Theander Elke, Manthorpe Rolf, Jacobsson Lennart T H
Department of Rheumatology, Malmö University Hospital, Lund University, Lund, Sweden.
Arthritis Rheum. 2004 Apr;50(4):1262-9. doi: 10.1002/art.20176.
This study was undertaken to analyze standardized mortality ratios (SMRs) and causes and predictors of death in primary Sjögren's syndrome (SS) diagnosed according to 3 different classification criteria sets (the Copenhagen criteria, the European criteria, and the American-European consensus criteria (AECC).
A linked registry study using information from the Malmö Primary SS Registry combined with the Swedish Cause-of-Death Registry was performed, and SMRs were calculated. Kaplan-Meier survival curves and log rank tests were used to compare survival probability between subgroups of patients with primary SS. Cox regression analysis was used to study the predictive value of various laboratory findings at the time of diagnosis.
Four hundred eighty-four patients with a median followup of 7 years (range 1 month to 17 years 11 months) were included. The SMR for those fulfilling the AECC (n = 265) was 1.17 (95% confidence interval [95% CI] 0.81-1.63). Thirty-four deaths occurred in this group of patients. Excess mortality was found only for lymphoproliferative malignancy (cause-specific SMR 7.89 [95% CI 2.89-17.18]), corresponding to 2.53 excess deaths per 1,000 person-years at risk. In those not fulfilling the AECC (n = 219), 14 deaths occurred, the SMR was 0.71 (95% CI 0.39-1.20), and no excess mortality due to any specific cause was found. Hypocomplementemia, defined as C3 and/or C4 values in the lowest quartile of the SS patients' values at the time of diagnosis, was a significant predictor of death, mainly due to lymphoproliferative malignancy.
No increased all-cause mortality could be detected for patients with primary SS compared with the general population. When subgroups of primary SS were compared, excess mortality due to lymphoproliferative malignancy was found in patients fulfilling the AECC, the strongest predictor for unfavorable outcome being low C3 and/or C4 levels at the time of diagnosis.
本研究旨在分析根据3种不同分类标准集（哥本哈根标准、欧洲标准和欧美共识标准（AECC））诊断的原发性干燥综合征（SS）的标准化死亡比（SMR）以及死亡原因和预测因素。
进行一项关联登记研究，利用马尔默原发性SS登记处与瑞典死亡原因登记处的信息计算SMR。采用Kaplan-Meier生存曲线和对数秩检验比较原发性SS患者亚组之间的生存概率。使用Cox回归分析研究诊断时各种实验室检查结果的预测价值。
纳入484例患者，中位随访时间为7年（范围1个月至17年11个月）。符合AECC的患者（n = 265）的SMR为1.17（95%置信区间[95%CI]0.81 - 1.63）。该组患者中有34例死亡。仅在淋巴增殖性恶性肿瘤中发现超额死亡率（病因特异性SMR 7.89[95%CI 2.89 - 17.18]），相当于每1000人年有2.53例超额死亡。在不符合AECC的患者（n = 219）中，有14例死亡，SMR为0.71（95%CI 0.39 - 1.20），未发现任何特定病因导致的超额死亡率。低补体血症定义为诊断时C3和/或C4值处于SS患者值的最低四分位数，是死亡的重要预测因素，主要是由于淋巴增殖性恶性肿瘤。
与一般人群相比，未检测到原发性SS患者全因死亡率增加。当比较原发性SS亚组时，符合AECC的患者中发现淋巴增殖性恶性肿瘤导致的超额死亡率，诊断时低C3和/或C4水平是不良结局的最强预测因素。
