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171例系统性硬化症(硬皮病)患者的生存预测因素。

Predictors of survival in 171 patients with systemic sclerosis (scleroderma).

作者信息

Nagy Z, Czirják L

机构信息

3rd Department of Internal Medicine, University Medical School of Debrecen, Hungary.

出版信息

Clin Rheumatol. 1997 Sep;16(5):454-60. doi: 10.1007/BF02238937.

DOI:10.1007/BF02238937
PMID:9348139
Abstract

Predictors of survival were determined in 171 patients with systemic sclerosis by univariate analysis, and the Cox proportional hazards model using both cross sectional data at entry into the follow-up and time-dependent follow-up data. Clinical and laboratory data were evaluated from 1982 to the end of 1993. The presence of diffuse scleroderma, kidney and cardiac involvements were unfavourable prognostic signs in both the univariate analysis, and the Cox proportional hazards models. The Cox model, using the variables detected at study entry, indicated that pericarditis, and anaemia were bad prognostic signs. Analysis with time dependent data has not been reported in systemic sclerosis. The appearance of pigmentation disturbances, anaemia, and respiratory failure during the follow-up also caused a poor prognosis of the disease by the Cox model. In the stepwise selection models, diffuse scleroderma, internal organ manifestations including renal, and cardiac involvements were predominantly selected as the most unfavourable factors for survival. As to the extent of skin involvement and internal organ manifestations, the general behaviour of the disease seems to be similar throughout the world. The early appearance of pericarditis and pigmentation disturbances at study entry are bad prognostic signs.

摘要

通过单因素分析以及使用进入随访时的横断面数据和随时间变化的随访数据的Cox比例风险模型,确定了171例系统性硬化症患者的生存预测因素。对1982年至1993年底的临床和实验室数据进行了评估。在单因素分析和Cox比例风险模型中,弥漫性硬皮病、肾脏和心脏受累均为不良预后征象。使用研究开始时检测到的变量的Cox模型表明,心包炎和贫血是不良预后征象。关于系统性硬化症,尚未有使用随时间变化数据进行分析的报道。随访期间出现色素沉着紊乱、贫血和呼吸衰竭,根据Cox模型,也提示疾病预后不良。在逐步选择模型中,弥漫性硬皮病、包括肾脏和心脏受累在内的内脏器官表现主要被选为最不利于生存的因素。至于皮肤受累程度和内脏器官表现,世界各地该疾病的总体表现似乎相似。研究开始时心包炎和色素沉着紊乱的早期出现是不良预后征象。

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