Overlap syndrome of seronegative primary biliary cholangitis and small duct primary sclerosing cholangitis: a first case report and literature review
Primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and autoimmune hepatitis (AIH) are distinct liver diseases. Cases combining PBC and PSC, are extremely rare. Here, we present a case of a 39-year-old woman with a history of colonic Crohn's disease treated with azathioprin...
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| Format: | Article |
| Language: | English |
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Taylor & Francis Group
2024-12-01
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| Series: | Future Science OA |
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| Online Access: | https://www.tandfonline.com/doi/10.2144/fsoa-2023-0187 |
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| author | Salma Souissi Sarah Laabidi Nadia Ben Mustpha Ines Chelly Meriem Serghini Monia Fekih Asma Laabidi Jalel Boubaker |
| author_facet | Salma Souissi Sarah Laabidi Nadia Ben Mustpha Ines Chelly Meriem Serghini Monia Fekih Asma Laabidi Jalel Boubaker |
| author_sort | Salma Souissi |
| collection | DOAJ |
| description | Primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and autoimmune hepatitis (AIH) are distinct liver diseases. Cases combining PBC and PSC, are extremely rare. Here, we present a case of a 39-year-old woman with a history of colonic Crohn's disease treated with azathioprine. Discontinuation of the medication was prompted by abnormal liver function tests, but subsequent evaluations revealed persistent liver injury. Extensive diagnostic investigations, including imaging, serological tests, and liver biopsy, were conducted leading to a diagnosis of PBC-PSC overlap syndrome based on the presence of concentric lamellar fibrosis and chronic non-suppurative destructive cholangitis. The patient responded well to ursodeoxycholic acid treatment. This case emphasizes the importance of recognizing and diagnosing rare overlap syndromes, particularly those involving PBC and PSC, to ensure appropriate management and improve patient outcomes. |
| format | Article |
| id | doaj-art-5e496a3fc241430a94be4eb0049e00a7 |
| institution | Kabale University |
| issn | 2056-5623 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Future Science OA |
| spelling | doaj-art-5e496a3fc241430a94be4eb0049e00a72025-08-20T03:27:44ZengTaylor & Francis GroupFuture Science OA2056-56232024-12-0110110.2144/fsoa-2023-0187Overlap syndrome of seronegative primary biliary cholangitis and small duct primary sclerosing cholangitis: a first case report and literature reviewSalma Souissi0Sarah Laabidi1Nadia Ben Mustpha2Ines Chelly3Meriem Serghini4Monia Fekih5Asma Laabidi6Jalel Boubaker7Departement of Gastroenterology A, La Rabta Hospital, Tunis, 1007, TunisiaDepartement of Gastroenterology A, La Rabta Hospital, Tunis, 1007, TunisiaDepartement of Gastroenterology A, La Rabta Hospital, Tunis, 1007, TunisiaFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, 1007, TunisiaDepartement of Gastroenterology A, La Rabta Hospital, Tunis, 1007, TunisiaDepartement of Gastroenterology A, La Rabta Hospital, Tunis, 1007, TunisiaDepartement of Gastroenterology A, La Rabta Hospital, Tunis, 1007, TunisiaDepartement of Gastroenterology A, La Rabta Hospital, Tunis, 1007, TunisiaPrimary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and autoimmune hepatitis (AIH) are distinct liver diseases. Cases combining PBC and PSC, are extremely rare. Here, we present a case of a 39-year-old woman with a history of colonic Crohn's disease treated with azathioprine. Discontinuation of the medication was prompted by abnormal liver function tests, but subsequent evaluations revealed persistent liver injury. Extensive diagnostic investigations, including imaging, serological tests, and liver biopsy, were conducted leading to a diagnosis of PBC-PSC overlap syndrome based on the presence of concentric lamellar fibrosis and chronic non-suppurative destructive cholangitis. The patient responded well to ursodeoxycholic acid treatment. This case emphasizes the importance of recognizing and diagnosing rare overlap syndromes, particularly those involving PBC and PSC, to ensure appropriate management and improve patient outcomes.https://www.tandfonline.com/doi/10.2144/fsoa-2023-0187overlap syndromeprimary biliary cholangitisprimary sclerosing cholangitis |
| spellingShingle | Salma Souissi Sarah Laabidi Nadia Ben Mustpha Ines Chelly Meriem Serghini Monia Fekih Asma Laabidi Jalel Boubaker Overlap syndrome of seronegative primary biliary cholangitis and small duct primary sclerosing cholangitis: a first case report and literature review Future Science OA overlap syndrome primary biliary cholangitis primary sclerosing cholangitis |
| title | Overlap syndrome of seronegative primary biliary cholangitis and small duct primary sclerosing cholangitis: a first case report and literature review |
| title_full | Overlap syndrome of seronegative primary biliary cholangitis and small duct primary sclerosing cholangitis: a first case report and literature review |
| title_fullStr | Overlap syndrome of seronegative primary biliary cholangitis and small duct primary sclerosing cholangitis: a first case report and literature review |
| title_full_unstemmed | Overlap syndrome of seronegative primary biliary cholangitis and small duct primary sclerosing cholangitis: a first case report and literature review |
| title_short | Overlap syndrome of seronegative primary biliary cholangitis and small duct primary sclerosing cholangitis: a first case report and literature review |
| title_sort | overlap syndrome of seronegative primary biliary cholangitis and small duct primary sclerosing cholangitis a first case report and literature review |
| topic | overlap syndrome primary biliary cholangitis primary sclerosing cholangitis |
| url | https://www.tandfonline.com/doi/10.2144/fsoa-2023-0187 |
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