Autoimmune pancreatitis is an exceptional autoimmune condition. It is a rare form of chronic pancreatitis affecting men more often than women. Dr. Lizhi Zhang and Dr. Thomas C. Smyrk of the Mayo Clinic, Rochester, Minnesota, provided a review.
The diagnosis of autoimmune pancreatitis was first proposed in 1995, although a 1961 report identified the primary inflammatory nature of the condition.
Autoimmune pancreatitis (AIP) is difficult to distinguish from pancreatic cancer, Zhang and Smyrk reported. In fact, many cases have been diagnosed during surgery for what the doctors thought was cancer.
Symptoms of AIP include abdominal pain, weight loss, and jaundice. It may be associated with other autoimmune conditions including Sjogren syndrome and inflammatory bowel disease. When multiple organs are affected, the diagnosis is IgG4-related systemic disease.
The Mayo Clinic developed diagnostic criteria summarized by the nmemonic HISORt, which stands for Histology, Imaging, Serology, other Organ involvement, and Response to corticosteroid therapy.