Sinico R A, Fornasieri A, Fiorini G, Paracchini M L, Renoldi P, Maldifassi P, Bucci A, Ferrario F, D'Amico G
Int J Artif Organs. 1985 Jul;8 Suppl 2:15-8.
Plasma exchange is increasingly used for management of Essential Mixed Cryoglobulinemia. However little is known about the long term effects of this treatment. Therefore we have reviewed the clinical and laboratory data of 20 patients with type II EMC who were followed for a mean of 24 months. 16 patients had renal involvement, which was characterized histologically in all of them: 9 had diffuse proliferative GN plus endoluminal "thrombi" in 6 and vasculitis in 5, 4 had lobular membranoproliferative GN and 3 had focal proliferative GN. 14 patients had renal failure and 13 had proteinuria greater than or equal to 2 g/24 hr. PE (combined with immunosuppressive drugs in 18) was performed for a mean of 18 procedures. The combined treatment induced prompt remission of extrarenal and renal involvement. Serum creatinine and proteinuria decreased significantly in all but 2 patients during the treatment (s. creatinine from 2.9 to 1.6 mg/dl; proteinuria from 3.5 to 1.6 g/24 hr). Analysis of long term follow up revealed that these effects were long lasting in all the cases. We conclude that PE should be used for EMC nephropathy whenever prompt remission is not obtained by conventional therapy especially in consideration of its long term beneficial effects.
血浆置换越来越多地用于治疗原发性混合性冷球蛋白血症。然而，对于这种治疗的长期效果知之甚少。因此，我们回顾了20例II型原发性混合性冷球蛋白血症患者的临床和实验室数据，这些患者平均随访了24个月。16例患者有肾脏受累，所有患者的肾脏受累均有组织学特征：9例为弥漫性增殖性肾小球肾炎，其中6例伴有管腔内“血栓”，5例伴有血管炎；4例为小叶性膜增生性肾小球肾炎；3例为局灶性增殖性肾小球肾炎。14例患者出现肾衰竭，13例患者蛋白尿≥2g/24小时。平均进行了18次血浆置换（18例联合免疫抑制药物）。联合治疗使肾外和肾脏受累迅速缓解。除2例患者外，所有患者在治疗期间血清肌酐和蛋白尿均显著下降（血清肌酐从2.9降至1.6mg/dl；蛋白尿从3.5降至1.6g/24小时）。长期随访分析显示，所有病例的这些效果都是持久的。我们得出结论，对于原发性混合性冷球蛋白血症肾病，只要常规治疗不能迅速缓解，尤其是考虑到其长期有益效果，就应使用血浆置换。
