Takada K, Illei G G, Boumpas D T
Arthritis and Rheumatism Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA.
Lupus. 2001;10(3):154-61. doi: 10.1191/096120301671376017.
Aggressive immunosuppressive therapy with cyclophosphamide has improved the outcome of major organ disease in lupus patients. Controlled trials have shown that pulse cyclophosphamide is the treatment of choice for patients with moderate to severe proliferative nephritis. Long-term follow-up of patients participating in these controlled trials suggests that combining pulse cyclophoshamide with pulse methylprednisolone increases efficacy but not toxicity. Retrospective case series have also shown that pulse cyclophosphamide therapy may be effective for the management of severe or refractory to standard therapy neuropsychiatric, pulmonary, cardiovascular and hematologic disease. Pulse cyclophosphamide is associated with an increased risk for herpes zoster infections in the short term and with sustained amenorrhea in the long-term. Recent studies have also drawn attention to the lack of response (or incomplete response) and flare of lupus after an initial response. In an effort to circumvent these limitations, current investigations explore the therapeutic potential of high-dose, immunoablative cyclophosphamide therapy or low-dose cyclophosphamide in combination with nucleoside analogs or biologic response modifiers.
使用环磷酰胺进行积极的免疫抑制治疗改善了狼疮患者主要器官疾病的预后。对照试验表明，脉冲环磷酰胺是中度至重度增殖性肾炎患者的首选治疗方法。参与这些对照试验的患者的长期随访表明，将脉冲环磷酰胺与脉冲甲基泼尼松龙联合使用可提高疗效但不会增加毒性。回顾性病例系列也表明，脉冲环磷酰胺疗法可能对治疗严重或对标准疗法难治的神经精神、肺部、心血管和血液系统疾病有效。脉冲环磷酰胺在短期内与带状疱疹感染风险增加相关，在长期内与持续性闭经相关。最近的研究还提请人们注意在初始反应后狼疮缺乏反应（或不完全反应）和病情复发的情况。为了规避这些局限性，目前的研究探索了高剂量免疫清除性环磷酰胺疗法或低剂量环磷酰胺与核苷类似物或生物反应调节剂联合使用的治疗潜力。
