Dankó Katalin, Ponyi Andrea, Constantin Tamás, Borgulya Gábor, Szegedi Gyula
From the 3rd Department of Internal Medicine, Division of Clinical Immunology, Medical and Health Science Center, University of Debrecen (KD, AP, GS), Debrecen; and the 2nd Department of Pediatrics, Semmelweis University, Faculty of Medicine (TC, GB), Budapest, Hungary.
Medicine (Baltimore). 2004 Jan;83(1):35-42. doi: 10.1097/01.md.0000109755.65914.5e.
The idiopathic inflammatory myopathies are characterized by chronic muscle inflammation and involvement of internal organs, which contribute considerably to the morbidity and mortality of the disease. We conducted the current study to determine the survival data for patients with idiopathic inflammatory myopathies according to the presence of extramuscular clinical manifestations. We also determined the cumulative survival probability and the long-term prognosis and analyzed the causes of death at a single clinical immunology center.A survival analysis was performed using data for 162 patients diagnosed between 1976 and 1997 according to Bohan and Peter's criteria. Patients were followed up for a minimum of 5 years (median, 101.5 mo) or to date of death. Cumulative survival probability was calculated by the Kaplan-Meier method. The influence of extraskeletal and extramuscular involvement was analyzed as prognostic factors for death by Cox proportional hazards survival model. Eighteen disease-specific deaths occurred; pulmonary and cardiac complications were the most frequent causes of death. Global survival rates were 95%, 92%, and 89% for 1, 5, and 10 years, respectively. Analysis for clinicopathologic subgroups revealed that cancer-associated myositis had the worst prognosis, while juvenile and overlap myositis had the best prognosis. Five- and 10-year survival rates were 94.2% and 89.4% for patients with primary polymyositis and 90.1% and 86.4% for primary dermatomyositis patients, respectively. In the whole group of patients with idiopathic inflammatory myopathy, cardiac (p < 0.01) and respiratory muscle involvement (p = 0.045) were significant prognostic factors for death. In the group of patients with primary polymyositis/dermatomyositis, cardiac involvement was the main prognostic factor for death (p < 0.01). Myositis patients described in this study have higher survival rates than reported previously worldwide. We examine the reasons for the differences between the data in the current study and the available survival data in the relevant literature.
特发性炎性肌病的特征是慢性肌肉炎症和内脏器官受累，这在很大程度上导致了该疾病的发病率和死亡率。我们开展了本研究，以根据是否存在肌肉外临床表现来确定特发性炎性肌病患者的生存数据。我们还确定了累积生存概率和长期预后，并在单一临床免疫中心分析了死亡原因。
根据博汉和彼得的标准，对1976年至1997年间确诊的162例患者的数据进行了生存分析。对患者进行了至少5年（中位数为101.5个月）的随访或直至死亡日期。采用Kaplan-Meier法计算累积生存概率。通过Cox比例风险生存模型分析骨骼外和肌肉外受累情况作为死亡预后因素的影响。发生了18例疾病特异性死亡；肺部和心脏并发症是最常见的死亡原因。1年、5年和10年的总体生存率分别为95%、92%和89%。对临床病理亚组的分析显示，癌症相关性肌炎预后最差，而青少年型和重叠性肌炎预后最好。原发性多发性肌炎患者的5年和10年生存率分别为94.2%和89.4%，原发性皮肌炎患者分别为90.1%和86.4%。在整个特发性炎性肌病患者组中，心脏受累（p<0.01）和呼吸肌受累（p=0.045）是死亡的重要预后因素。在原发性多发性肌炎/皮肌炎患者组中，心脏受累是死亡的主要预后因素（p<0.01）。本研究中描述的肌炎患者的生存率高于此前全球报告的生存率。我们研究了本研究数据与相关文献中现有生存数据之间差异的原因。
