Airio A, Kautiainen H, Hakala M
Rheumatism Foundation Hospital, Heinola, Finland.
Clin Rheumatol. 2006 Mar;25(2):234-9. doi: 10.1007/s10067-005-1164-z. Epub 2006 Feb 14.
The objective of this study was to assess the long-term outcome of polymyositis (PM) and dermatomyositis (DM) and the factors predictive of this outcome in a nationwide series in Finland. One hundred and seventy-six patients with PM and 72 patients with DM diagnosed in Finland in 1969-1985 were selected from the national hospital discharge register according to the diagnostic criteria of Bohan and Peter and followed up until death or till the end of August 1995. Gender, age, delay of therapy, serum creatine kinase, erythrocyte sedimentation rate, initial dose of corticosteroids and duration of cytostatic therapy were assessed as factors prognostic of death. The 5-year survival rate for PM was 75% [95% confidence interval (CI): 68-81%] and that for DM 63% (50-73%), and the respective 10-year survival rates were 55% (47-62%) and 53% (41-64%). The median survival for polymyositis was 11.0 years (95% CI: 9.5-13.3) and that for DM 12.3 years (5.5-20.7). The standardized mortality ratio for the combined group of PM and DM showed approximately threefold mortality compared to the general population. Compared to PM patients, the subjects with DM had a 1.47-fold (95% CI: 0.99-2.12) age- and sex-adjusted risk of mortality. Except for age in both groups and the delay in diagnosis in the PM group, no other individual factor reached significance as a predictor of death. However, cancer had a hazard ratio (HR) of 2.16 for death (95% CI: 0.95-4.50) in the DM group and 1.99 (95% CI: 1.01-3.94) in the PM group. A comparison of the causes of death in the PM and DM groups showed that the patients with DM had a greater risk of dying from cancer (age-adjusted HR 5.11, 95% CI: 2.31-11.3). According to this nationwide analysis of survival and its prognostic factors in patients with PM and DM, the latter group had an increased age-adjusted risk for mortality compared to the former. The difference seems to be mostly explained by the fact that the patients with DM had a higher risk of dying from cancer.
本研究的目的是评估芬兰全国范围内多发性肌炎（PM）和皮肌炎（DM）的长期预后以及预测该预后的因素。根据博汉和彼得的诊断标准，从国家医院出院登记册中选取了1969年至1985年在芬兰诊断出的176例PM患者和72例DM患者，并随访至死亡或1995年8月底。将性别、年龄、治疗延迟、血清肌酸激酶、红细胞沉降率、皮质类固醇初始剂量和细胞毒性治疗持续时间作为死亡预后因素进行评估。PM的5年生存率为75%[95%置信区间（CI）：68 - 81%]，DM为63%（50 - 73%），相应的10年生存率分别为55%（47 - 62%）和53%（41 - 64%）。多发性肌炎的中位生存期为11.0年（95%CI：9.5 - 13.3），DM为12.3年（5.5 - 20.7）。PM和DM联合组的标准化死亡比显示，与普通人群相比，死亡率约为其三倍。与PM患者相比，DM患者经年龄和性别调整后的死亡风险为1.47倍（95%CI：0.99 - 2.12）。除了两组的年龄以及PM组的诊断延迟外，没有其他个体因素作为死亡预测指标具有统计学意义。然而，癌症在DM组中的死亡风险比（HR）为2.16（95%CI：0.95 - 4.50），在PM组中为1.99（95%CI：1.01 - 3.94）。PM组和DM组死亡原因的比较表明，DM患者死于癌症的风险更高（年龄调整后HR 5.11，95%CI：2.31 - 11.3）。根据对PM和DM患者生存情况及其预后因素的全国性分析，与前者相比，后者经年龄调整后的死亡风险增加。这种差异似乎主要是因为DM患者死于癌症的风险更高。
