:932-7. [PMID 15128363] (FULL TEXT: http://www.nature.com/ajg/journal/v99/n5/full/ajg2004177a.html)

Copyright (c) 2010 Takahiro Nakazawa, Itaru Naitoh, Tomoaki Ando, Kazuki Hayashi, Fumihiro Okumura, Katsuyuki Miyabe, Michihiro Yoshida, Hirotaka Ohara, Takashi Joh

This work is licensed under a Creative Commons Attribution 4.0 International License.
As a member of Publisher International Linking Association, PILA, iMedPub Group’s JOP follows the Creative Commons Attribution License and Scholars Open Access publishing policies. Journal of the Pancreas is the Council Contributor Member of Council of Science Editors (CSE) and following the CSE slogan Education, Ethics, and Evidence for Editors.