Francioni C, Fioravanti A, Gelli R, Megale F, Marcolongo R
Istituto di Reumatologia, Università, Siena.
Recenti Prog Med. 1993 Oct;84(10):679-86.
Treatment of several autoimmune diseases with intravenous gammaglobulins (IvIg) has been demonstrated to be safe and effective in determining clinical improvement and decreasing autoantibody titer. We tested the hypothesis that IvIg would be effective in patients with Systemic Lupus Erythematosus (SLE).
We conducted an open trial involving 12 patients with SLE refractory to conventional treatments with monthly infusion of IvIg at a dosage of 400 mg/kg/day for 5 consecutive days. The duration of the therapy with the same dose every 4 weeks was from 16 to 24 months.
A progressive clinical improvement was observed in 11 patients during IvIg therapy, and it persisted during the all period of treatment. The clinical improvement was associated with an increase of haemoglobin, albumin levels, total serum complement and C3 and C4 components, platelets count in 2 thrombocytopenic patients, and a progressive reduction of ESR, plasma immunocomplexes and antinuclear antibodies. A marked improvement in serum urea, creatinine clearance and proteinuria was also observed in the patients with renal involvement. We have not observed any adverse effects with the long-term use of this treatment. Several mechanisms have been postulated to explain the effect of IVIg in the treatment of autoimmune diseases. Many experimental and clinical data support the interesting hypothesis that IVIg may be effective in some autoimmune diseases by restoring a normal function or the physiological immune network through the same regulatory mechanisms leading to a suppression of autoimmune disorders in normal individuals. The presence of a wide spectrum of anti-idiotypic antibodies in IVIg could regulate T and B-cell activities preventing the emergence of B-cell clones with specificity for autoantigens epitopes and down-regulating autoantibodies production.
The results obtained suggest that IvIg therapy seems to be a promising and beneficial approach in the treatment of SLE. However, double-blind studies are necessary to confirm the results obtained particularly to ascertain the optimal dosage, the schedule of infusion and the duration of maintenance therapy with IVIg, and to determine their long-term effectiveness, the reduction in late morbidity and mortality and the effect on the quality of life of patients with SLE. Moreover, because of the high cost of IVIg, their use seems to be especially indicated for patients non responders to conventional treatments and for these with infectious complications.
静脉注射免疫球蛋白（IvIg）治疗多种自身免疫性疾病已被证明在改善临床症状和降低自身抗体滴度方面是安全有效的。我们检验了IvIg对系统性红斑狼疮（SLE）患者有效的假设。
我们进行了一项开放性试验，纳入12例对传统治疗无效的SLE患者，每月连续5天以400mg/kg/天的剂量输注IvIg。每4周以相同剂量进行治疗，疗程为16至24个月。
11例患者在IvIg治疗期间临床症状逐渐改善，且在整个治疗期间持续存在。临床改善与血红蛋白、白蛋白水平、总血清补体及C3和C4成分增加、2例血小板减少患者的血小板计数增加以及血沉、血浆免疫复合物和抗核抗体逐渐降低有关。在有肾脏受累的患者中，还观察到血清尿素、肌酐清除率和蛋白尿有明显改善。长期使用这种治疗方法未观察到任何不良反应。已经提出了几种机制来解释IVIg在自身免疫性疾病治疗中的作用。许多实验和临床数据支持了一个有趣的假设，即IVIg可能通过恢复正常功能或生理免疫网络，通过与正常个体中抑制自身免疫性疾病相同的调节机制，在某些自身免疫性疾病中发挥作用。IVIg中存在广泛的抗独特型抗体可调节T和B细胞活性，防止产生针对自身抗原表位具有特异性的B细胞克隆，并下调自身抗体的产生。
所获得的结果表明，IvIg治疗似乎是治疗SLE的一种有前景且有益的方法。然而，需要进行双盲研究来证实所获得的结果，特别是确定最佳剂量、输注方案和IVIg维持治疗的持续时间，并确定其长期有效性、晚期发病率和死亡率的降低以及对SLE患者生活质量的影响。此外，由于IVIg成本高昂，其使用似乎特别适用于对传统治疗无反应的患者以及有感染并发症的患者。
