Schmidt C, Schooneman F, Siebert P, Weber M, Dureux J B, Streiff F, Schmitt J
Secteur d'Angéiologie, Médecine H, Hôpital Central, CHRU Nancy.
Ann Med Interne (Paris). 1988;139 Suppl 1:20-2.
Generalized scleroderma (GS) is associated with dysimmunity anomalies suggesting possible benefits of plasma exchange (PE) therapy. Nineteen patients with GS were treated by PE (volume of plasma exchange equivalent to 5-6% body weight and replacement by 4% human albumin), initially three times weekly, then weekly, bi-monthly and monthly (total duration 12-18 months). Clinical and paraclinical follow up was for an average of more than 2 years after the end of PE (mean number 17 per patient). Clinical results were assessed as positive and lasting in 11 cases (57.9%), two cases remaining stable and three cases worsening (one death from heart failure). The remaining three cases were failures in application of treatment (difficult venous approach). Improvement was noted in cutaneous sclerosis (62% of cases), trophic disorders (recovery in 6 of 7 cases) and articular manifestations. Vasomotor disorders were improved in only 20% of cases and visceral lesions unaltered. Results of capillaroscopy showed improvement in 5 of 11 cases. Biological values could not be correlated with either the course or the therapeutic efficacy. General tolerance to PE was good but the venous approach must be of good quality. These findings suggest the need for a randomized trial to define the place of PE in the treatment of GS.
全身性硬皮病（GS）与免疫功能异常有关，提示血浆置换（PE）治疗可能有益。19例GS患者接受了PE治疗（血浆置换量相当于体重的5 - 6%，用4%的人白蛋白替代），最初每周3次，然后每周1次、每2周1次和每月1次（总疗程12 - 18个月）。PE结束后平均临床和辅助检查随访超过2年（每位患者平均17次）。临床结果评估为11例（57.9%）为阳性且持久，2例保持稳定，3例恶化（1例死于心力衰竭）。其余3例为治疗应用失败（静脉穿刺困难）。皮肤硬化（62%的病例）、营养障碍（7例中的6例恢复）和关节表现有所改善。血管舒缩障碍仅20%的病例得到改善，内脏病变无变化。毛细血管镜检查结果显示11例中的5例有改善。生物学指标与病程或治疗效果均无相关性。对PE的总体耐受性良好，但静脉穿刺必须顺利。这些发现表明需要进行一项随机试验来确定PE在GS治疗中的地位。
