Clinicopathological Features And Prognosis Of Kimura's Disease With Renal Involvement In Chinese Patients

Objective:Kimura's disease(KD)with renal involvement is a rare disease.Optimal treatments are still not well established.It is necessary to analyze clinicopathological features,treatment responses and prognosis for improving KD diagnosis and treatment.Methodology:Clinicopathological data,treatment responses and prognosis were collected and analyzed retrospectively.Results:The patients consisted of 27 males and 2 females and the median age was 40 year(20.50?47.00)years.Twenty-seven exhibited proteinuria and the the median of proteinuria was 6.47g/24h(3.17g/24h?8.26g/24h).Hypertension,renal insufficiency[serum creatinine(Ser)>1.24 mg/dl],microhematuria occurred in 4(13.8%),11(37.9%),13(44.8%)cases respectively.Light microscopy(LM)identified mesangium proliferation,minimal change,FSGS,membranous glomerulonephritis,membranoproliferative glomerulonephritis(MPGN)and acute tubular necrosis in 14,8,3,2,1,1 case respectively.All were treated with Tripterygium wilfordii(TW),prednisone,leflunomide(LEF),tacrolimus(FK506),myophenolate mofetil(MMF),and/or renin-angiotensin system blockers(RASI).Twenty-six patients were followed up and the median of follow-up time was 40.52 months(10.71-59.00).After treatments,urinary red blood cells(RBC)decreased in all cases.The amount of 24-hour urinary protein(24-hUPE)decreased in 24 patients.Twenty-two reached complete remission(CR),four partial remissions(PR).The patients who never relapsed were younger than those relapsed.Conclusion:KD with renal involvement occurs predominantly among 35-50 years old Chinese patients with male predilection.The most common features are proteinuria,hypertension,micro hematuria with minimal change and mesangial proliferative glomerulonephritis.Most were responsive to treatment,but could relapse.Age,hypertension,Blood urea nitrogen(BUN)and Scr are associated with recurrence.The prognosis is good mostly.