Abstract:

Ｏｂｊｅｃｔｉｖｅ：To summarize the clinical and pathological features of IgG4 related diseases (IgG4RD).
Ｍｅｔｈｏｄｏｌｏｇｙ：The clinical manifestations,pathological features,treatment and prognosis of IgG4RD patients admitted to Jinling hospital from 2012 to 2017 were analyzed retrospectively.
Ｒｅｓｕｌｔｓ：There were 26 male and 6 female with an average age of （573±138） years old.20/32 cases had organs swelling,and the most common affected organs were kidney (17/32),pancreas (7/32),submandibular gland (8/31),etc. Increase of serum IgG4 was in 29/29,peripheral blood eosinophils in 8/32,globulin in 21/32,IgG in 16/22,IgE in 17/22,and low serum C3 and C4 levels in 8/22.Renal damage was characterized by abnormal urine tests,enlarged kidneys and renal dysfunction.Compared with the no renal involvement group,there was more severe anemia and higher responder  in renal involvement group.Compared with the normal complement group,the IgG4 level was higher in the low complement group.12 patients underwent renal biopsy,the histological diagnosis was IgG4related tubulointerstitial nephritis (TIN) in 10 cases,membranous glomerulonephropathy  in 2 cases.15 cases with renal involvement treated with glucocorticoid or immunosuppressive agents all showed improvement in urine test or renal function
Ｃｏｎｃｌｕｓｉｏｎ：
IgG4RD is a multisystemic inflammatory disease with multiple clinical manifestations,and renal involvement is mainly characterized by TIN.Most patients respond well to glucocorticoid therapy.

Key words: IgG4-related diseases, tubulointerstitial nephritis, membranous glomerulonephropathy, treatment