IgG4-related sclerosing disease

Table 1 Clinicopathological findings of IgG4-related sclerosing disease

Clinicopathological findings
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