Autoimmune pancreatitis: Difference between revisions
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{{Infobox medical condition | |||
| name = Autoimmune pancreatitis | |||
| image = [[File:Diffuse_autoimmune_pancreatitis.jpg|250px]] | |||
| caption = Diffuse autoimmune pancreatitis | |||
| field = [[Gastroenterology]] | |||
| synonyms = AIP | |||
| symptoms = [[Jaundice]], [[abdominal pain]], [[weight loss]], [[diabetes mellitus]] | |||
| complications = [[Pancreatic cancer]], [[bile duct obstruction]], [[diabetes mellitus]] | |||
| onset = Typically in [[middle age]] | |||
| duration = Chronic | |||
| types = Type 1 (lymphoplasmacytic sclerosing pancreatitis), Type 2 (idiopathic duct-centric pancreatitis) | |||
| causes = [[Autoimmune disease]] | |||
| risks = [[IgG4-related disease]], [[other autoimmune diseases]] | |||
| diagnosis = [[Imaging studies]], [[serology]], [[biopsy]] | |||
| differential = [[Pancreatic cancer]], [[chronic pancreatitis]] | |||
| treatment = [[Corticosteroids]], [[immunosuppressive drugs]] | |||
| medication = [[Prednisone]], [[azathioprine]] | |||
| prognosis = Generally good with treatment | |||
| frequency = Rare | |||
}} | |||
{{Short description|A form of pancreatitis caused by the body's immune system attacking the pancreas}} | {{Short description|A form of pancreatitis caused by the body's immune system attacking the pancreas}} | ||
'''Autoimmune pancreatitis''' (AIP) is a rare form of [[chronic pancreatitis]] characterized by an autoimmune inflammatory process that affects the [[pancreas]]. It is part of a spectrum of [[autoimmune diseases]] and is often associated with other systemic autoimmune conditions. | '''Autoimmune pancreatitis''' (AIP) is a rare form of [[chronic pancreatitis]] characterized by an autoimmune inflammatory process that affects the [[pancreas]]. It is part of a spectrum of [[autoimmune diseases]] and is often associated with other systemic autoimmune conditions. | ||
==Classification== | ==Classification== | ||
Autoimmune pancreatitis is classified into two types: | Autoimmune pancreatitis is classified into two types: | ||
* '''Type 1 AIP''': Also known as [[lymphoplasmacytic sclerosing pancreatitis]], this type is associated with [[IgG4-related disease]]. It is characterized by elevated serum [[IgG4]] levels and the presence of IgG4-positive [[plasma cells]] in the pancreas. | * '''Type 1 AIP''': Also known as [[lymphoplasmacytic sclerosing pancreatitis]], this type is associated with [[IgG4-related disease]]. It is characterized by elevated serum [[IgG4]] levels and the presence of IgG4-positive [[plasma cells]] in the pancreas. | ||
* '''Type 2 AIP''': Also known as [[idiopathic duct-centric pancreatitis]], this type is not associated with IgG4-related disease. It is characterized by [[granulocytic epithelial lesions]] and is often associated with [[inflammatory bowel disease]], such as [[ulcerative colitis]]. | * '''Type 2 AIP''': Also known as [[idiopathic duct-centric pancreatitis]], this type is not associated with IgG4-related disease. It is characterized by [[granulocytic epithelial lesions]] and is often associated with [[inflammatory bowel disease]], such as [[ulcerative colitis]]. | ||
==Signs and symptoms== | ==Signs and symptoms== | ||
Patients with autoimmune pancreatitis may present with a variety of symptoms, including: | Patients with autoimmune pancreatitis may present with a variety of symptoms, including: | ||
* [[Jaundice]] due to bile duct obstruction | * [[Jaundice]] due to bile duct obstruction | ||
* [[Abdominal pain]] | * [[Abdominal pain]] | ||
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* [[Diabetes mellitus]] | * [[Diabetes mellitus]] | ||
* [[Pancreatic insufficiency]] leading to [[steatorrhea]] | * [[Pancreatic insufficiency]] leading to [[steatorrhea]] | ||
==Diagnosis== | ==Diagnosis== | ||
The diagnosis of autoimmune pancreatitis is based on a combination of clinical, serological, radiological, and histological findings. Key diagnostic criteria include: | The diagnosis of autoimmune pancreatitis is based on a combination of clinical, serological, radiological, and histological findings. Key diagnostic criteria include: | ||
* Elevated serum IgG4 levels (particularly in Type 1 AIP) | * Elevated serum IgG4 levels (particularly in Type 1 AIP) | ||
* Characteristic imaging findings on [[CT scan]] or [[MRI]], such as diffuse or focal pancreatic enlargement and a "sausage-shaped" pancreas | * Characteristic imaging findings on [[CT scan]] or [[MRI]], such as diffuse or focal pancreatic enlargement and a "sausage-shaped" pancreas | ||
* Histological examination showing lymphoplasmacytic infiltration and fibrosis | * Histological examination showing lymphoplasmacytic infiltration and fibrosis | ||
* Response to [[corticosteroid]] therapy | * Response to [[corticosteroid]] therapy | ||
==Treatment== | ==Treatment== | ||
The primary treatment for autoimmune pancreatitis is corticosteroid therapy, which typically leads to rapid improvement in symptoms and imaging findings. In some cases, additional immunosuppressive agents such as [[azathioprine]] or [[mycophenolate mofetil]] may be used to maintain remission. | The primary treatment for autoimmune pancreatitis is corticosteroid therapy, which typically leads to rapid improvement in symptoms and imaging findings. In some cases, additional immunosuppressive agents such as [[azathioprine]] or [[mycophenolate mofetil]] may be used to maintain remission. | ||
==Prognosis== | ==Prognosis== | ||
With appropriate treatment, the prognosis for autoimmune pancreatitis is generally good. However, relapses can occur, and long-term follow-up is necessary to monitor for recurrence and manage any associated autoimmune conditions. | With appropriate treatment, the prognosis for autoimmune pancreatitis is generally good. However, relapses can occur, and long-term follow-up is necessary to monitor for recurrence and manage any associated autoimmune conditions. | ||
==See also== | |||
== | |||
* [[Pancreatitis]] | * [[Pancreatitis]] | ||
* [[Chronic pancreatitis]] | * [[Chronic pancreatitis]] | ||
* [[IgG4-related disease]] | * [[IgG4-related disease]] | ||
* [[Autoimmune disease]] | * [[Autoimmune disease]] | ||
[[Category:Autoimmune diseases]] | [[Category:Autoimmune diseases]] | ||
[[Category:Pancreatic disorders]] | [[Category:Pancreatic disorders]] | ||
Latest revision as of 17:08, 4 April 2025

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC
| Autoimmune pancreatitis | |
|---|---|
| Synonyms | AIP |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Jaundice, abdominal pain, weight loss, diabetes mellitus |
| Complications | Pancreatic cancer, bile duct obstruction, diabetes mellitus |
| Onset | Typically in middle age |
| Duration | Chronic |
| Types | Type 1 (lymphoplasmacytic sclerosing pancreatitis), Type 2 (idiopathic duct-centric pancreatitis) |
| Causes | Autoimmune disease |
| Risks | IgG4-related disease, other autoimmune diseases |
| Diagnosis | Imaging studies, serology, biopsy |
| Differential diagnosis | Pancreatic cancer, chronic pancreatitis |
| Prevention | N/A |
| Treatment | Corticosteroids, immunosuppressive drugs |
| Medication | Prednisone, azathioprine |
| Prognosis | Generally good with treatment |
| Frequency | Rare |
| Deaths | N/A |
A form of pancreatitis caused by the body's immune system attacking the pancreas
Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis characterized by an autoimmune inflammatory process that affects the pancreas. It is part of a spectrum of autoimmune diseases and is often associated with other systemic autoimmune conditions.
Classification[edit]
Autoimmune pancreatitis is classified into two types:
- Type 1 AIP: Also known as lymphoplasmacytic sclerosing pancreatitis, this type is associated with IgG4-related disease. It is characterized by elevated serum IgG4 levels and the presence of IgG4-positive plasma cells in the pancreas.
- Type 2 AIP: Also known as idiopathic duct-centric pancreatitis, this type is not associated with IgG4-related disease. It is characterized by granulocytic epithelial lesions and is often associated with inflammatory bowel disease, such as ulcerative colitis.
Signs and symptoms[edit]
Patients with autoimmune pancreatitis may present with a variety of symptoms, including:
- Jaundice due to bile duct obstruction
- Abdominal pain
- Weight loss
- Diabetes mellitus
- Pancreatic insufficiency leading to steatorrhea
Diagnosis[edit]
The diagnosis of autoimmune pancreatitis is based on a combination of clinical, serological, radiological, and histological findings. Key diagnostic criteria include:
- Elevated serum IgG4 levels (particularly in Type 1 AIP)
- Characteristic imaging findings on CT scan or MRI, such as diffuse or focal pancreatic enlargement and a "sausage-shaped" pancreas
- Histological examination showing lymphoplasmacytic infiltration and fibrosis
- Response to corticosteroid therapy
Treatment[edit]
The primary treatment for autoimmune pancreatitis is corticosteroid therapy, which typically leads to rapid improvement in symptoms and imaging findings. In some cases, additional immunosuppressive agents such as azathioprine or mycophenolate mofetil may be used to maintain remission.
Prognosis[edit]
With appropriate treatment, the prognosis for autoimmune pancreatitis is generally good. However, relapses can occur, and long-term follow-up is necessary to monitor for recurrence and manage any associated autoimmune conditions.