IgG-4-related disease is a clinical entity characterised by significant elevation in serum IgG-4 levels, infiltration of IgG-4+ plasma cells into the involved tissues, enhanced fibrosis, and good therapeutic response to corticosteroids. The IgG-4 associated disease mostly affects two organs. The salivary and lacrimal gland enlargement and inflammation is known as Mikulicz's disease, which had been previously known as a subtype of Sjogren's syndrome for a long time. The other commonly involved organ is the pancreas, in which a special form of chronic pancreatitis, namely autoimmune pancreatitis develops. IgG-4 associated disease as a separate disease has been suggested by Japanese authors. Previously published data support the common pathogenesis of autoimmune pancreatitis and Mikulicz's disease. Besides these two manifestations, similar histological lesions and elevated IgG-4 levels have been demonstrated in many other organs. At present, it is not clearly demonstrated whether IgG-4 syndrome is an autoimmune disorder, and we do not know the exact reason of the elevated IgG-4 levels in patients with this syndrome. IgG-4 is a regulatory immunoglobulin, the main function of which is to decelerate immune responses, thus its pathologic role in tissue destruction is difficult to explain. It is not clear either, whether IgG-4 syndrome is indeed a single disease, or only an example of overlapping symptoms of various diseases. On the basis of the characteristic histological lesions in various organs, IgG-4 syndrome is similar to multi-organ diseases, such as sarcoidosis or vasculitis.
|Translated title of the contribution||IgG4-related disease|
|Number of pages||5|
|Journal||Lege Artis Medicinae|
|Publication status||Published - Apr 1 2011|
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