IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis
IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a perva...
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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Gastrointestinal Medicine |
| Online Access: | http://dx.doi.org/10.1155/2015/591360 |
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| _version_ | 1849305092553965568 |
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| author | Allon Kahn Anitha D. Yadav M. Edwyn Harrison |
| author_facet | Allon Kahn Anitha D. Yadav M. Edwyn Harrison |
| author_sort | Allon Kahn |
| collection | DOAJ |
| description | IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention. |
| format | Article |
| id | doaj-art-0de34e9816b34353b691e0da77e8de7a |
| institution | Kabale University |
| issn | 2090-6528 2090-6536 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Gastrointestinal Medicine |
| spelling | doaj-art-0de34e9816b34353b691e0da77e8de7a2025-08-20T03:55:33ZengWileyCase Reports in Gastrointestinal Medicine2090-65282090-65362015-01-01201510.1155/2015/591360591360IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing CholangitisAllon Kahn0Anitha D. Yadav1M. Edwyn Harrison2Department of Medicine, Mayo Clinic, Scottsdale, AZ, USADivision of Gastroenterology and Hepatology, Mayo Clinic, Scottsdale, AZ, USADivision of Gastroenterology and Hepatology, Mayo Clinic, Scottsdale, AZ, USAIgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.http://dx.doi.org/10.1155/2015/591360 |
| spellingShingle | Allon Kahn Anitha D. Yadav M. Edwyn Harrison IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis Case Reports in Gastrointestinal Medicine |
| title | IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis |
| title_full | IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis |
| title_fullStr | IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis |
| title_full_unstemmed | IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis |
| title_short | IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis |
| title_sort | igg4 seronegative autoimmune pancreatitis and sclerosing cholangitis |
| url | http://dx.doi.org/10.1155/2015/591360 |
| work_keys_str_mv | AT allonkahn igg4seronegativeautoimmunepancreatitisandsclerosingcholangitis AT anithadyadav igg4seronegativeautoimmunepancreatitisandsclerosingcholangitis AT medwynharrison igg4seronegativeautoimmunepancreatitisandsclerosingcholangitis |