Clinicopathological Characteristics And Outcome Of Lupus Nephritis With Anti-neutrophil Cytoplasmic Antibody Positivity

Part ?:Clinicopathological characteristics and outcome of lupus nephritis with anti-neutrophil cytoplasmic antibody positivityObjective:Analyze the clinical features,pathological presentations,and outcome of lupus nephritis(LN)with anti-neutrophil cytoplasmic antibody(ANCA)positivity.Methodology:We searched the pathology database at our institution for cases that showed LN with ANCA positivity from January 1,1985 to December 31,2014.According to ANCA antigen specificity,we divided patients into MPO-ANCA LN group and PR3-ANCA LN group.We compared clinicopathological features,treatment and outcome of LN with ANCA positivity of different types.Results:The cohort consisted of 88 women and 11 men with an average age of 34(IQR,25-43)years at kidney biopsy,including 69 MPO-ANCA,26 PR3-ANCA and 4 with double positivity.LN with ANCA positivity exhibited obvious hematuria(162 x 104/mL),serum creatinine levels(92.8 ?mol/L),and SLE-DAI scores(15 scores).On pathological evaluation,Class IV LN was predominant,accounting for 50.5%.Light microscopy revealed significantly higher scores of cellular crescents,interstitial inflammation,and fibrous crescent.LN with MPO-ANCA positivity exhibited higher serum creatinine levels(109.6 ?mol/L vs.74.3 ?mol/L,P = 0.02)than LN with PR3-ANCA positivity.Light microscopy revealed greater abundance of CI score(P = 0.007),tubular atrophy(P=0.03),and interstitial fibrosis(P = 0.001)in LN patients with MPO-ANCA positivity.After induction therapy,LN patients with MPO-ANCA positivity had lower complete remission(69.6%vs.84.6%,P = 0.1)and remission rates(81.2%vs.88.5%,P = 0.02)than PR3-ANCA positivity.During follow-up,end-stage renal disease(ESRD)developed in 15(15.2%)ANCA-positive LN patients,all of those were MPO-ANCA positive.The renal survival rates of LN with MPO-ANCA positivity were 94.1%at 1 year,83.3%at 5 years and 79.7%at 10 years;these values were lower than LN with PR3-ANCA positivity group,at 100%,100%and 100%(P = 0.05),respectively.By univariate analysis,MPO-ANCA positivity,higher CI score,lower eGFR at first kidney biopsy,and cyclophosphamide issued as induction therapy were associated with a higher risk of developing ESRD.Higher eGFR(HR,0.96;95%,0.94 to 0.98;P = 0.001)at first kidney biopsy and mycophenolate mofetil issued as induction therapy(HR,0.13;95%,0.03 to 0.59;P =0.008)were independent factors associated with better renal outcome in multivariate analysis.Conclusion:LN with ANCA positivity had massive hematuria,advanced renal insufficiency and crescents.LN with MPO-ANCA positivity had advanced renal insuffi'ciency and renal chronic diseases on pathological evaluation,lower rates of remission,and poor renal outcome.Higher eGFR at first kidney biopsy and mycophenolate mofetil issued as induction therapy were independent factors associated with better renal outcome in multivariate analysis.Part II:Compare effects of mycophenolate mofetil and cyclophosphamide for lupus nephritis with anti-neutrophil cytoplasmic antibody positivityObjective:Compare effects of mycophenolate mofetil and cyclophosphamide for lupus nephritis(LN)with anti-neutrophil cytoplasmic antibody(ANCA)positivity.Methodology:We searched the pathology database at our institution for cases that showed LN with ANCA positivity from January 1,1985 to December 31,2014.Among the 99 LN patients with ANCA positivity,37 required mycophenolate mofetil as induction therapy,34 received cyclophosphamide,and 28 assigned to the other regimens group.We compared effects and outcome of mycophenolate mofetil and cyclophosphamide for lupus nephritis with anti-neutrophil cytoplasmic antibody positivity.Results:There was no difference about baseline condition at between the two groups.During follow-up,mycophenolate mofetil group had higher complete remission rates(94.6%vs.55.9%,P = 0.001),higher remission rates(97.3%vs.76.5%,P = 0.002),and shorter duration of response(6.4 months vs.8.3 months,P =0.01)than cyclophosphamide group.Furthermore,mycophenolate mofetil group exhibited lower levels of proteinuria(0.3 g/24h vs.0.7 g/24h,P<0.001),hematuria(1×104/mL vs.8.5×104/mL,P = 0.009),and SCr(69.8 ?mol/L vs,91.9 ?moI/L,P?0.006),and also had lower scores of SLEDAI(2 scores vs.4 scores,P = 0.001)and higher levels of SAlb(43.5 g/L vs.38.4 g/L,P = 0.001)at the last follow-up.The incidence of adverse event was similar to each other group.The renal survival rates for mycophenolate mofetil group were 100%at 1 year,97.2%at 5 years and 97.2%at 10 years;these values were higher than cyclophosphamide group,at 91.0%,77.9%and 69.2%(P = 0.02),respectively.Conclusion:LN patients with ANCA positivity receiving mycophenolate mofetil as induction therapy had higher remission rate and better renal outcome than those requiring cyclophosphamide.