Background Sclerosing pancreatitis is a unique form of pancreatitis that is characterized by irregular narrowing of the main pancreatic duct, lymphoplasmacytic inflammation of the pancreas, and hypergammaglobulinemia and that responds to glucocorticoid treatment. Preliminary studies suggested that serum IgG4 concentrations are elevated in this disease but not in other diseases of the pancreas or biliary tract. Methods We measured serum IgG4 concentrations using single radial immunodiffusion and an enzyme-linked immunosorbent assay in 20 patients with sclerosing pancreatitis, 20 age- and sex-matched normal subjects, and 154 patients with pancreatic cancer, ordinary chronic pancreatitis, primary biliary cirrhosis, primary sclerosing cholangitis, or Sjogren's syndrome. Serum concentrations of immune complexes and the IgG4 subclass of immune complexes were determined by means of an enzyme-linked immunosorbent assay with monoclonal rheumatoid factor. Results The median serum IgG4 concentration in the patients ...

https://www.nejm.org/doi/pdf/10.1056/NEJM200103083441005

High serum IgG4 concentrations in patients with sclerosing pancreatitis.

Objectives:To achieve the goal of developing international consensus diagnostic criteria (ICDC) for autoimmune pancreatitis (AIP).Methods:An international panel of experts met during the 14th Congress of the International Association of Pancreatology held in Fukuoka, Japan, from July 11 through 13,

International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology.

https://www.cghjournal.org/article/S1542-3565(06)00486-1/pdf

Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience.

IgG4-related disease (IgG4-RD) is a novel clinical disease entity characterized by an elevated serum IgG4 concentration and tumefaction or tissue infiltration by IgG4-positive plasma cells. IgG4-RD encompasses a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), hypophysitis, Riedel thyroiditis, tubulointerstitial nephritis, prostatitis, retroperitoneal fibrosis, inflammatory aortic aneurysm and inflammatory pseudotumor. However, like a crow flying on a dark night, IgG4-RD passed through the history of medicine, until the concept of an overarching of these systemic disorders was established in Japan at the beginning of the present century. In addition, most clinical practitioners are not yet familiar with IgG4-RD, and thus one cannot diagnose what one does not know. The purpose of the book [1] is to raise awareness of this disease and its diagnostic pitfalls. Another goal was to delineate the characteristic features of individual organs in IgG4-RD radiologically and histopathologically with many color photographs. Most of the authors are members of the IgG4 team of the Japanese the Ministry of Health, Labor and Welfare (MHLW), and experts who have published numerous important papers in the field. Thus, this book will be useful to physicians in various disciplines such as gastroenterology, rheumatology, ophthalmology, otolaryngology, urology, hematology, respiratory medicine and oral medicine, not only as a textbook but also as an authoritative and comprehensive reference work.

IgG4-Related Disease

https://www.nejm.org/doi/pdf/10.1056/NEJMra1104650

IgG4-related disease

Several authors have reported a case of chronic pancreatitis associated with Sjogren's syndrome in which an autoimmune mechanism may have been involved in the etiology and in which steroid therapy was effective. We recently encountered a patient with pancreatitis who had hyperglobulinemia, was autoantibody-positive, and responded to steroid therapy. This patient, however, failed to show any evidence of association with Sjogren's syndrome or other collagen diseases. Although the concept of autoimmune hepatitis and the criteria for diagnosing it have been established, autoimmune pancreatitis has not yet been defined as a clinical entity. We report a case of chronic pancreatitis in which an autoimmune mechanism is involved in the etiology and summarize the cases of pancreatitis suspected of being caused by an autoimmune mechanism in the Japanese and English literature.

Chronic pancreatitis caused by an autoimmune abnormality : proposal of the concept of autoimmune pancreatitis

https://www.jstage.jst.go.jp/article/gee1973b/38/5/38_5_1208/_pdf

Kenji Yoshida

Papers

Chronic pancreatitis caused by an autoimmune abnormality : proposal of the concept of autoimmune pancreatitis

Two cases of pancreaticoduodenal fistula

A case of ulcerative colitis associated with pemphigus vulgaris and erythema nodosum