Author: Leo
Keywords: HSPN | Childhood case | Purpura nephritis | Crescent | Tripterygium
polyglycoside
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| From google images |
Hench-Schonleinpurpuranephritis (HSPN) is a rare chronic kidney disease. Since the prognosis of the kidney is due to the progress of the crescent of sclerosis, the treatment should be taken as soon as possible to prevent the pathological type from changing to the fibrosis process.
Second, the treatment of children with HSPN
1. Treatment of moderate to severe HSPN
It is usually considered that the patient is manifested as nephritis or nephrotic syndrome or persistent non-nephrotic proteinuria, normal renal function, <50% crescent formation or sclerosis injury, can be considered in patients with moderate to severe HSPN, clinical chronic or persistent state.
If there is no timely treatment, glomerular crescent disease can quickly evolve into complete glomerular sclerosis. Treatment options include corticosteroid immunosuppressive agents (such as cyclophosphamide, cyclosporine A, etc.) angiotensin converting enzyme inhibitors (ACEI) and tonsillectomy.
The use of tonsillectomy alone or in combination with glucocorticoids and cyclophosphamide has been shown to be effective in the treatment of HSPN. It is important to note that the severity of nephropathy in the study population is either different or non-randomized, or a small sample series , So the existing data as evidence of recommended specific treatment is inadequate and requires a higher level of evidence to demonstrate.
At present, due to mildew vinegar in children with less use, so there is no recommended use of the drug guide. In the treatment of adult IgA patients, it has not been known whether it is effective than a single renin-angiotensin (RAS) system blocker.
The author observed the effect of mold vinegar in the treatment of 12 children with HSPN, of which 5 cases of medication for 10 months, medication 12 months, the case of medication for 15 months, all children during follow-up showed proteinuria negative, renal function Return to normal, no serious adverse drug reactions. Recently, three cases of hormone-resistant HSPN (2 cases of acute nephritis and 1 case of nephrotic syndrome) were reported. The treatment of renal function and proteinuria was improved in children with mildew.
Tripterygium wilfordii polyglycoside is an active ingredient of traditional Chinese medicine Tripterygium wilfordii and has been widely used in the treatment of autoimmune and inflammatory diseases such as rheumatoid arthritis, psoriasis and so on. Tripterygium wilfordii polyglycoside in adult refractory nephrotic syndrome meta-analysis, suggesting that tripterygium polyglycoside compared with cyclophosphamide and placebo have a significant effect. WuL and so on with tripterygium glycosides treatment of children with moderate to severe HSPN, 95% (40/42) of children with renal damage indicators short-term significant remission, but its long-term effect remains to be seen.
2. Treatment of rapid or crescent HSPN
Recurrent or no renal dysfunction with severe proteinuria or nephrotic syndrome, and / or more than 50% of glomerular involvement in crescentic glomerulonephritis, can be defined as acute or crescent HSPN.
A randomized controlled trial (RCT) involving 56 children with HSPN, histologically confirmed that renal pathological changes in children belonging to the International Society for Children with Nephrology (ISKDC) grade II or higher, proteinuria and / or glomerular filtration (GFR) decreased for at least 1 month. Children were randomized to receive oral cyclophosphamide and supportive therapy for 6 weeks, or with supportive therapy alone, with an average follow-up of 6.9 years. There was no difference in ESRD odds between the two groups.
A recent prospective study of children with HSPN in the nephropathy range of proteinuria or ISKDC grade III-IV was compared with cyclosporine A (CsA) for 12 months with methylprednisolone (MP) The results showed that CsA appeared to be more effective in predicting disease remission than MP and prednisone in the two groups, with an average follow-up of 2.9 years.
Hormone alone or in combination with azathioprine or cyclophosphamide or both with its effect is obvious, or hormone and CsA, ACEI or a variety of other combinations of treatment are also effective. MP stroke therapy with oral administration of prednisolone or 36% of oral administration of prednisolone, or in more severe cases to add cyclophosphamide, can alleviate the degree of HSPN renal damage, reduce the incidence of ESRD patients.
Cyclophosphamide combined with oral prednisolone and dipyridamole in the protection of normal renal function, or remove or significantly reduce proteinuria, and reduce the activity index, prevention of disease histological progress have a significant effect. CsA alone or with glucocorticoids, eight CEI can be used together to relieve the disease, reduce the degree of renal histopathological changes.
In contrast to these favorable results, a recent retrospective analysis of 27 patients found that long-term (10 15 months) with prednisolone and cyclophosphamide followed by azathioprine treatment for long-term efficacy of the disease.
HSPN children can be given three or more drug treatments, including intravenous and / or oral corticosteroids, cyclophosphamide and antiplatelet and / or anticoagulant drugs. Renal tissue Mb-class changes in HSPN children, given prednisolone and urokinase intravenous shock treatment, followed by oral prednisolone, dipyridamole, warfarin at least 6 months after the urine protein was significantly reduced, after the urine protein 10 The disease activity index decreased, but the chronic index did not change. After an average follow-up of 10 years, only 1 case had a decrease in glomerular filtration rate.
XiaYue see 101 cases of renal pathology ISKDC grade Ⅲa / Ⅲb or more HSPN children with tripterygium glycosides efficacy, the results show that both the use of tripterygium polyglycoside alone, or combined steroid treatment, can not improve these patients The long-term prognosis of children. For pathology Ⅱ b / Ⅲ a class or non-nephrotic levels of proteinuria in children, the authors believe that Tripterygium wilfordii polyphenols on the recent improvement of proteinuria have a significant effect.
Since the prognosis of the kidney is due to the progress of the crescent of sclerosis, the treatment should be taken as soon as possible to prevent the pathological type from changing to the fibrosis process. Plasma exchange (PP) can remove circulating immune complexes in the blood to prevent crescent formation. PP can be used alone or in combination with other immunomodulators.
Shcnoy et al. Used PP as the sole treatment in 14 patients with acute progressive Mb-grade histological grading, followed by 4 years of follow-up, with 1 patient receiving kidney transplantation, 3 normal GFRs, normal urine test With mild proteinuria, all children without hypertension, suggesting that alone PP treatment of severe HSPN can play a positive role, at least with the immunosuppressive efficacy, and no immunosuppressant potential adverse reactions.
Gianviti and other PP and immunomodulator treatment of acute HSPN and crescent involving 60% of glomeruli in children, found that PP treatment in the onset of January will have a better effect, long-term ESRD and chronic kidney The probability of functional failure is reduced.
Third, children HSPN clinical recommended treatment program
Often, clinicians have to balance the risk of CKD in children with HSPN and the risk associated with treatment. Considering that the disease may naturally recover completely, or may be due to continuous ultrafiltration pressure on the renal unit damage, whether to choose treatment or when to choose treatment there is a big puzzle.
The current treatment of the literature recommended by the lack of high-level evidence-based medical evidence, so that clinicians for the choice of treatment program is very difficult, and even people on the current treatment is effective doubts.
In general, children in the field of nephrologists and clinical kidney disease experts are convinced of the need for treatment, but for different cases to take different interventions. In mild cases of renal symptoms, such as children only showed microscopic hematuria, short-term gross hematuria or mild proteinuria, the occurrence of low risk of CKD, clinicians may not take treatment.
But during the follow-up should be carefully observed in children's clinical and laboratory indicators of any changes occurred, and finally may take the type of renal biopsy pathological changes to decide whether to give patients treatment. In patients with nephritis syndrome or nephrotic range of proteinuria, or even clinically occurring nephrotic syndrome, 15% of cases are likely to develop CKD after a long period of illness, so active treatment is recommended.
As most cases alone oral prednisone is invalid, it is recommended as soon as possible intravenous use of methylprednisolone shock treatment. In addition to the use of immunosuppressive agents, the use of immunosuppressive agents in the process of the need to closely observe the adverse drug reactions. In addition, the use of immunosuppressive agents in the course of the use of immunosuppressive agents need to closely observe the adverse drug reactions.
PP as a powerful treatment can be used in patients with severe clinical manifestations, ISKDC pathological grade IV or V grade; especially when the patient is ineffective in the treatment of hormones and immunosuppressive agents, or in the absence of treatment of the initial patient that is expressed with a large number of Crescent formation of nephritis and nephrotic syndrome, PP should be considered early use. ACE inhibitors can be used for any degree of proteinuria and can be used alone for renal biopsy to suggest a high degree of chronic index of persistent proteinuria.
Fourth, the conclusion
Kidney involvement is the most important factor affecting the prognosis of HSP. The predictors of HSPN in children include severe abdominal symptoms, persistent purpura and older children. Prophylactic short-term use of prednisone can not reduce the risk of long-term renal injury in children with HSP. For the existing kidney disease, there is no recommended treatment.
Moderate to severe HSPN children can try glucocorticoid ± immunosuppressive or ACEI treatment, progressive increase in HSPN children can choose early PP or combined with MP intravenous therapy. Although the clinical severity of HSPN is associated with the prognosis of the disease, there is currently no single risk factor that can predict the risk of HSPN progression and a clear and effective treatment regimen. Long-term follow-up is essential.
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