IgG4-related sclerosing disease: autoimmune pancreatitis and extrapancreatic manifestations

Paraskevi A Vlachou, Korosh Khalili, Hyun-Jung Jang, Sandra Fischer, Gideon M Hirschfield, Tae Kyoung Kim

Research output: Contribution to journalArticlepeer-review

139 Citations (Scopus)


Autoimmune pancreatitis is the pancreatic manifestation of IgG4-related sclerosing disease, which recently was recognized as a distinct disease entity. Numerous extrapancreatic organs, such as the bile ducts, gallbladder, kidneys, retroperitoneum, thyroid, salivary glands, lung, mediastinum, lymph nodes, and prostate may be involved, either synchronously or metachronously. Most cases of autoimmune pancreatitis are associated with elevated serum IgG4 levels; extensive IgG4-positive plasma cells; and infiltration of lymphocytes into various organs, which leads to fibrosis. There are several established diagnostic criteria systems that are used to diagnose autoimmune pancreatitis and that rely on a combination of imaging findings of the pancreas and other organs, serologic findings, pancreatic histologic findings, and response to corticosteroid therapy. It is important to recognize multiorgan involvement of IgG4-related sclerosing disease and be familiar with its clinical and imaging features because it demonstrates a favorable response to treatment.

Original languageEnglish
Pages (from-to)1379-402
Number of pages24
Issue number5
Publication statusPublished - 16 Sept 2011


  • Adolescent
  • Adrenal Cortex Hormones
  • Adult
  • Aged
  • Aged, 80 and over
  • Autoimmune Diseases
  • Blood Vessels
  • Cholangiopancreatography, Endoscopic Retrograde
  • Cholangiopancreatography, Magnetic Resonance
  • Cholangitis, Sclerosing
  • Diagnostic Imaging
  • Female
  • Humans
  • Hypergammaglobulinemia
  • Immunoglobulin G
  • Immunosuppressive Agents
  • Jaundice, Obstructive
  • Male
  • Middle Aged
  • Organ Specificity
  • Pancreatitis
  • Plasma Cells
  • Prostate
  • Retrospective Studies
  • Salivary Glands
  • Sclerosis
  • Tomography, X-Ray Computed
  • Viscera
  • Young Adult


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