Autoimmune pancreatitis

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Abstract

Autoimmune pancreatitis is a rare systemic fibrotic inflammatory disorder that can affect organs such as the bile ducts, salivary glands, and retroperitoneal lymph nodes, in addition to the pancreas. Morphological characteristics of autoimmune pancreatitis include a diffusely enlarged 'sausage-shaped' pancreas and an irregularly narrowed duct of Wirsung. According to the revised Japan Pancreas Society criteria, the diagnosis of autoimmune pancreatitis requires that one or more secondary serologic or histologic criteria are also met: the presence of autoantibodies, elevated levels of γ-globulins, IgG or IgG4, a lymphoplasmacytic infiltrate, or pancreatic fibrosis. The presence in any affected organ of a lymphoplasmacytic inflammatory infiltrate containing greater than 10 IgG4-positive cells per high-power field is pathognomonic for autoimmune pancreatitis. Precise data on the incidence and prevalence of autoimmune pancreatitis are currently not available because most reports involve either limited patient series or resection specimen cohorts. New diagnostic tools and further studies of the underlying pathophysiology and prognosis of autoimmune pancreatitis are needed for adequate and effective treatment strategies to be developed. The most crucial issue when caring for patients with suspected autoimmune pancreatitis is to differentiate autoimmune pancreatitis from pancreatic carcinoma, because pancreatic carcinoma requires surgery, whereas autoimmune pancreatitis responds well to steroid treatment.

Key Points

  • The appearance of autoimmune pancreatitis (AIP) on imaging studies can mimic that of pancreatic cancer

  • At present, diagnostic criteria for AIP are constantly being updated to take new findings into account and are far from being firmly established

  • Data indicate that the autoantibody profile of AIP might be very different in patients from the West compared with those from Japan and Korea

  • Endoscopic ultrasound-guided Tru-cut® biopsy seems to be more effective than fine-needle aspiration cytology as an aid to the diagnosis of AIP, but it is not universally available

  • AIP responds well to conventional steroid therapy, but no controlled treatment trials are currently available to help predict whether a patient is suitable for, and likely respond to, therapy

  • International patient registries and further controlled studies are needed to raise awareness of AIP and to collect data on the course of the disease and its possible long-term complications

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Figure 1: Imaging and histologic features associated with autoimmune pancreatitis
Figure 2: An algorithm for the diagnosis and subsequent therapy of patients with suspected AIP used at Greifswald University Hospital

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Acknowledgements

We are most grateful to Helmut Friess and Jörg Kleeff for providing us with radiographic images of autoimmune pancreatitis and to Heinz Pickartz for providing us with histology micrographs of autoimmune pancreatitis.

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Correspondence to Markus M Lerch.

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The authors declare no competing financial interests.

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