I. Preface Purpura nephritis is one of the common secondary glomerular diseases in pediatrics, and the reported rate of renal damage among patients with allergic purpura varies widely due to inconsistent diagnostic criteria and differences in observation and follow-up time, with 20% to 100% reported in the literatureIl cited. Brogan and Dillon [41 based on the diagnosis of clinical manifestations, the incidence of purpura nephritis is 40% to 50% of cattle. In November 2000, the Pediatric Nephrology Group of the Chinese Medical Association developed a draft of the diagnosis and treatment protocol for purpura nephritis in Zhuhai, China, and in 2008, the Nephrology Group of the Pediatric Branch of the Chinese Medical Association developed this guideline on the basis of the principles of curse E medicine; it applies to pediatricians who have some basis or experience in the diagnosis and treatment of renal diseases in children. The best clinical evidence and available medical resources for this disease should be fully understood in order to adopt the appropriate treatment plan for specific patients. Evidence sources 1. Literature search: The cited evidence is mainly from Chinese and English literature. Electronic search: Pubmed (1963-June 2008), EMBASE (1982-June 2008), China Biomedical Database (1994-June 2008), China Journal Network (1978-June 2008). Two researchers read the titles and abstracts separately, screened the literature according to the inclusion and exclusion criteria, read the full text after determining the inclusion, and graded the literature according to the evidence grading principle, and consulted the third evaluator when the opinions of the two evaluators differed. The final selection of 125 papers was made, including: 2 systematic reviews ‘6., j, 4 Meta-analyses ‘8’], and 7 RCTs. III. Level of evidence and recommendation level The level of evidence and recommendation level in this guideline refer to the European Society of Cardiovascular Diseases to fb the grading of evidence and recommendation recommendations (Table 1), the level of evidence is divided into 3 levels, and the recommendation is divided into 4 levels, in the guideline with [level of evidence/recommendation level] standard/program/guideline. Table 1 Level of evidence and grade of recommendation Level of evidence Study design status A B C Source of evidence j II Multiple randomized clinical trials (RCTs) or meta-analyses derived from single randomized clinical trials or large-sample nonrandomized clinical studies Evidence derived from expert consensus and/or small-sample studies, retrospective studies, and registries Grade of recommendation Grade I Evidence and/or consensus for diagnostic procedures or treatment is Determine the efficacy 1I a level of disagreement on the effectiveness of the treatment, but mainly evidence of effectiveness II b level of disagreement on the effectiveness of the treatment, but mainly evidence of poor efficacy III level of evidence that the treatment is ineffective or even harmful IV. Diagnosis of purpura nephritis 1. Diagnostic criteria: 97% of children with renal damage occur within 6 months of the onset of the disease ∞ 1. To further standardize the clinical diagnosis, the diagnostic criteria are now In order to further standardize the clinical diagnosis, the diagnostic criteria are modified as follows: within 6 months of the course of allergic purpura, hematuria and (or) proteinuria appear. The diagnostic criteria of hematuria and proteinuria are as follows: (1) hematuria: visual hematuria or microscopic hematuria; (2) proteinuria: if any of the following is satisfied: ① 3 times urine routine protein positive within 1 week; ② 24h urine protein quantification >150mg; ③ 3 times urine microalbumin above normal value within 1 week¨2’1 correction. Very few children with recurrence of purpura after 6 months of acute course of allergic purpura, along with the first occurrence of hematuria and/or proteinuria, should strive for renal biopsy, if it is a thylakoid hyperplastic glomerulonephritis dominated by IgA thylakoid aversion deposits, it should also be diagnosed as purpura nephritis. Clinical classification: ① isolated hematuria type; ② isolated proteinuria type; ③ hematuria and proteinuria type; ④ acute nephritis type; ⑤ nephrotic syndrome type; ⑥ acute nephritis type; ⑦ chronic nephritis type. 3, pathological grading: kidney biopsy pathological examination is the gold standard to determine the degree of kidney damage, the current commonly used pathological grading index for the 1974 ISKDC and the 2000 Chinese Medical Association Pediatrics Branch Nephrology Group developed. In recent years, clinical and pathological studies on purpura nephritis have found that renal tubular interrogative mass injury is closely related to the outcome and regression of purpura nephritis¨” cited. Glomerular pathological grading: Grade I: minor glomerular abnormalities. Grade II: simple thylakoid hyperplasia, classified as: disorganized focal tong segment; b. diffuse. Grade III: thylakoid hyperplasia with <50% glomerular crescent formation/segmental lesions (sclerosis, adhesions, thrombosis, necrosis) with thylakoid hyperplasia that can be: shan-local-tong segmental; b. diffuse. Grade IV: lesions with grade III, 50% a 75% of glomeruli with 1-. The above lesions are classified as: & focal segments;b- diffuse. grade V: lesions are the same as grade III, >75% of glomeruli with the above lesions are classified as “-ill focal/segmental;h diffuse. Grade VI: membrane-increased bullous glomerulonephritis. 4, kidney biopsy indications: for children without contraindications, especially those with proteinuria as the first or main manifestation (clinical manifestations of nephrotic syndrome, acute nephritis, acute progressive nephritis), kidney biopsy should be performed as early as possible, and the treatment plan should be selected according to the pathological classification. V. Treatment The clinical manifestations of children with purpura nephritis are not completely consistent with the degree of renal pathological damage, and the latter can more accurately reflect the extent of the lesion. When there is no condition to obtain pathological diagnosis, the corresponding treatment plan can be selected according to their l clinical grading. 1, isolated hematuria or pathology l grade: experts suggest: only the corresponding treatment for allergic purpura, microscopic hematuria has not been reported in the literature with definite efficacy. Children should be closely monitored for changes in their condition, and at least 3-5 years of follow-up nursing disc 1 [A/I] is recommended. 2, Isolated proteinuria, hematuria and proteinuria or pathology grade IIa: fewer foreign studies have been reported. Angiotensin-converting enzyme inhibitors (ACEI) and/or angiotensin receptor antagonists (ARB) class of drugs have a hypoproteinuric effect A/1] and are recommended for use in II ding. Domestic treatment is also carried out with rehmannia polysaccharide, rehmannia polysaccharide 1mr,/(kg・d), divided into 3 oral doses of up to 60mg per day for 3 months [C/IIa], but attention should be paid to its side effects of gastrointestinal reactions, liver function damage, bone marrow suppression and possible gonadal damage. 3. Non-nephrotic level proteinuria or pathology grade IIb and IIIa: there is little evidence from foreign studies, and the dosing can be referred to the previous level. Domestic reports have reported the use of rehmannia polysaccharide 1 mg (kg・d) in 3 oral doses, with a maximum daily dose of no more than 60 lIImg for 3-6 months B/IIa]. Hormone combined with immunosuppressive therapy has also been reported, such as hormone combined with cyclophosphamide therapy’ for 1 [C/II8], combined with cyclosporine A therapy [c/IIa]; the long-term efficacy of active treatment in this group of children remains to be studied. 4, nephrotic level proteinuria, nephrotic syndrome or pathology grade IIIb, IV: children in this group have more severe clinical symptoms and pathological damage, and now tend to use hormone combined with immunosuppressive therapy, of which the most certain efficacy is glucocorticoid combined with cyclophosphamide therapy”. If the clinical symptoms are warmer and the pathology is diffuse or with crescent formation, methylprednisolone shock therapy can be used, 15-30me/(kg・d) or 1000me/(1.73m2・d), with a maximum daily amount of no more than 1 g, daily or every other day, 3 times as a course of treatment [B/IIa]. In addition, some studies have shown that other immunosuppressive agents such as azathioprine m-33][C/IIa], cyclosporine A[C/IIa], and morte-macrolimus (MMF) o~1[c/IIa] also have significant efficacy. The guidelines suggest that glucocorticoids combined with cyclophosphamide shock therapy is preferred, and when cyclophosphamide therapy is ineffective or when the child cannot tolerate cyclophosphamide. Other immunosuppressive agents may be substituted. (1) Glucocorticoid combined with cyclophosphamide shock therapy: Prednisone 1.5-2mg,/(kg・d), orally for 4 weeks and then tapered, and cyclophosphamide 8-g,/(kg・d), intravenously, for 2 days, with 2 weeks interval as a course of treatment, total 6-8 courses. -The cumulative amount of cyclophosphamide should be <150mg/kg [A/I]. (2) Glucocorticoid combined with other immunosuppressive therapy ①Glucocorticoid + azathioprine 'sampan]: Prednisone 2 rag/(1(g・d) orally in divided doses, with azathioprine 2 rag/(kg・d), prednisone 2 mg/(1[g・d) every other day in a single dose, and gradually reduce the dose after 2 months; total course of azathioprine 8 months [c/1Ia]. . Glucocorticoids + cyclosporine A: cyclosporine A is given orally at 5 mg/(kg・d), blood concentration is monitored, and the trough concentration is maintained at 100-200 ng/ml for 8-12 months; at the same time, prednisone is given orally at 1-2 ms/(kg'd), and the dose is gradually reduced and stopped [C/1Ia]. lIa]. ③Glucocorticoid + mortezolomide (MMF)'3*-36': MMF 15-20mg,/(kg・d), maximum dose 1 g/d, divided into 2-3 oral doses, . After 4 months, the alligator is gradually reduced to 0.25-0.5me,/(kg・d) for 3-6 months; combined with prednisone 0.5-1mg/(kg・d) and gradually reduced [C/lIa]. In addition to the above immunosuppressants, there are clinical reports on hormone combined with Vincristine [c/lIa] or Leflunomide '381 [C/IIa] treatment in China, but the clinical typing and efficacy relationship is poorly evaluated, and there is a lack of pathological basis, which still needs further study. 5. Acute nephritis or pathology grade IV and V: these children have severe clinical symptoms and rapid progression of disease, and are now mostly treated with triple or quadruple therapy. Methylprednisolone combined with urokinase shock therapy + oral prednisone + cyclophosphamide + warfarin + dipyridamole therapy has also been reported in the literature (ix) 1 [B/IIa]. In addition to pharmacological treatment, plasma replacement therapy has been reported in recent years to effectively remove antibodies, complement and immune response mediators from patients' plasma, thus alleviating the progression of the disease in children¨¨[c/IIa], but it is a small sample non-randomized study, and the exact efficacy still needs further confirmation. 6. Adjuvant therapy: In addition to the above graded therapy, anticoagulants and/or anti-platelet aggregation agents, mostly dipyridamole 5 me'(kg・d) and heparin 1-2 mg/(kg・d), can be added [C/I]. ACEI and/or ARB drugs have a hypoproteinuric effect [A/I] and are recommended for children with proteinuria, regardless of whether they are combined with hypertension. ACEI commonly used preparations are benazepril, 5-10 called dU dose; ARB preparations are colesartan, 25-50 me/d oral old 1. VI. Prevention The preventive effect of glucocorticoids on renal damage in children with allergic purpura is still controversial. Prospective studies of hormone prophylaxis Kun showed that early hormone therapy did not prevent the development of renal damage H cited [B/IIb], and even retrospective studies found that children with allergic purpura who received hormone therapy were more likely to relapse III3 [C/Hb]. Meta-analyses on hormone prophylaxis showed the opposite results, with one Meta-analysis¨ showing that early hormone treatment in children with allergic purpura significantly reduced the occurrence of renal damage without adverse effects; the other two systematic reviews or Meta-analyses pu'"1 both suggested a tendency to reduce the occurrence of renal damage with early application of hormones, but The difference was not statistically significant A/IIa]. Therefore, the effectiveness of hormone prophylaxis remains to be studied clinically. Although purpura nephritis is self-limiting, some children still have prolonged disease course and even progress to chronic renal insufficiency. It is necessary for clinicians to pay attention to the treatment and further strengthen the follow-up. For children with abnormal urinalysis during the course of the disease, the follow-up period should be extended, and at least 3-5 years is recommended.