治疗幼年系统性红斑狼疮肾损害的方法：国际多中心 PRINTO 研究。

Therapeutic approaches for the treatment of renal disease in juvenile systemic lupus erythematosus: an international multicentre PRINTO study.

机构信息

Division of Pediatric Rheumatology, University of Calgary, Alberta Children's Hospital, Calgary, Canada.

出版信息

Ann Rheum Dis. 2013 Sep 1;72(9):1503-9. doi: 10.1136/annrheumdis-2012-201937. Epub 2012 Oct 25.

DOI:10.1136/annrheumdis-2012-201937

PMID:23100607

Abstract

OBJECTIVES

To evaluate therapeutic approaches and response to therapy in juvenile systemic lupus erythematosus (SLE) with renal involvement in a large prospective international cohort from four geographic areas.

METHODS

New onset and flared patients with active renal disease (proteinuria ≥0.5 g/24 h) were enrolled in 2001-2004. Therapeutic approaches and disease activity parameters were analysed at baseline, 6, 12 and 24 months. Response was assessed by the PRINTO/ACR criteria.

RESULTS

218/557 (79.8% female subjects, 117 new onset and 101 flared) patients with active renal disease were identified; 66 patients were lost to follow-up and 11 died. Mean age at disease onset for new onset group was higher than for flared group (13.1 vs 10.2 years, p<0.0001). At baseline, both groups had similar renal activity with similar median doses of corticosteroids (1.0-0.76 mg/kg/day). Cyclophosphamide (43.1%) and azathioprine (22%) were the most common immunosuppressive drugs. At baseline, South American patients received higher doses of corticosteroids than in other areas in new onset (median 1.16 vs 0.8-1 mg/kg/day) while cyclophosphamide use was similar in all four regions in the new onset group. There were no differences regarding the use of azathioprine or mycophenolate mofetil worldwide. PRINTO 70 response was reached in a greater percentage of new onset versus flared patients (74.8% vs 53.3%; p=0.005) at 6 months while at 24 months ACR 90 was reached by 69.9% and 56.1%, respectively.

CONCLUSIONS

New onset and flared juvenile SLE improved similarly over 24 months with minimal differences in therapeutic approaches worldwide.

摘要

目的

评估新发病例和复发的伴有肾脏受累的幼年系统性红斑狼疮（SLE）患者的治疗方法和对治疗的反应，该研究为来自四个地理区域的大型前瞻性国际队列研究。

方法

2001-2004 年，新发病例和复发的伴有活动期肾脏疾病（蛋白尿≥0.5 g/24 h）的患者被纳入研究。在基线、6、12 和 24 个月时分析治疗方法和疾病活动参数。采用 PRINTO/ACR 标准评估缓解情况。

结果

共纳入 218/557 例（79.8%为女性，117 例为新发病例，101 例为复发）伴有活动期肾脏疾病的患者；66 例患者失访，11 例死亡。新发病例组患者的疾病发病年龄高于复发组（13.1 岁比 10.2 岁，p<0.0001）。基线时，两组的肾脏活动度相似，皮质激素的中位数剂量相似（1.0-0.76 mg/kg/天）。环磷酰胺（43.1%）和硫唑嘌呤（22%）是最常用的免疫抑制剂。基线时，与其他地区相比，南美地区新发病例组患者接受的皮质激素剂量更高（中位数 1.16 比 0.8-1 mg/kg/天），而环磷酰胺的使用在新发病例组的四个地区相似。在全球范围内，硫唑嘌呤或吗替麦考酚酯的使用没有差异。6 个月时，新发病例组达到 PRINTO 70 缓解的患者比例高于复发组（74.8%比 53.3%；p=0.005），而 24 个月时达到 ACR 90 的患者比例分别为 69.9%和 56.1%。