Autoimmune pancreatitis
Type of chronic pancreatitis / From Wikipedia, the free encyclopedia
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Autoimmune Pancreatitis (AIP) is an increasingly recognized type of chronic pancreatitis that can be difficult to distinguish from pancreatic carcinoma but which responds to treatment with corticosteroids, particularly prednisone.[1] Although autoimmune pancreatitis is quite rare, it constitutes an important clinical problem for both patients and their clinicians: the disease commonly presents itself as a tumorous mass which is diagnostically indistinguishable from pancreatic cancer, a disease that is much more common in addition to being very dangerous. Hence, some patients undergo pancreatic surgery, which is associated to substantial mortality and morbidity, out of the fear by patients and clinicians to undertreat a malignancy. However, surgery is not a good treatment for this condition as AIP responds well to immunosuppressive treatment. There are two categories of AIP: Type 1 and Type 2, each with distinct clinical profiles.
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Autoimmune Pancreatitis | |
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Other names | AIP |
Specialty | Gastroenterology |
Symptoms | Painless jaundice, pancreatic mass |
Types | Type 1 and Type 2 |
Causes | IgG4-related disease |
Diagnostic method | Biopsy, imaging, serology |
Differential diagnosis | Pancreatic cancer |
Treatment | Corticosteroids (first line), azathioprine, rituximab |
Type 1 AIP is now regarded as a manifestation of IgG4-related disease,[2] and those affected have tended to be older and to have a high relapse rate. Type 1 pancreatitis, is as such as manifestation of IgG4 disease, which may also affect bile ducts in the liver, salivary glands, kidneys and lymph nodes. Type 2 AIP seems to affect only the pancreas, although about one-third of people with type 2 AIP have associated inflammatory bowel disease. AIP occurring in association with an autoimmune disorder has been referred to as "secondary" or "syndromic" AIP. AIP does not affect long-term survival.[3]