Tavoni A, Mosca M, Ferri C, Moriconi L, La Civita L, Lombardini F, Bombardieri S
Clinical Immunology Unit, University of Pisa, Italy.
Clin Exp Rheumatol. 1995 Nov-Dec;13 Suppl 13:S191-5.
The principal therapeutic procedures and when they are clinically indicated in the management of essential mixed cryoglobulinemia (EMC) have been the subject of much debate. This paper reviews current knowledge and our experience in the treatment of this complex disease. It is generally agreed that patients with purpura, the primary symptom of EMC, should avoid long periods of sitting or standing in the same position. Non-steroidal antiinflammatory drugs can be used for the management of arthralgias and/ or arthritis. Low dose steroids (0.5-0.3 mg/kg/die) are usually sufficient to control the purpura, arthralgias, arthritis and weakness, while larger doses (0.5-1.5 mg/kg/die) are needed to treat the renal involvement, peripheral neuropathy and serositis. Since the discovery of the association between EMC and viral infections, the appropriateness of cytotoxic drugs has been re-evaluated and they are no longer used. With the low antigen content diet, a regimen designed to restore a saturated mononuclear phagocytic system, good results have been obtained in the treatment of purpura, arthralgias, weakness and peripheral neuropathy. Furthermore, this dietary regimen may play a steroid sparing role. Plasma exchange is widely used in the management of severe renal involvement, hyperviscosity syndrome, sensory motor neuropathy and liver involvement in EMC.
原发性混合性冷球蛋白血症（EMC）的主要治疗方法以及它们在临床治疗中的适用时机一直是诸多争论的焦点。本文回顾了关于这种复杂疾病治疗的现有知识和我们的经验。人们普遍认为，患有紫癜（EMC的主要症状）的患者应避免长时间保持同一姿势坐着或站立。非甾体类抗炎药可用于治疗关节痛和/或关节炎。低剂量类固醇（0.5 - 0.3毫克/千克/天）通常足以控制紫癜、关节痛、关节炎和虚弱症状，而治疗肾脏受累、周围神经病变和浆膜炎则需要更大剂量（0.5 - 1.5毫克/千克/天）。自从发现EMC与病毒感染之间的关联后，细胞毒性药物的适用性已被重新评估，不再使用。通过低抗原含量饮食（一种旨在恢复饱和单核吞噬系统的方案），在治疗紫癜、关节痛、虚弱和周围神经病变方面取得了良好效果。此外，这种饮食方案可能具有减少类固醇用量的作用。血浆置换广泛应用于治疗EMC严重的肾脏受累、高粘滞综合征、感觉运动神经病变和肝脏受累。
