Hoffman G S
Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Foundation, OH 44195.
Curr Opin Rheumatol. 1993 Jan;5(1):11-7. doi: 10.1097/00002281-199305010-00003.
In recent years, interest in Wegener's granulomatosis has been stimulated by an increasing appreciation of the chronic relapsing nature of this disease and its association with antibodies to proteinase 3. Although conventional therapy with cyclophosphamide and glucocorticoids has produced remission in most patients, remission may not occur immediately and, in at least 50% of patients, may be followed by relapse. As a result, most patients experience some form of permanent morbidity from disease or treatment, or both. These observations have led to renewed efforts to identify more effective and less toxic therapies. Preliminary studies have evaluated other cytotoxic agents, such as methotrexate, and biologic products, such as high-dose immunoglobulin and monoclonal antibodies. It is hoped that a better understanding of the possible pathogenic role of anti-proteinase 3 antibodies may contribute to improved therapy. Unfortunately, research is handicapped by lack of an animal model, without which it will be difficult to prove convincingly that anti-proteinase 3 antibodies are important in expressing disease.
近年来，由于对韦格纳肉芽肿病的慢性复发性本质及其与抗蛋白酶3抗体的关联有了越来越多的认识，人们对该病的兴趣被激发起来。尽管环磷酰胺和糖皮质激素的传统疗法已使大多数患者病情缓解，但缓解可能不会立即出现，并且至少50%的患者可能会复发。因此，大多数患者会因疾病本身或治疗，或两者兼而有之，而经历某种形式的永久性病变。这些观察结果促使人们重新努力寻找更有效且毒性更小的治疗方法。初步研究评估了其他细胞毒性药物，如甲氨蝶呤，以及生物制品，如大剂量免疫球蛋白和单克隆抗体。希望对抗蛋白酶3抗体可能的致病作用有更深入的了解有助于改进治疗。不幸的是，由于缺乏动物模型，研究受到了阻碍，没有动物模型就很难令人信服地证明抗蛋白酶3抗体在疾病表现中很重要。
