Systemic lupus erythematosus: Follow-up study of 148 patients. I: Classification, clinical and laboratory findings, course and outcome

Systemic lupus erythematosus: Follow-up study of 148 patients. I: Classification, clinical and laboratory findings, course and outcome
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系统性红斑狼疮:148 名患者的随访研究。

DOI:
10.1007/bf02200995
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发表时间:
1987
影响因子:
3.4
通讯作者:
A. Wiik
A. Wiik
中科院分区:
医学3区
文献类型:
--
作者:
P. Halberg;B. Alsbjørn;J. Balsløv;J. Gerstoft;I. Lorenzen;S. Ullman;A. Wiik

文献摘要

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本文描述了作者之一在18年中提出的148例循环抗核抗体和多系统疾病患者,并对其进行了随访。78%的患者符合1971年ARA系统性红斑狼疮分类标准,92%符合1982年ARA标准。85%为女性,SLE发病的平均年龄为32岁。80%的患者的早期表现为颧骨皮疹和关节炎,而大约一半的患者的肾病、中枢神经系统表现、浆液炎和外周血细胞减少症的发病时间延迟。在最年轻的患者中观察到肾病和血小板减少症。观察期的平均持续时间为8年。10年存活率为80%,半数死亡病例可能与SLE无关。死于系统性红斑狼疮和其他原因的患者的平均年龄分别为30岁和52岁。18%的死亡是尿毒症造成的,18%是感染造成的。总死亡率和SLE相关死亡率在整个观察期间均匀分布。发病率(新ARA标准的发生率和其他表明活动性疾病的结果)在观察的第一年下降,但在随后的几年中很少完全消退。观察超过10年的所有患者在其余观察期内均显示活动性疾病的证据,大多数患者显示肾脏疾病的证据。
The present paper is a description of 148 patients with circulating antinuclear antibodies and multisystemic disease filed during 18 years by one of the authors and followed up to date in 1981–83. Seventy-eight per cent of the patients satisfied the 1971 ARA criteria for the classification of systemic lupus erythematosus and 92 per cent fulfilled the 1982 ARA criteria. Eighty-five per cent were women, the mean age at onset of SLE was 32 years. Malar rash and arthritis were early manifestations in 80 per cent of the patients whereas the onset of nephropathy, CNS manifestations, serositis, and peripheral cytopenia was delayed in about half of the patients. Nephropathy and thrombocytopenia were observed particularly in the youngest patients. The mean duration of the observation period was 8 years. The 10-year-survival was 80 per cent. Half of the deaths were presumably unrelated to SLE. The mean ages at entry of patients who died of SLE and of unrelated causes were 30 and 52 years respectively. Eighteen per cent of the deaths were caused by uremia and 18 per cent by infections. The total and the SLE related mortalities were evenly distributed throughout the observation period. The morbidity (incidence of new ARA criteria and other findings indicating active disease) decreased during the first year of observation but rarely subsided completely during the following years. All patients observed for more than 10 years showed evidence of active disease during the rest of the observation period and most showed evidence of renal disease.