Refractory nephrotic syndrome contains the following two kinds: ① Primary nephrotic syndrome has treated with enough hormone for 8~12 weeks but invalid, this is also called steroid-resistant nephrotic syndrome;② Primary nephrotic syndrome has been relief with treatment of prednisone. But, when patients decrease medicine or stop it, the disease recurrently attacks (one year three recurrence or half one year more than 2 times). This is called capricious nephrotic syndrome or steroid-dependent nephrotic syndrome.
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The pathologys of steroid-resistant nephrotic syndrome are mostly mesangial proliferative glomerulonephritis, terminal membranous nephropathy, and terminal focal segmental glomerulosclerosis; the pathologys of capricious nephrotic syndrome or steroid-dependent nephrotic syndrome are minimal change nephropathy, mesangial proliferative glomerulonephritis and focal segmental glomerulosclerosis. The refractory nephrotic syndrome is a tough treatment.
When treating it, there are several tips:
① Hormone use. At the beginning of treatment, the prednisone dosage should be heavy enough; treatment is enough longer; decrement is enough slow, which is the key of promoting longer periods of remission and reduce the frequency. Common dosage : adult - 1 mg/(kg·d), treatment periods—8~12 weeks, later gradually decrease the dose until maintenance. The total course is more than 1 year.
② Cytotoxyc drugs use. The effect is good when steroid is used in combination with cytotoxyc drugs. Recently, common cytotoxyc drugs in clinic have cyclophosphamide and azathioprine.
③ Methylprednisolone Pulse treatment. Methylprednisolone Pulse treatment can bring into full play the effects of the immunosuppression and reduce adverse reaction. When treating, it’s important to note the adverse reactions of water-sodium retention, high blood pressure, infection after pulsing.
④ Cyclosporine A Application. Cyclosporine A can make some refractory nephrotic syndrome patients relief and promote remission time. However, this drug has certain adverse reaction, especially for renal interstitium damage, so it should have a close observation.
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