Background: Autoimmune pancreatitis, in comparison to other benign chronic pancreatic diseases, is characterized by the possibility of curing the illness with immunosuppressant drugs. The open question is whether to differentiate autoimmune pancreatitis as a primary or secondary disease based on the presence or absence of other autoimmune diseases or whether to consider autoimmune pancreatitis a clinical and pathological systemic entity, called IgG4-related sclerosing disease, since this aspect is also very important from a therapeutic point of view.
Methods: In this paper, we reviewed the conventional therapeutic approach used to treat autoimmune pancreatitis patients and the clinical outcome related to each treatment modality. We also reviewed some aspects which are important for the correct management of autoimmune pancreatitis, such as the surgical approach, the outcome of surgically treated autoimmune pancreatitis patients, whether medical treatment is always necessary, and, finally, when medical treatment should be initiated.
Conclusions: Steroids are useful in alleviating the symptoms of the acute presentation of autoimmune pancreatitis, but some questions remain open such as the dosage of steroids in the acute phase and the duration of steroid therapy; finally, it should be assessed if other immunosuppressive non-steroidal drugs may become the first-line therapy in patients with AIP without jaundice and without atrophic pancreas.