Progress in the evaluation and treatment of refractory focal segmental glomerulosclerosis (two)

(5) collapse type: the pathological feature is to collapse capillary loops, podocyte hypertrophy, cell type and its main difference is the l


(5) collapse type: the pathological feature is to collapse capillary loops, podocyte hypertrophy, cell type and its main difference is the lack of endothelial cells and mesangial cell proliferation, balloon adhesions and hyalinization is not common. The progress of pathological types can emerge quickly, balloon adhesions and glomerulosclerosis.

The pathological indicators of poor prognosis is mainly the proportion and degree of glomerulosclerosis, podocyte proliferation of interstitial fibrosis in proportion, so according to the prognosis of pathological types, from light to heavy, smooth arrangement is the top door type, special type, type, type of cell collapse. The apical response to hormone therapy was relatively good, the better. The cell type and the collapse type developed rapidly, and the clinical manifestations were worse. Door type and non special type between.

After the pathological types and prognosis of focal segmental glomerular sclerosis with knowing how to treat FSGS?

Six, FSGS treatment

The most common clinical manifestation of FSGS nephrotic syndrome, so treatment is the first choice of glucocorticoid, glucocorticoid application principle is now accepted is: must receive prednisone in the treatment of 0.5 ~ 2 mg• d-1• kg-1, duration of 6 months. If the clinical dosage is more than 60 mg per day, it is recommended to change to mg• (kg-1), d-1•, for a period of 3 months. However, it must be noted that the total glucocorticoid treatment course to be longer, not less than 6 months. Second, FSGS is mostly hormone dependent or steroid resistant, so it is necessary to use other immunosuppressive agents. It is now recognized that the role of the protein is effective, it is recommended daily dosage of 5 ~ 6 mg /kg. However, the characteristics of cyclosporine is often recurrent after reduction, especially after the withdrawal of 75% of patients with recurrence, the need for long-term small dose maintenance. The other is can be used with cyclophosphamide, azathioprine and chlorambucil. In addition, for patients with renal transplantation, FSGS recurrence can be applied to plasma exchange or protein adsorption. At present, ACEI and A R can reduce proteinuria and delay the development of renal failure, so it can also be used in the treatment of FSGS. These views are based on a large number of clinical evidence-based medicine, the following are introduced.

The first is a long course of treatment, prednisone 0.5 ~ 2 mg• d-1• kg-1, if less than 2 months of treatment, the complete remission rate is less than 30%, and if the course more than 5 months, complete remission rate of more than 30%.

If there is a hormone resistant FSGS, recommended intravenous methylprednisolone, combined with cytotoxic drugs, combined the remission rate of 60%. The combination of cytotoxic drugs, including CTX, can be administered orally or intravenously. The recommended dosage is 2 mg• d• kg-1, the effect is better, it is recommended once a month, each time from 0.6 to 1G. It can also be used to study the effect of nitrogen mustard, 0.1 ~ 0.2 mg• d-1• kg-1. But these cytotoxic drugs must be used simultaneously with hormones.

CTX is a drug commonly used in patients with steroid resistant FSGS. A clinical study showed that in 54 patients with hormone dependence or hormone resistance, the complete remission rate of hormone therapy alone was seven years, and the partial remission rate was up to 14.8% (). If combined with CTX, the complete remission rate was 26.7%, and the partial remission rate was up to 20%. CTX application can not only alleviate the rate increase, the more important is the seven years of follow-up, patients with 1/4 of simple hormone application for end-stage renal failure, and cyclophosphamide combined with only 10% of patients develop end-stage renal failure.

Cyclosporine is also an effective treatment for FSGS. The total course of treatment is more than one year, the initial medication 3 ~ 5 mg• d-1• kg-1, after six months can be reduced to 3.5 mg• d-1• kg-1. Current reports of cyclosporine in 50% to 70% of patients with steroid resistant FSGS effective. And most of the onset of a month, the onset of fast, but after the reduction of more recurrence, it is recommended that in the absence of contraindications, the treatment can be extended. Up to 2 to 3 years.

Another five years of follow-up, 51 cases of FSGS patients with renal syndrome 25 cases are treated by prednisone or combined with azathioprine or cyclosporine mg•, 3; d-1• kg-1. In addition, the control group was 26 patients who did not receive immunosuppressive therapy. The results showed that the remission rate of the immunosuppressive therapy group was significantly higher than that of the untreated group. Were 75%, 0.7%, a statistically significant. Second, is the simple application of hormone therapy remission rate is 62.5%, and the remission rate combined with azathioprine was 80%, combined with cyclosporin is 85.7%, therefore, the treatment of FSGS renal syndrome more recommended with cyclosporine or azathioprine combined with hormone therapy.


Another 49 cases of hormone resistant patients by clinical research of application showed that the application of prednisone and placebo group, four years after the 4% remission, and cyclosporine combined application of remission rate reached 70%, including 60% partial remission, 9% complete remission. More importantly, the effect of the combination of cyclosporine on renal function, renal function in patients with 1/4 decreased by half, while only half of the patients with only half of the kidney function decreased by half.

MMF FSGS is a second-line drug combination, the initial dosage is 1 ~ 2 g/d, the drug is well tolerated and low toxicity, so when used in other immunosuppressive drugs have side effects or not can be considered for MMF applications. Non controlled studies suggest that MMF can reduce proteinuria, but complete remission rate is low, but also the lack of long-term therapeutic efficacy evaluation.

Duncan reports that patients who are not able to respond to cyclosporine can be relieved with FK506, and renal function can be improved. 25 patients were reported to have an effective rate of up to 17, but in the other 68% patients, there were reversible renal impairment in 10 patients (40%). FK506 is more effective than cyclosporine, there is a lack of controlled randomized studies, and the sample size is also reduced, so only as a three line drug therapy.

Evidence based medicine combined with the use of immunosuppressive agents in the treatment of FSGS. A clinical study of 15 patients with steroid resistant and cyclosporine resistant FSGS nephropathy syndrome. Cyclosporine regimen is invalid, then using MMF, MMF every 4 months after the gradual reduction, once a month during the intravenous injection of CTX, if there is still no response, can be combined with CTX again three times, with methylprednisolone intravenous pulse therapy of methylprednisolone, then changed to oral corticosteroids.

ACEI and ARB have been recognized to delay the progress of renal function, so it is also used in the treatment of refractory primary FSGS. The general recommendation is to use without contraindications. Once there is a hormone resistance, in addition to the use of immunosuppressive agents, can be added with ACEI or ARB, help to reduce proteinuria and protect renal function.

LDL separation therapy is a new treatment plan. LDL is one of the most important independent risk factors for FSGS progression. Muso reported 10 cases of steroid resistant cases, in 9 weeks out of 7 cases of LDL were treated, the proteinuria reduction, remission rate increase. Hengshan et al. Reported that the 6 LDL treatment resulted in improvement of clinical symptoms and histology. However, it must be noted that the treatment of LDL isolation may be better and faster if combined with hormone immunosuppressive therapy.

For Japan's 36 hospitals recently reported the intractable FSGS kidney comprehensive five year follow-up patients, 29 cases were followed up for two years by LDL separation of remission rate of 62%, 15 cases were followed up for 5 years the remission rate reached 87%, indicating the early separation of the rapid decline in treatment of blood LDL is long-term therapeutic effect of the favorable factors.

Plasma exchange and immune adsorption are also the treatment of primary FSGS and refractory FSGS. However, there are few reported cases. In recent years, the treatment of refractory idiopathic nephrotic syndrome with FSGS has been combined with the use of hormone immunosuppressive agents. The remission rate can reach 50%. However, it is difficult to make a clear judgment on its curative effect because of its humoral factor is not clear.

Other treatments include diet control, stable blood pressure and lower blood lipids. Traditional Chinese medicine is also a good method, and can not be ignored in the treatment of some complications, such as infection, edema, etc.. A better way, and can not be ignored in the treatment of some complications, such as infection, edema, etc..


In this paper, the mechanism of glucocorticoid in renal disease and the principle of hormone therapy, the pathogenesis, pathological type and prognosis of glucocorticoid resistance were discussed. In conclusion, FSGS is a common pathological type of refractory nephrotic syndrome, and its incidence has increased significantly in the past 20 years. It is advocated that prolonged treatment and combination therapy can improve the clinical remission rate.

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