Boumpas D T, Austin H A, Fessler B J, Balow J E, Klippel J H, Lockshin M D
National Institutes of Health, Bethesda, Maryland, USA.
Ann Intern Med. 1995 Jun 15;122(12):940-50. doi: 10.7326/0003-4819-122-12-199506150-00009.
To review advances and controversies in the diagnosis and management of systemic lupus erythematosus with visceral involvement (renal, neuropsychiatric, cardiopulmonary, and hematologic disease).
Review of the English-language medical literature with emphasis on articles published in the last 5 years. More than 400 articles were reviewed.
Recent debates pertaining to lupus nephritis have focused on the value of kidney biopsy data and the role of cytotoxic drug therapies. Many studies have shown that estimates of prognosis are enhanced by consideration of clinical, demographic, and histologic features. For patients with severe lupus nephritis, an extended course of pulse cyclophosphamide therapy is more effective than a 6-month course of pulse methylprednisolone therapy in preserving renal function. Adding a quarterly maintenance regimen to monthly pulse cyclophosphamide therapy reduces the rate of exacerbations. Plasmapheresis appears not to enhance the effectiveness of prednisone and daily oral cyclophosphamide. Small case series have shown pulses of cyclophosphamide to be beneficial in patients with lupus and neuropsychiatric disease refractory to glucocorticoid therapy, acute pulmonary disease (pneumonitis or hemorrhage), and thrombocytopenia. Patients with systemic lupus erythematosus have an increased prevalence of valvular and atherosclerotic heart disease, apparently because of factors related to the disease itself and to drug therapy.
Cytotoxic agents are superior to glucocorticoid therapy for the treatment of proliferative lupus nephritis, but the optimal duration and intensity of cytotoxic therapy remain undefined. Definitive studies of the treatment of autoimmune thrombocytopenia and acute pulmonary disease and of the diagnosis and treatment of neuropsychiatric disease are not available.
综述系统性红斑狼疮合并内脏受累（肾脏、神经精神、心肺和血液系统疾病）的诊断与管理方面的进展及争议。
回顾英文医学文献，重点关注过去5年发表的文章。共查阅了400多篇文章。
近期关于狼疮性肾炎的争论集中在肾活检数据的价值以及细胞毒性药物治疗的作用上。许多研究表明，综合考虑临床、人口统计学和组织学特征可提高预后评估的准确性。对于重症狼疮性肾炎患者，在保留肾功能方面，延长疗程的脉冲环磷酰胺治疗比6个月疗程的脉冲甲泼尼龙治疗更有效。在每月脉冲环磷酰胺治疗基础上增加季度维持方案可降低病情加重率。血浆置换似乎并不能提高泼尼松和每日口服环磷酰胺的疗效。小病例系列研究显示，环磷酰胺脉冲治疗对糖皮质激素治疗无效的狼疮合并神经精神疾病、急性肺部疾病（肺炎或出血）及血小板减少症患者有益。系统性红斑狼疮患者瓣膜性和动脉粥样硬化性心脏病的患病率增加，显然是由于与疾病本身及药物治疗相关的因素所致。
细胞毒性药物在治疗增殖性狼疮性肾炎方面优于糖皮质激素治疗，但细胞毒性治疗的最佳持续时间和强度仍不明确。目前尚无关于自身免疫性血小板减少症和急性肺部疾病治疗以及神经精神疾病诊断与治疗的确切研究。
