Analysis on urinary system lesions of IgG4-related disease / 中华肾脏病杂志

ABSTRACT

Objective To explore the clinical features of renal and urinary lesions in IgG4-related disease (IgG4-RD).Methods Clinical manifestation,laboratory profiles,iconography images,pathologic findings,treatment and prognosis of 6 IgG4-RD patients with renal and urinary system involvement from Peking Union Medical College Hospital during Aug 2010 to Dec 2011 were analyzed retrospectively.Results Six patients had renal and/or urinary lesions among IgG4-RD cases diagnosed in our hospital,including 4 males and 2 women,with median age of 59 years (36 to 72 years) and median disease course of 10.5 months.All the patients presented multiple organ involvement simultaneously.Urinary system lesion varied,including renal dysfunction,abdominal pain and edema.Hyperglobulinemia,elevated serum IgG (median 23.3 g/L) and IgG4 (median 4227.0 mg/L),tubular proteinuria were found in all the 6 patients,and elevated Scr (median 237 μmol/L) in 5 cases.Kidney CT image often showed renal swelling,hydronephrosis,multiple low density focus with attenuation and kidney atrophy.Renal pathology revealed interstitial inflammatory cells infiltration comprising predominantly plasma cells and lymphocytes,with a high prevalence of IgG4-positive cells often admixed with fibrosis,which fit the features of tubulointerstitial nephritis.Patients with IgG4-RD nephropathy presented good response to glucocorticoids.After therapy,the symptoms were improved and serum IgG,IgG4 and Scr decreased.Conclusions Renal and urinary lesions of IgG4-RD are heterogeneous in clinical manifestation,and are often complicated with various organ lesions.The feature of renal histopathology is tubulointerstitial nephritis infiltrated by plasma cells and lymphocytes with positive IgG4.Glucocorticoids treatment is effective for this disease.