ABSTRACT
Objeetive To analyze the clinicopathologic features of proliferative sclerosing IgA nephropathy and the efficacy of prednisone therapy.Methods A retrospective analysis was conducted,enrolling 50 patients with biopsy-proven primary proliferative sclerosing IgA nephropathy who were admitted in the Hospital from January 2005 to June 2015-26 males and 24 females,mean age (36.8 ± 10.4) years.Clinicopathologic features and prednisone therapeutic effect were analyzed.Results The clinical manifestations of 50 cases were nephritis syndrome with varying degrees of renal insufficiency,including 32 cases (64.0％) with hypertension,15 cases (30.0％) with microscopic hematuria.Renal biopsy showed the incidence of glomerular global sclerosis was 17.0％-47.2％,tubular atrophy/ interstitial fibrosis outstanding (T0 50％,T1 32％,T2 18％).After prednisone treatment,compared with sustained remission group and relapse group,invalid patients had higher incidence of hypertension (P ＜ 0.05),relatively lower Hb (P ＜ 0.01) and serum albumin,more significant renal dysfunction (P ＜ 0.01),more severe glomerular global sclerosis,segmental sclerosis,tubular atrophy/ interstitial fibrosis,while the lower interstitial inflammatory cell infiltration.During the follow-up,which lasted from 6 to 132 months (median 27.3 months),the effective rate of treatment was 74.0％ after sufficient prednisone or half dose prednisone therapy.Repeated recurrence rate was 32.0％.At the end of the follow-up period,13(26.0％) patients entered the stage of uremia.Conclusions Application of glucocorticoids in the treatment of proliferative sclerosing IgA nephropathy can protect renal function and delay the progression of renal impairment.The efficacy of glucocorticoids therapy is significantly associated with the presence or absence of hypertension,the degree of renal function impairment,and the severity of the onset of renal pathology.
ABSTRACT
Objective:To investigate the expression of anti-endothelial cell antibodies(AECA) in patients serum with anti-neutrophil cytoplasmic antibody( ANCA) negative pauci-immune deposition type crescentic glomerulonephritis and its relationship with clinical signatures. Methods:We selected 94 pauci-immune deposition type crescentic glomerulonephritis patients from 2010 to 2015 treated in our hospital,45 of which with ANCA-negative( observation group) and 49 cases of ANCA-positive patients ( control group) , AECA levels of each groups serum were detected by Western blot test. Results: The average age of the observation group and Bermingham vasculitis activity score(BVAS) respectively (41. 08 +9. 43) years old and (15. 03 +3. 82),significantly lower than the control group (P<0. 05);the observation group had fever,joint pain accounted for 26. 67% and 13. 33%,significantly lower than the control group ( P<0. 05 );the observation group of nephrotic syndrome accounted for 48. 89%, higher than the control group ( P<0. 05);observation group positive rate of serum AECA was 46. 67%,significantly lower than the control group 81. 63% (P<0. 05);the observation group IgG-AECA identified 7 proteins,while the control group had identified 11 proteins,of which the observation group the positive rate of anti 90 kD antibody was 13. 33%( 6/45 ) , significantly lower than the control group 51. 02%( 25/49 ) ( P<0. 05 );observation group of anti 76 kD antibody positive patients had rash ratio of 100%, significantly higher than negative patients ( P<0. 05),anti 200 kD antibody positive the BVAS score of the patients was (18. 02 + 2. 51),which was significantly higher than that of the negative patients ( P < 0. 05 ) . Conclusion: The different level of AECA in ANCA negative pauci-immune crescentic glomerulonephritis patients may be associated with certain clinical manifestations;clinical manifestations differences between the ANCA negative and positive patients may be associated with different expression of the AECA related,the detail need a further study.