Autoimmune Pancreatitis (AIP) is a peculiar form of pancreatitis different from all other type of pancreatitis ('one like no one pancreatitis') and characterized by a dramatic response to steroids. AIP can be classified histologically in type 1, included in a larger group of diseases named IgG4-related disease, and type 2. At imaging, AIP may involve all (diffuse form) or only a part (focal form) of the pancreatic gland. Areas covered: In this article, the clinical approach to diagnosis and different therapeutic strategies of this complicated disease are reviewed. Literature search was undertaken using PubMed database entering autoimmune pancreatitis [title] or IgG4-related diseases [title], and selecting the more relevant papers for diagnosis and therapy of AIP in clinical practice. We focus on diagnosis of AIP in focal and diffuse form, and how to achieve diagnosis for type 1 and type 2 AIP. We finally analyzed the different strategies proposed in induction of remission and maintenance therapy (long-term low-dose steroids, immunosuppressants or biologics). Expert commentary: The main issue in clinical practice is how to achieve the diagnosis. A second key point is how to prevent the disease relapse.
Keywords: Autoimmune pancreatitis; clinical management; diagnosis; maintenance therapy; remission.