Autoimmune Pancreatitis: An Update

Aravind Sugumar; Suresh Chari


Expert Rev Gastroenterol Hepatol. 2009;3(2):197-204. 

In This Article

Abstract and Introduction


Autoimmune pancreatitis (AIP) is the pancreatic manifestation of a systemic fibroinflammatory disorder. It has been recognized as a distinct clinical entity, only recently. Multiple organs, such bile ducts, salivary glands, kidneys and lymph nodes, can be involved either synchronously or metachronously. It is one of the few autoimmune conditions that predominantly affects male subjects in the fifth and sixth decades of life. Obstructive jaundice is the most common presenting symptom but the presentation can be quite nonspecific. There are established diagnostic criteria to diagnose AIP, most of which rely on a combination of clinical presentation, imaging of the pancreas and other organs (by CT scan, MRI and endoscopic retrograde pancreatography), serology, pancreatic histology and response to steroids to make the diagnosis. It is imperative to differentiate AIP from pancreatic cancer owing to the vastly different prognostic and therapeutic implications. AIP responds dramatically to steroid treatment but relapses are common. Relapse of AIP can often be retreated with steroids. As the collective experience with this condition increases, a better understanding of the natural history of this disease is emerging.


Autoimmune pancreatitis (AIP) is a disease that is being increasingly recognized as a distinct clinical entity worldwide. While Sarles et al. may have been the first to describe the condition in 1961,[1] it was not until the early-to-mid-1990s that the name AIP was first suggested and the initial descriptions and case series published.[1,2,3,4] In 1991, a variant of primary sclerosing cholangitis (PSC) extensively involving the pancreas was described from Japan.[3] Subsequent reports and case series reported that, unlike PSC, this variant disease responded to steroids.[2] In 1995, Yoshida et al. described a typical case of AIP and highlighted 12 criteria suggestive of AIP.[4] A critical milestone in AIP was reached in 2001, when Hamano reported high serum IgG4 levels in AIP patients.[5] Subsequently, Kamisawa et al. described intense infiltration with IgG4-positive cells in AIP, not only in the pancreas but also multiple other organs.[6,7] These observations led them to suggest that AIP may be part of a systemic disease known as IgG4-associated disease (ISD).[8]


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