Abstract
IgG4-related disease is an emerging pathological condition characterized by one or several fibrosing and inflammatory organ involvements. Histological findings are typical and associate storiform fibrosis with polyclonal lymphocytic and plasma-cell infiltrate, with predominant IgG4-expressing plasma cells. Sclerosing lymphoplasmocytic pancreatitis, or type 1 auto-immune pancreatitis, and other organ involvements have been reported: sclerosing cholangitis, sialadenitis, dacryoadenitis, retroperitoneal fibrosis, aortitis, interstitial nephritis, polyadenopathy and inflammatory pseudo-tumors. Serum IgG4 level is elevated in most of patients, but the histological documentation remain necessary for the diagnosis. Dramatic response to steroids is usual but relapses are frequent.
MeSH terms
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Autoimmune Diseases / diagnosis
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Autoimmune Diseases / epidemiology
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Autoimmune Diseases / immunology*
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Autoimmune Diseases / therapy
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Cholangitis / diagnosis
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Cholangitis / epidemiology
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Cholangitis / pathology
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Cholangitis / therapy
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Humans
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Immunoglobulin G / immunology
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Immunoglobulin G / physiology*
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Models, Biological
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Nephritis, Interstitial / diagnosis
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Nephritis, Interstitial / epidemiology
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Nephritis, Interstitial / pathology
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Nephritis, Interstitial / therapy
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Pancreatitis / diagnosis
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Pancreatitis / epidemiology
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Pancreatitis / etiology
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Pancreatitis / therapy
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Retroperitoneal Fibrosis / diagnosis
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Retroperitoneal Fibrosis / epidemiology
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Retroperitoneal Fibrosis / etiology
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Retroperitoneal Fibrosis / therapy
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Sclerosis / diagnosis
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Sclerosis / epidemiology
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Sclerosis / pathology
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Sclerosis / therapy
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Sialadenitis / diagnosis
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Sialadenitis / epidemiology
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Sialadenitis / etiology
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Sialadenitis / therapy