Treatment included remission induction and maintenance therapy.
1. Aspires Remission induction is fast disease control, disease strive complete remission (including functional recovery of serological markers, symptoms and organ damage).
2. Maintenance therapy with low dose immunosuppressive agents to control the disease, prevention of disease recurrence and small drug side effects.
(1) Glucocorticoids: It's main drug treatment can significantly improve clinical and prognosis of patients, but the specific product should be based on whether there SLE disease activity and treatment type and follow the principle of individual classification.
① Prednisone: Adults are 0.8 ~ l mg / (kg / d), a total of 8 ~ 12 weeks after stable condition, enters phase reduction treatment to the maintenance dose (every other day 0.4mg / kg), full course of treatment of 1-2 years or more.
② The methylprednisolone pulse therapy: activities for SLE and LN pathological changes in severe cases, such type Ⅳ ln fusion crescent formation. Common scenarios: Each 1 g of methylprednisolone intravenously daily or every two days, a time, three times during a course, if necessary, within 3 to 7 days repeat, Total 1 ~ 3 courses. But pay attention to complications such as infections and sodium retention.
(2) Cytotoxic drugs: For the type of diffuse proliferative LN or poor patients hormone efficacy must be added cytotoxic drugs.
① Cyclophosphamide (cyclophosphamide, CTX): General methods oral CTX is 2 ~ 4 mg / (kg / d), but now considered NIH program is superior to conventional methods orally, used CTX 0.5 ~ 1.0g / m2 surface area body, enters 0.9% saline intravenous infusion of 250 ml, less than 1 hour, 1 time each month crash, a total of 6 times, then every three months shock 1 time still active after leaving impact 1 year, Total <12g. Treatment should pay attention to the fully hydrated alkaline urine, and to monitor changes in the blood, adverse reactions are reversible myelosuppression, infection, have nausea, vomiting, alopecia, gonadal suppression, hemorrhagic cystitis, carcinogenic, teratogenic ect. European programs: CTX 500 mg iv / 2 weeks, a total of 6 times, then give azathioprine 2 mg / kg, Total 2.5 years. NIH and European programs combined with hormone therapy.
② The azathioprine (azathioprine): Dose l ~ 2mg / (kg / d), dose glucocorticoid therapy usually combined, or as maintenance therapy.
③ mycophenolate mofetil (MMF): As a new immunosuppressant, can selectively inhibit the proliferation of T and B lymphocytes suitable for the treatment of refractory ln, produce slow efficacy, along with hormone over, starting dose is 1.5 ~ 2.0g / d, achieved clinical remission after reduced to 1.0g / d, after the past six months, down to 0.75g / d, maintenance dose is usually not less than 0.5g / d, total treatment 1.5 ~ 2 years. Gastrointestinal side effects were reactions, infections, bone marrow suppression.
④ glycosides Tripterygium: 60 mg / day, divided doses, combined with hormones have some effect on LN, for mild or hormone, upon removal of reducing immunosuppression is most suitable for maintenance treatment, major adverse reactions were myelosuppression, gonadal suppression, liver toxicity, menstrual disorders and gastrointestinal symptoms.
⑤ Cyclosporine (cyclosporine): Using starts 3 ~ 5mg / (kg / d), after taking 2-3 months Monthly least lmg / kg, reduced to 2.5mg / kg maintenance therapy doing. The main adverse reactions is liver toxicity, gastrointestinal symptoms, gingival hyperplasia and hirsutism.
⑥ Tacrolimus (tacrolimus) inhibit the activity of T cells and inflammatory cytokine responses, some scholars have suggested for the treatment of V-LN. Initial amount 0.08 ~ 0.1mg / (kg · d), two oral, with a low trough concentration is 5 ~ 15ng / ml. Should be based on the plasma concentration and dose adjustment Scr, after remission can be reduced 0.05mg / (kg / d), continued six months. Side effects include muscle tremors, hyperglycemia, A transient increase in creatinine, hepatic failure.
⑦ Leflunomide (1eflunomide): Leflunomide activity is an anti - immunomodulators proliferative different azoles, its mechanism is inhibition of dihydroorotate dehydrogenase activity and affects the pyrimidine synthesis of activated lymphocytes. Use once a day, every 2. Initially, three days dose (50mg / day) load, after the maintenance dose of 20 mg / day. Side effect is diarrhea, rash, bone marrow suppression, gonadal suppression, liver toxicity, small number of patients with pulmonary fibrosis.
(3) The ways of treatment are studied:
① The x-ray irradiation Systemic lymph, 20 Gy course of 4 to 6 weeks.
② Dealing in vitro immunoadsorption, general 3 ~ 7 times.
③ therapy with anti-CD4 monoclonal antibodies as 0.3 mg / kg intravenously.
④ immune reconstitution therapy: The use of large doses of CTX with hematopoietic stem cells, eliminating the disease in bone marrow-derived immune cells.
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