In March 2009, in the famous Chinese kidney disease experts, the Chinese medical doctor association nephrology branch President, Peking union medical college hospital professor heering p under the launch of China's primary nephrotic syndrome (INS) treatment expert consensus group was formed. A total of 14 nephrology specialists from three major hospitals across the country were involved in the writing of the consensus. Renal medicine ming-hui zhao, the first hospital, Beijing university professor, Shanghai huashan hospital kidney medical professor gu, professor heering p respectively as focal segmental glomerulosclerosis, membranous nephropathy and minimal change disease (nephrotic syndrome three team leader, after 1 year time, five big discussion and review, a large number of domestic and foreign latest literatures, brings together a wide range of expert opinion, finally completed the "expert consensus on treatment of primary nephrotic syndrome in China". This is the first system to introduce Chinese expert consensus clinical treatment of primary nephrotic syndrome, for our country's renal physician clinical treatment of primary nephrotic syndrome provides a very practical guidance.
This consensus will be published by the people's health press in April. The summary of the consensus is published first, to be used as a reader.
Summary of nephrotic syndrome
Nephrotic syndrome is a clinical syndrome caused by various glomerular diseases. Definition: large amount of proteinuria 33.5 g/d; Low albuminemia, plasma albumin < 30 g/L; Edema and hyperlipidemia, which are often associated with varying degrees of degree, are required to diagnose a large amount of albuminuria and hypoalbuminemia.
Adult primary nephrotic syndrome in the main pathologic type of membranous nephropathy (MN), focal segmental glomerulosclerosis (FSGS) and nephrotic syndrome is minimal change disease (MCD), the cause is still unknown. Primary nephrotic syndrome is diagnosed after careful removal of secondary factors, including genetic factors.
Expert consensus on the treatment of primary nephrotic syndrome
Minor disease transsexual nephrotic syndrome
MCD is typical clinical manifestations of nephrotic syndrome, only about 15% with microscopic haematuria, generally no persistent high blood pressure and renal function impairment, develop into end-stage renal disease (ESRD). Because adrenocorticoid therapy can significantly increase the proportion of patients with total relief early on, the treatment is preferred. Prednisone mg/kg d (no more than 80 mg/d), and about 76 per cent of patients were fully relieved for 8 weeks, with a maximum of 16 weeks. During treatment to pay attention to the adjustment of adrenal cortical hormone dose, after every 2 ~ 4 weeks to reduce 10% of the original usage, below 15 mg/d the reduction should be more slowly, in order to reduce recurrence.
For patients with adrenocorticoid dependence or recurrent relapse, they can be treated for 12 to 16 weeks with prednisone 1 mg/kg/kg/kg/kg/kg. If the treatment is not able to maintain long-term relief of the patient, or with the use of the taboo and glucocorticoid resistance in glucocorticoids, the treatment of cyclosporin is not possible. Starting dose 3.5 ~ 4 mg/kg · d, titration valley concentration in 100 ~ 200 ng/ml, 4 ~ 6 months, if there is a partial response or complete response, began to slow, reducing every 1 ~ 2 month minus 0.5 mg/kg · d, course of treatment at least 1 year, can small doses (1 ~ 1.5 mg/kg · d) maintain for a long time. The combination of small doses of glucocorticoid (0.5 mg/kg/kg) may increase the rate of reduction. If it is not valid, the cyclosporin should be stopped, and after repeated renal biopsies, it will be taken into consideration in order of the FK506, which is the MMF and the purine.
To reduce the number of hormone dependent on patients with adrenal cortical hormone dependent patients, as well as to the ring spore element resistance can try FK506, starting dose of 0.05 ~ 0.1 mg/kg · d, titration valley concentration 5 ~ 10 ng/ml, 4 ~ 6 months, if there is a partial response or complete response, began to slowly reduce the dose. Its clinical experience still needs to be accumulated.
Patients who are not treated with this treatment can try MMF or thiazole.
Focal renal glomerulus of the focal area
FSGS in glomerular sclerosis of focal segmental distribution of a group as the basic pathological changes of glomerular disease, cause genetic three categories, including primary, secondary and pathology classification for: collapse, cutting-edge, cell type, door type and are not different.
In patients with FSGS, 100% have different level of proteinuria, more than 60% for nephrotic syndrome, about 50% had different degree of blood in the urine, a third have disease with high blood pressure, renal insufficiency, often have impaired renal tubules. For persistent nephrotic syndrome, more than 50% of patients will enter ESRD within 5-10 years without treatment.
Due to separate use of adrenal cortical hormone therapy in patients with complete remission rate is low, long-term high-dose prednisone (1 mg/kg · d) using often accompanied by glucocorticoid obvious side effects, high doses of corticosteroids is preferred treatment for prednisone (1 mg/kg · d) combined cyclophosphamide or small doses of corticosteroids (prednisone < 0.5 mg/kg · d) joint ring spore, A treatment.
For patients who are not treated with cyclosporin A, he (0.05 to 0.1 mg/kg/kg) may be effective.
For glucocorticoid dependence, repeated recurrence, cyclophosphamide, ring a spore element, azathioprine, for he phenol ester (1 ~ 2 g/d, and 3 ~ 6 months) could help to extend the time maintain remission.
Patients who are not treated with the above treatment may have a trial of lituximab or plasma replacement.
Most of the patients with membranous nephropathy are nephrotic syndrome, and the rest are non-nephrotic proteinuria. 50% of the patients may have microscopically hematuria, rare with red blood cells or macroscopically. Most patients have normal blood pressure. Most patients have normal kidney function, and the kidney function is usually slow.
The natural course of membranous nephropathy is very different, some patients are naturally relieved, some patients progress to ESRD. Normal, proteinuria is less than 4 g/d for low risk patients; The blood creatinine is normal or nearly normal, and the urine protein is 4 ~ 8 g/d for the middle and dying patients; The abnormal or continuous deterioration of creatinine, or the urinary protein > 8 g/d, is a high-risk patient. For middle and low risk patients, only non-immunosuppressive therapy may be considered. For high-risk patients, active immunosuppression is required. The use of glucocorticoid therapy alone does not improve the full rate of remission and improves the long-term survival of the kidney. The joint use of the preferred glucocorticoid and alkane in immunosuppression therapy; For sufficient corticosteroids have concerns or have glucocorticoid contraindicated, available or ring ring spore element spore element with small dose of glucocorticoid treatment, the curative effect is; Low doses (1 ~ 1.5mg/kg d) will prevent recurrence after a slow reduction in cyclosporin. The FK506 also has some effect in alleviating albuminuria and protecting kidney function, and the treatment plan remains to be determined after the accumulation of experience. It may be useful in the short term to reduce urine protein, for lack of more validity evidence, only when the treatment is ineffective. Azathioprine cannot improve the overall response rate of patients with IMN proteinuria, also cannot improve kidney survival rate for a long time, is not recommended as a conventional immunosuppressive treatment of membranous nephropathy.