Clinical and genetic factors predictive of mortality in early systemic sclerosis.

Clinical and genetic factors predictive of mortality in early systemic sclerosis.
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DOI:
10.1002/art.24734
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发表时间:
2009-10-15
影响因子:
4.7
通讯作者:
Mayes, Maureen D.
Mayes, Maureen D.
中科院分区:
医学2区
文献类型:
--
作者:
Assassi, Shervin;del Junco, Deborah;Sutter, Kari;McNearney, Terry A.;Reveille, John D.;Karnavas, Andrew;Gourh, Pravitt;Estrada-Y-Martin, Rosa M.;Fischbach, Michael;Arnett, Frank C.;Mayes, Maureen D.

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研究在硬皮病预后研究(GENISOS)队列中,预测遗传与环境的生存的临床和遗传变量。GENISOS是一项多种族早期系统性硬化症(SSc)队列的前瞻性观察性研究。迄今为止,共有250名患者入组。除了临床和实验室数据外,还获得了每位患者的心电图(ekg)、胸片和肺功能检查。还收集了改良罗德曼皮肤厚度评分、HLA II类基因分型和Medsger损伤指数。我们采用有目的的模型构建策略,利用Cox回归进行了多变量分析。该研究分析了122名白人、47名非裔美国人和71名西班牙裔SSc患者,入组时平均病程为2.6年。在分析时,250例患者中有52例(20.8%)死亡。在排除HLA基因的最终多变量模型中,有7个变量成为死亡率的重要预测因素:入组时年龄≥65岁,预测的强制肺活量<50%,心电图显示有临床意义的心律失常,无抗着丝点抗体,高血压,提示肺纤维化的胸片和低体重指数(BMI)。在包含HLA基因的单独建模中,HLA等位基因DRB1*0802和DQA1*0501除上述预测因子外,是死亡率的显著预测因子。入院时收集的有限数量的变量,包括BMI,能够预测早期SSc患者的死亡率。此外,一些与SSc易感性相关的HLA基因可用于预测SSc的预后。
To investigate the clinical and genetic variables at initial presentation that predict survival in the Genetics versus Environment in Scleroderma Outcome Study (GENISOS) cohort. GENISOS is a prospective, observational study of a multiethnic early systemic sclerosis (SSc) cohort. To date, a total of 250 patients have been enrolled. In addition to clinical and laboratory data, electrocardiograms (EKGs), chest radiographs, and pulmonary function tests have been obtained from each patient. A modified Rodnan skin thickness score, HLA class II genotyping, and a Medsger Damage Index also have been collected. We performed multivariable analyses utilizing the Cox regression following a purposeful model building strategy. The study analyzed 122 white, 47 African American, and 71 Hispanic SSc patients with an average disease duration of 2.6 years at enrollment. At the time of analysis, 52 (20.8%) of the 250 patients had died. In the final multivariable model excluding HLA genes, 7 variables emerged as significant predictors of mortality: age ≥65 years at enrollment, forced vital capacity <50% predicted, clinically significant arrhythmia on EKG, absence of anticentromere antibodies, hypertension, chest radiograph suggestive of pulmonary fibrosis, and low body mass index (BMI). In separate modeling that included HLA genes, HLA alleles DRB1*0802 and DQA1*0501 were significant predictors of mortality in addition to the predictors mentioned above. A limited number of variables collected at presentation, including BMI, are able to predict mortality in patients with early SSc. In addition, some of the HLA genes associated with SSc susceptibility are useful for predicting SSc outcome.
DOI: 10.1002/art.22532
发表时间: 2007-03-15
期刊: ARTHRITIS & RHEUMATISM-ARTHRITIS CARE & RESEARCH
影响因子: --
作者:
McNearney, Terry A.;Reveille, John D.;Mayes, Maureen D.
通讯作者: Mayes, Maureen D.
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影响因子: 13.6
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