BACKGROUND: IgG4-associated autoimmune diseases are systemic diseases affecting multiple organs of the body. Autoimmune pancreatitis, with a prevalence of 2.2 per 100,000 people, is one such disease. Because these multi-organ diseases present in highly variable ways, they were long thought just to affect individual organ systems. This only underscores the importance of familiarity with these diseases for routine clinical practice. METHODS: This review is based on pertinent articles retrieved by a selective search in PubMed, and on the published conclusions of international consensus conferences. RESULTS: The current scientific understanding of this group of diseases is based largely on case reports and small case series; there have not been any randomized controlled trials (RCTs) to date. Any organ system can be affected, including (for example) the biliary pathways, salivary glands, kidneys, lymph nodes, thyroid gland, and blood vessels. Macroscopically, these diseases cause diffuse organ swelling and the formation of pseudotumorous masses. Histopathologically, they are characterized by a lymphoplasmacytic infiltrate with IgG4-positive plasma cells, which leads via an autoimmune mechanism to the typical histologic findings--storiform fibrosis ("storiform" = whorled, like a straw mat) and obliterative, i.e., vessel-occluding, phlebitis. A mixed Th1 and Th2 immune response seems to play an important role in pathogenesis, while the role of IgG4 antibodies, which are not pathogenic in themselves, is still unclear. Glucocorticoid treatment leads to remission in 98% of cases and is usually continued for 12 months as maintenance therapy. Most patients undergo remission even if untreated. Steroid-resistant disease can be treated with immune modulators. CONCLUSION: IgG4-associated autoimmune diseases are becoming more common, but adequate, systematically obtained data are now available only from certain Asian countries. Interdisciplinary collaboration is a prerequisite to proper diagnosis and treatment. Treatment algorithms and RCTs are needed to point the way to organ-specific treatment in the future.
BACKGROUND: IgG4-associated autoimmune diseases are systemic diseases affecting multiple organs of the body. Autoimmune pancreatitis, with a prevalence of 2.2 per 100,000 people, is one such disease. Because these multi-organ diseases present in highly variable ways, they were long thought just to affect individual organ systems. This only underscores the importance of familiarity with these diseases for routine clinical practice. METHODS: This review is based on pertinent articles retrieved by a selective search in PubMed, and on the published conclusions of international consensus conferences. RESULTS: The current scientific understanding of this group of diseases is based largely on case reports and small case series; there have not been any randomized controlled trials (RCTs) to date. Any organ system can be affected, including (for example) the biliary pathways, salivary glands, kidneys, lymph nodes, thyroid gland, and blood vessels. Macroscopically, these diseases cause diffuse organ swelling and the formation of pseudotumorous masses. Histopathologically, they are characterized by a lymphoplasmacytic infiltrate with IgG4-positive plasma cells, which leads via an autoimmune mechanism to the typical histologic findings--storiform fibrosis ("storiform" = whorled, like a straw mat) and obliterative, i.e., vessel-occluding, phlebitis. A mixed Th1 and Th2 immune response seems to play an important role in pathogenesis, while the role of IgG4 antibodies, which are not pathogenic in themselves, is still unclear. Glucocorticoid treatment leads to remission in 98% of cases and is usually continued for 12 months as maintenance therapy. Most patients undergo remission even if untreated. Steroid-resistant disease can be treated with immune modulators. CONCLUSION: IgG4-associated autoimmune diseases are becoming more common, but adequate, systematically obtained data are now available only from certain Asian countries. Interdisciplinary collaboration is a prerequisite to proper diagnosis and treatment. Treatment algorithms and RCTs are needed to point the way to organ-specific treatment in the future.
Authors: Terumi Kamisawa; Suresh T Chari; Samuel A Giday; Myung-Hwan Kim; Jae Bock Chung; Kyu Taek Lee; Jens Werner; Frank Bergmann; Markus M Lerch; Julia Mayerle; Tilman Pickartz; Matthias Lohr; Alexander Schneider; Luca Frulloni; George J M Webster; D Nageshwar Reddy; Wei-Chih Liao; Hsiu-Po Wang; Kazuichi Okazaki; Tooru Shimosegawa; Guenter Kloeppel; Vay Liang W Go Journal: Pancreas Date: 2011-08 Impact factor: 3.327
Authors: Vikram Deshpande; Arezou Khosroshahi; Gunnlaugur P Nielsen; Daniel L Hamilos; John H Stone Journal: Am J Surg Pathol Date: 2011-05 Impact factor: 6.394
Authors: Yazid J Resheq; Alexander Quaas; Daniel von Renteln; Christoph Schramm; Ansgar W Lohse; Stefan Lüth Journal: Dig Liver Dis Date: 2013-04-19 Impact factor: 4.088
Authors: T Kamisawa; T Shimosegawa; K Okazaki; T Nishino; H Watanabe; A Kanno; F Okumura; T Nishikawa; K Kobayashi; T Ichiya; H Takatori; K Yamakita; K Kubota; H Hamano; K Okamura; K Hirano; T Ito; S B H Ko; M Omata Journal: Gut Date: 2009-04-26 Impact factor: 23.059