IgG4-related kidney disease complicated with retroperitoneal fibrosis:A case report

BACKGROUND IgG4-related disease(IgG4-RD)is a chronic fibrotic disease mediated by immunity recognized by clinicians in recent years.When the kidney is involved,it is called IgG4-related kidney disease(IgG4-RKD).IgG4-related tubulointerstitial nephritis(IgG4-TIN)is a representative manifestation of IgG4-RKD.IgG4-TIN can cause obstructive nephropathy complicated by retroperitoneal fibrosis(RPF).Cases of IgG4-TIN complicated with RPF are rare.Glucocorticoids are the firstline therapeutic medication for IgG4-RD and can significantly improve renal function.CASE SUMMARY Herein,we report the case of a 56-year-old man with IgG4-RKD complicated with RPF.The patient presented to the hospital with complaints of elevated serum creatinine(Cr),nausea,and vomiting.During hospitalization,Cr was 1448.6μmol/L,and serum IgG4 was increased.A total abdominal computed tomography(CT)scan and enhanced CT scan obviously indicated RPF.Although this patient had a long course and renal insufficiency,we performed a kidney biopsy.Renal biopsy showed that the renal tubulointerstitium had focal plasma cell infiltration and increased lymphocyte infiltration accompanied by fibrosis.After combining the biopsy results with immunohistochemistry,it was found that the absolute number of positive IgG4+cells per high power field exceeded 10,and the ratio of IgG4/IgG was over 40%.Finally,the patient was diagnosed with IgG4-TIN complicated with RPF and given glucocorticoids as long-term maintenance therapy,helping him keep out of dialysis.After a follow-up of 19 mo,the patient had recovered well.Previous literature on IgG4-RKD and RPF was retrieved from PubMed to characterize the clinical and pathological features and to identify the diagnosis and treatment of IgG4-RKD.CONCLUSION Our case report demonstrates the clinical characteristics of IgG4-RKD complicated with RPF.Serum IgG4 is a favorable indicator for screening.Performing renal biopsy actively plays a vital role in diagnosis and treatment,even if the patient has a long course and manifests with renal insufficiency.It is remarkable to treat IgG4-RKD with glucocorticoids.Hence,early diagnosis and targeted therapy are essential for reversing renal function and improving extrarenal manifestations in patients with IgG4-RKD.

Clinical observation of 36 patients with chronic nephritis proteinuria treated with Zhuangshen Gujing decoction

Objective:Our goal was to examine the impact of Zhuangshen Gujing decoction on proteinuria associated with chronic nephritis.Methods:72 patients with chronic nephritis proteinuria were divided randomly into two groups;one group was treated with Zhuangshen Gujing(n=36;treatment group)and the other group was treated with irbesartan(n=36;control group).After 6 months’treatment,urine protein and renal function were evaluated.Results:Proteinuria resolved completely in four patients in the treatment group;complete disease resolution was observed only among three individuals in the control group.Of the 32 cases remaining in the treatment group,15 had substantial responses to Zhuangshen Gujing decoction,9 had partial responses,and 8 had no response to treatment;the overall the response rate was 73.33%.Among the 33 cases remaining in the control group,12 had a substantial response to irbesartan,5 had a partial response,and 16 had no response to treatment;the overall response rate among the controls was 57.67%(P=0.031).We also observed statistically significant differences with respect to quantitative comparisons of urinary protein at 24 days after a single course of treatment(P=0.001).Conclusion:Zhuangshen Gujing decoction is effective in treating chronic nephritis proteinuria.