IgG4 related disease in pancreas, bile duct and liver

نویسنده

  • Ming-Chu Chang
چکیده

IgG4-related disease is a unique systemic inflammatory condition characterized by pseudotumorous formation or swelling of affected organs and high-serum IgG4 concentrations. Further studies have demonstrated that IgG4-related disease can involve a variety of organs including the pancreas ( type 1 autoimmune pancreatitis) , bile duct ( IgG4-related sclerosing cholangitis), liver (IgG4-related hepatitis and inflammatory pseudotumor), gallbladder (IgG4-related cholecystitis), salivary gland (IgG4-related sialadenitis),retroperitoeneum (IgG4-related retroperitoneal fibrosis), kidney (IgG4-related tubulointerstitial nephritis), lung (IgG4-related interstitial pneumonia, inflammatory pseudotumor), prostate (IgG4-related prostatitis), lymph node (IgG4-related lymphadenopathy) et al. Presence of typical histopathological features, including the existence of numerous IgG4 positive plasma cells within affected tissue, is the gold standard for the diagnosis of IgG4-related disease. Responsiveness to glucocorticoid is particularly characteristic early in the disease course, before the onset of significant tissue fibrosis. Increasing prevalence of IgG4 disease is reported worldwide.

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تاریخ انتشار 2012