Systemic disease characterized histopathologically by extensive IgG4-positive plasma cell infiltration of various organs together with T lymphocytes |
Major clinical manifestations are apparent in the organs in which tissues fibrosis with obstructive phlebitis is pathologically induced |
Pancreas |
Autoimmune pancreatitis |
Bile duct |
IgG4-related sclerosing cholangitis |
Gallbladder |
IgG4-related sclerosing cholangitis |
Salivary gland |
IgG4-related sclerosing cholangitis |
Retroperitoneum |
IgG4-related retroperitoneal fibrosis |
Kidney |
IgG4-related tubulointerstitial nephritis |
Lung |
IgG4-related interstitial pneumonia |
Prostate |
IgG4-related prostatitis |
Some inflammatory pseudotumors (liver, lung and hypophysis) may be involved in this disease |
Occasional association with lymphadenopathy |
Elderly male preponderance |
Frequent elevation of serum IgG4 levels |
Favorite response to steroid therapy |
Differentiation from malignant tumor is important |
Precise pathogenesis and pathophysiology remain unclear |