Miettunen Paivi M, Ortiz-Alvarez Oliva, Petty Ross E, Cimaz Rolando, Malleson Peter N, Cabral David A, Ensworth Stephanie, Tucker Lori B
Division of Rheumatology, Department of Pediatrics, University of Calgary, Calgary, AB, Canada.
J Rheumatol. 2004 Aug;31(8):1650-4.
To investigate the associations of gender and ethnic origin with longterm outcome in childhood-onset systemic lupus erythematosus (SLE).
The study cohort consisted of 51 patients (13 males and 38 females) with childhood-onset SLE followed for > or = 5 years at the British Columbia Children's Hospital in Vancouver. Fifteen patients were Caucasian, 14 Chinese, 9 East Indian, and 13 patients were of other ethnic backgrounds: none was African-American or Hispanic. Outcome measures assessed retrospectively included Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index score (SDI), SLE-related death, need for dialysis or renal transplantation, and use of intensive immunosuppressive therapy. A SDI > or = 2 was assigned as poor outcome.
The median age at diagnosis was 10.8 years and the median duration of followup was 7.2 years. Five-year survival was 100%; 10-year survival was 85.7% (12/14 patients). The median SDI score at last followup was 2.0 (range 0-9); 2.0 for male, 1.5 for female; 2.0 for Caucasian and 2.03 for non-Caucasian patients. Twenty-six out of 51 patients (51%) had poor outcome (SDI score > 2). Three female patients required dialysis: 2 had subsequent renal transplants. Thirty patients received intensive immunosuppressive therapy. The SDI scores, mortality, and need for intensive immunosuppressive therapy were not influenced by either gender or ethnic origin.
The median SDI score was high for this cohort with childhood-onset SLE. In contrast to other published data, no association of male gender and/or non-Caucasian ethnicity with poor outcome was found in our study cohort.
探讨性别和种族与儿童期起病的系统性红斑狼疮(SLE)长期预后的相关性。
研究队列包括51例儿童期起病的SLE患者(13例男性和38例女性),在温哥华的不列颠哥伦比亚儿童医院随访≥5年。15例患者为白种人,14例为中国人,9例为东印度人,13例患者为其他种族背景:无非裔美国人或西班牙裔。回顾性评估的结局指标包括系统性红斑狼疮国际协作临床中心/美国风湿病学会损伤指数评分(SDI)、SLE相关死亡、透析或肾移植需求以及强化免疫抑制治疗的使用。SDI≥2被定义为预后不良。
诊断时的中位年龄为10.8岁,中位随访时间为7.2年。5年生存率为100%;10年生存率为85.7%(14例患者中的12例)。末次随访时的中位SDI评分为2.0(范围0 - 9);男性为2.0,女性为1.5;白种人为2.0,非白种人为2.03。51例患者中有26例(51%)预后不良(SDI评分>2)。3例女性患者需要透析:其中2例随后接受了肾移植。30例患者接受了强化免疫抑制治疗。SDI评分、死亡率和强化免疫抑制治疗的需求不受性别或种族的影响。
该儿童期起病的SLE队列的中位SDI评分较高。与其他已发表的数据相反,在我们的研究队列中未发现男性性别和/或非白种人种族与不良预后相关。