| Objective:Henoch-Sch(?)nlein purpura(HSP),also known as Ig A vasculitis,is a systemic vascular inflammatory disease caused by a systemic small vessel metaplasia;when associated with kidney damage,it is called Henoch-Sch(?)nlein purpura nephritis(HSPN)and is the most common secondary glomerular disease in childhood.It is the most common secondary glomerular disease of childhood.Because of the diversity and inconsistency of clinical symptoms and pathology in children with HSPN,the relationship between clinical and pathological features deserves further investigation.The most commonly used pathological grading method is the ISKDC pathological grading method,which has limitations in identifying renal pathological changes and judging the disease because it ignores tubulointerstitial and capillary lesions.A new pathological assessment method,the modified semi-quantitative pathological score(SQC),has also been proposed recently,but its clinical application needs to be further explored due to the small sample size included in the current study.Therefore,this paper aims to investigate the correlation between the clinical and pathological characteristics of HSPN and the interrelationship between the various pathological classifications,in order to provide better guidance for the clinical management of HSPN in the future.Methods:The clinical and pathological data of 809 children with purpura nephritis who had perfected renal puncture biopsy in Jiangxi Children’s Hospital in the past 15 years(from January 2006 to December 2021)were collected,and the pathological specimens were evaluated by ISKDC pathological grading,renal tubular-interstitial pathological grading and SQC score respectively,and statistical methods were applied to analyze their clinical and pathological characteristics and each pathological grading in the relevant rows.Results:(1)The peak incidence of purpura nephritis was>6 and≤9 years old(38.30%);the mean age was 8.78±2.86 years;the male to female ratio was 1.45:1;94.80%of children had a renal puncture biopsy within 6 months of onset;21.00%of children had a history of more than one episode of carnivorous haematuria before the renal biopsy;71.40%of children had joint symptoms;55.60%had gastrointestinal symptoms,of which 14.80%The most common clinical classification was mixed hematuria and proteinuria(38.80%),followed by nephrotic syndrome(37.80%).(2)The median(interquartile)24h UP at the time of renal biopsy was 35.73(14.80,70.50)mg/kg-d.The proportions exhibiting negative urinary protein,microproteinuria,mild,moderate and severe proteinuria were 9.00%,11.20%,20.50%,20.90%and 38.40%respectively;the proportions of microscopic haematuria negative,+,2+,3+and 4+were 12.60%,20.90%,25.00%,14.30%and 27.20%respectively;most of the children had Alb levels≥30g/L,accounting for 90.5%;only 3.20%had hypoprotei-nemia.The pre-biopsy TC level was 4.53±1.29 mmol/L,TG level was 1.44±0.94 mmol/L,Scr level was 41.96±15.74 umol/L,BUN level was 4.65±1.84 mmol/L and UA level was 272.17±84.46 umol/L in a total of 133children with hyperuricaemia(16.40%).The mean blood Ig A level was 2.14±0.8(g/L),the mean blood C3 level was 1.19±1.11(g/L)and the Ig A/C3 value was 2±1.32.(3)ISKDC pathology was most commonly graded as grade Ⅲa(57.20%)and grade Ⅱ(31.90%);tubulointerstitial pathology was most commonly graded as grade++(38.80%)and grade+(33.00%);no grade++++was seen;median activity score in SQC was 1(1,2),chronic score was 3(1,6)and total score was 5(3,8).The immune complexes were mainly deposited in the thylakoid region(72.30%),25.00%were also deposited in the thylakoid region with capillary loops and 2.70%were deposited in capillary loops only.Ig A deposits were detected in all cases,with Ig A+++being the most common(47.00%),Ig M deposits in 73.70%of children,with Ig M+being the most common(61.20%)and Ig G deposits in 40.20%of children,with Ig G+being the most common(31.00%).Only 23 children(2.80%)had C4 deposits.(4)In this group,56.30%of the children were followed up with a median follow-up time of 12.40(4.00,25.30)months.282(62.00%)of the children with HSPN were in Grade A(complete remission),followed by 163(35.80%)in Grade B(mild urinary abnormalities),10(2.20%)in Grade C(active renal disease)and no Grade D(renal insufficiency).(5)ISKDC pathological grading was positively correlated with male(rs=0.084,P=0.017),with gastrointestinal bleeding(r_s=0.094,P=0.008),with sarcoid hematuria(r_s=0.151,P<0.001),BUN at renal biopsy(r_s=0.131,P=0.001),TC(r_s=0.128,P<0.001)The relationship was negative with Alb levels(r_s=-0.321,P<0.001)and positive with clinical staging of purpura nephritis(r_s=0.326,P<0.001)and albumin grouping(r_s=0.181,P<0.001).There was no significant correlation with age,blood pressure,definite precipitating factors,arthralgia,abdominal pain,UA,blood Ig A level,Ig A/C3,Scr,TG.ISKDC pathological grading was associated with 24h UP(r_s=0.367,P<0.001),m Alb(r_s=0.173,P<0.001),urine protein subgroup(r_s=0.363,P<0.001),microscopic haematuria subgroup(r_s=0.187,P<0.001);positive correlation with the site of immune complex deposition(r_s=0.163,P<0.001);no correlation between different immune deposition types and ISKDC pathological grading(P>0.05);positive correlation between Ig M deposition intensity and ISKDC pathological grading(r_s=0.110,P=0.002),different Ig A deposition intensity,Ig G deposition intensity,C3 deposition intensity and C4 deposition intensity did not correlate significantly with ISKDC pathological grading,P>0.05.(6)Tubulo-interstitial pathological grading was positively correlated with carnal hematuria(r_s=0.261,P<0.001),BUN at renal biopsy(r_s=0.073,P=0.038),TC(r_s=0.118,P=0.001)and negatively correlated with Alb levels(r_s=-0.204,P<0.001);and with Age,blood pressure,definite precipitating factors,joint symptoms,GI symptoms,GI bleeding,UA,Ig A levels,Ig A/C3,Scr,TG no significant correlation;positive correlation with clinical typing of purpura nephritis(r_s=0.241,P<0.001),albumin grouping(r_s=0.158,P<0.001).24h UP,m Alb urine protein stratification,and microscopic haematuria grouping were positively correlated with tubulo-interstitial pathological grading,P<0.001,which was statistically significant.There was a significant positive correlation between the site of immune complex deposition and tubulo-interstitial pathological grading,r_s=0.126,P<0.001;there was no correlation between different types of immune deposition and intensity of deposition and tubulo-interstitial pathological grading,P>0.05,which was not statistically significant.(7)The three indicators of modified semi-quantitative product SQC were positively correlated with clinical typing of purpura nephritis and blood albumin grouping;negatively correlated with Alb,P<0.001.Gastrointestinal symptoms were weakly positively correlated with modified semi-quantitative product SQC activity score,r_s=0.075,P=0.034,but not with chronic score and total score,P>0.05;with gastrointestinal bleeding was positively correlated with SQC activity score(r_s=0.119,P=0.001)and total score(r_s=0.076,P=0.031),but not with chronic score;Scr at renal biopsy(r_s=0.108,P=0.002)was positively correlated with SQC activity score,but not with chronic score and total score;TG was positively correlated with chronic score(r_s=0.086,P=0.014)and total score(r_s=0.082,P=0.019)were weakly positively correlated with the activity score,but not with the activity score;the three indicators of SQC with sarcoid hematuria,BUN and TC levels were positively correlated with the modified semi-quantitative score,P<0.05,suggesting statistical significance.There was no correlation between age,gender,blood pressure,joint symptoms,blood uric acid,Ig A level,Ig A/C3 and SQC.24h UP,urine protein grouping,microscopic haematuria grouping and SQC activity score,chronic score and total score were all positively correlated,P<0.001,suggesting statistical significance.The m Alb was positively correlated with the modified semi-quantitative score SQC chronic score(r_s=0.224,P<0.001)and total score(r_s=0.228,P<0.001),but not with the activity score,P=0.084.There was a positive correlation between the site of immune complex deposition and all indicators of the modified semi-quantitative score,P<0.001;Spearman’s rank correlation was performed between the different types of immune deposition and the three indicators of SQC;cases with the type of deposition of Ig A+Ig M+C3 were correlated with the SQC activity score(r_s=0.078,P=0.026)and the total score(r_s=0.080,P=0.023)showed a significant weak positive correlation and no correlation with the chronic score;the remaining different immune deposition types did not correlate with the SQC scores,P>0.05.The intensity of Ig A deposition was weakly and negatively correlated with SQC activity score(r_s=-0.085,P=0.016),but not with chronic score and total score;the intensity of Ig G deposition was weakly and negatively correlated with SQC chronic score(r_s=-0.085,P=0.016),but not with activity score and total score;the intensity of Ig M deposition was strongly correlated with SQC total score(r_s=0.079,P=0.024)showed a weak positive correlation and no correlation with activity and chronic score;C3 and C4 deposition intensity did not correlate with the SQC scores,P>0.05.(8)ISKDC pathological grading was significantly and strongly positively correlated with tubulointerstitial pathological grading(r_s=0.406,P<0.001)and SQC activity score(r_s=0.446,P<0.001),chronic score(r_s=0.602,P<0.001),and total score(r_s=0.679,P<0.001);tubulointerstitial grading was significantly and positively correlated with SQC activity score(r_s=0.277,P<0.001),chronic score(r_s=0.699,P<0.001),and total score(r_s=0.681,P<0.001)also showed a significant strong positive correlation.(9)Clinical staging of purpura nephritis(r_s=0.112,P=0.017)was positively correlated with prognosis;24h UP(r_s=0.099,P=0.034)and urine protein subgroup(r_s=0.100,P=0.034)were weakly positively correlated with prognosis;Alb(r_s=-0.128,P=0.006)was negatively correlated with negative correlation with prognosis;age,sex,hypertension,abdominal pain,with gastrointestinal bleeding,with carnitic haematuria,Scr at renal biopsy,BUN,TC,Ig A/C3 and albumin subgroups were not significantly correlated with prognosis.Tubulo-interstitial pathological grading was positively correlated with prognosis,r_s=0.139,P=0.003.Total SQC score was positively correlated with prognosis,r_s=0.094,P=0.045;SQC activity score and chronic score were not correlated with prognosis.ISKDC pathological grading was not significantly correlated with prognosis.Conclusions:(1)The clinical staging of HSPN is dominated by mixed haematuria and proteinuria and nephrotic syndrome,with IKSDC pathological grades Ⅲa and Ⅱ being the most common and tubulointerstitial pathological grades+++and+being the most common.(2)The lower the serum Alb level of HSPN,the more severe the clinical staging and proteinuria,the more severe the microscopic haematuria,the immune complexes with capillary loop deposits and with Ig M deposits,the more severe the renal pathology.(3)ISKDC pathological grading is consistent with tubulo-interstitial pathological grading and the modified semi-quantitative score SQC in the evaluation of glomerular injury and tubulo-interstitial injury,and the SQC score is superior to IKSDC pathological grading,and the SQC score can be preferentially used for the assessment of pathological injury in HSPN.(4)The majority of children with HSPN have a good prognosis,with a relatively worse prognosis the lower the serum Alb,the higher the clinical staging,the degree of proteinuria and the total SQC score. |
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