Abstract:

IgG4 related disease (IgG4RD) is a kind of chronic systemic autoimmune disease， whose etiology is unknown. The clinical spectrum of IgG4RD is wide. The disease always involves systemic glandular organs， retroperitoneum， kidney， pituitary gland and lymph nodes. IgG4RD is characterized by swelling and fibrosclerosing change of affected organs，elevated serum IgG4 level， a large number of  lymphocytes and IgG4 positive plasma cells infiltration in involved organ and tissue．The diagnosis is confirmed by biopsy， the histopathological features include: dense lymphocytes and plasmacytes infiltration， storiform fibrosis and sclerosis， obliterative phlebitis. Glucocorticoids are the firstline agent for remission induction，some IgG4RD patients require the use of immunosuppressive  drugs or biological agents.

Key words: immune system diseases, autoimmune diseases；pancreatitis；corticosteroids；immunosuppressive agents