Biliary Involvement in Type 2 Autoimmune Pancreatitis
Autoimmune pancreatitis (AIP) is a rare condition classified in 2 subtypes. Their distinction relies on a combination of clinical, serological, morphological and histological features. Type 1 is a pancreatic manifestation of IgG4-related disease characterized by multiorgan infiltration by IgG4 plasm...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Karger Publishers
2019-04-01
|
| Series: | Case Reports in Gastroenterology |
| Subjects: | |
| Online Access: | https://www.karger.com/Article/FullText/499422 |
| _version_ | 1828802265927909376 |
|---|---|
| author | Diana Ollo Sylvain Terraz Gregoire Arnoux Giacomo Puppa Jean-Louis Frossard Philippe Bichard |
| author_facet | Diana Ollo Sylvain Terraz Gregoire Arnoux Giacomo Puppa Jean-Louis Frossard Philippe Bichard |
| author_sort | Diana Ollo |
| collection | DOAJ |
| description | Autoimmune pancreatitis (AIP) is a rare condition classified in 2 subtypes. Their distinction relies on a combination of clinical, serological, morphological and histological features. Type 1 is a pancreatic manifestation of IgG4-related disease characterized by multiorgan infiltration by IgG4 plasmocytes. In this condition, hepatobiliary infiltration is frequent and often mimics cholangiocarcinoma or primary sclerosing cholangitis. On the other hand, type 2 is commonly limited to the pancreas. Herein, we describe the case of a patient who presented a type 2 AIP associated with cholangiopathy, a condition not described in the established criteria. He first developed a pancreatitis identified as type 2 by the typical histopathological features and lack of IgG4 in the serum and tissue. Despite a good clinical response to steroids, cholestasis persisted, identified by MR cholangiography as a stricture of the left hepatic duct with dilatation of the intrahepatic bile duct in segments 2 and 3. Biliary cytology was negative. Evolution was favorable but after steroid tapering a few months later, the patient suffered from recurrence of the pancreatitis as well as progression of biliary attempt, suspicious for cholangiocarcinoma. As the investigations again ruled out neoplastic infiltration or primary sclerosing cholangitis, azathioprine was initiated with resolution of both pancreatic and biliary attempts. |
| first_indexed | 2024-12-12T07:01:01Z |
| format | Article |
| id | doaj.art-c205bf4242dc4645bd2cf9e5775fcdaa |
| institution | Directory Open Access Journal |
| issn | 1662-0631 |
| language | English |
| last_indexed | 2024-12-12T07:01:01Z |
| publishDate | 2019-04-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Gastroenterology |
| spelling | doaj.art-c205bf4242dc4645bd2cf9e5775fcdaa2022-12-22T00:33:51ZengKarger PublishersCase Reports in Gastroenterology1662-06312019-04-0113120020610.1159/000499422499422Biliary Involvement in Type 2 Autoimmune PancreatitisDiana OlloSylvain TerrazGregoire ArnouxGiacomo PuppaJean-Louis FrossardPhilippe BichardAutoimmune pancreatitis (AIP) is a rare condition classified in 2 subtypes. Their distinction relies on a combination of clinical, serological, morphological and histological features. Type 1 is a pancreatic manifestation of IgG4-related disease characterized by multiorgan infiltration by IgG4 plasmocytes. In this condition, hepatobiliary infiltration is frequent and often mimics cholangiocarcinoma or primary sclerosing cholangitis. On the other hand, type 2 is commonly limited to the pancreas. Herein, we describe the case of a patient who presented a type 2 AIP associated with cholangiopathy, a condition not described in the established criteria. He first developed a pancreatitis identified as type 2 by the typical histopathological features and lack of IgG4 in the serum and tissue. Despite a good clinical response to steroids, cholestasis persisted, identified by MR cholangiography as a stricture of the left hepatic duct with dilatation of the intrahepatic bile duct in segments 2 and 3. Biliary cytology was negative. Evolution was favorable but after steroid tapering a few months later, the patient suffered from recurrence of the pancreatitis as well as progression of biliary attempt, suspicious for cholangiocarcinoma. As the investigations again ruled out neoplastic infiltration or primary sclerosing cholangitis, azathioprine was initiated with resolution of both pancreatic and biliary attempts.https://www.karger.com/Article/FullText/499422Biliary involvementAutoimmune pancreatitis |
| spellingShingle | Diana Ollo Sylvain Terraz Gregoire Arnoux Giacomo Puppa Jean-Louis Frossard Philippe Bichard Biliary Involvement in Type 2 Autoimmune Pancreatitis Case Reports in Gastroenterology Biliary involvement Autoimmune pancreatitis |
| title | Biliary Involvement in Type 2 Autoimmune Pancreatitis |
| title_full | Biliary Involvement in Type 2 Autoimmune Pancreatitis |
| title_fullStr | Biliary Involvement in Type 2 Autoimmune Pancreatitis |
| title_full_unstemmed | Biliary Involvement in Type 2 Autoimmune Pancreatitis |
| title_short | Biliary Involvement in Type 2 Autoimmune Pancreatitis |
| title_sort | biliary involvement in type 2 autoimmune pancreatitis |
| topic | Biliary involvement Autoimmune pancreatitis |
| url | https://www.karger.com/Article/FullText/499422 |
| work_keys_str_mv | AT dianaollo biliaryinvolvementintype2autoimmunepancreatitis AT sylvainterraz biliaryinvolvementintype2autoimmunepancreatitis AT gregoirearnoux biliaryinvolvementintype2autoimmunepancreatitis AT giacomopuppa biliaryinvolvementintype2autoimmunepancreatitis AT jeanlouisfrossard biliaryinvolvementintype2autoimmunepancreatitis AT philippebichard biliaryinvolvementintype2autoimmunepancreatitis |