IgG4-related autoimmune diseases: Polymorphous presentation complicates diagnosis and treatment

Deutsches Ärzteblatt International
Alexander KlegerJulia Mayerle

Abstract

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. This review is based on pertinent articles retrieved by a selective search in PubMed, and on the published conclusions of international consensus conferences. 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" ...Continue Reading

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Jul 17, 2015·Deutsches Ärzteblatt International·Thomas Seufferlein
Jul 17, 2015·Deutsches Ärzteblatt International·Orlando Guntinas-Lichius, Stephan Ihrler
Jul 27, 2021·Der Internist·Dominique EichstädtGötz von Wichert

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