Chen Y J, Wu C Y, Shen J L
Department of Dermatology, Taichung Veterans General Hospital, no. 160, Sec. 3, Taichung-Kang Road, Taichung, Taiwan China Medical College, Taichung, Taiwan.
Br J Dermatol. 2001 Apr;144(4):825-31. doi: 10.1046/j.1365-2133.2001.04140.x.
An association between dermatomyositis (DM)/polymyositis (PM) and malignancies has been widely reported in the literature. The validity of extensive evaluation for malignancies in those patients has also been questioned for decades. Only limited papers regarding the signs of malignancy and the prognostic factors in DM/PM have been reported.
To define the potential risk factors of concomitant neoplastic diseases in patients diagnosed as having DM/PM.
From 1 April 1983 to 30 June 1999, 147 patients were diagnosed as having probable or definite DM/PM at the Veterans General Hospital, Taichung, Taiwan. We excluded four patients who had preceding neoplastic diseases diagnosed before DM/PM, then retrospectively reviewed the data of the remaining 143 patients and subgrouped the cases as four main types: primary idiopathic DM, primary idiopathic PM, juvenile DM/PM and amyopathic DM (ADM). We next performed univariate analysis using logistic regression to evaluate the possible predictive factors for malignancies, such as mean age at onset, gender, manifestations at onset, association with other connective tissue diseases, initial skin presentations, complications and laboratory data. Then we chose the significant factors for multivariate analysis by logistic regression, to determine the independent risk factors of malignancies in DM/PM patients.
Among the 143 patients, DM was the most common type (64%), followed by ADM (14%), juvenile DM/PM (13%) and PM (10%). The mean age at onset overall was 42.4 years. Other connective tissue diseases were present in 22% of all patients, especially PM (50%) and juvenile DM/PM patients (28%). Internal malignancies were present in 13% of patients, and most were associated with DM. Nasopharyngeal carcinomas (NPCs) were the most common tumours. Patients with primary idiopathic DM, with an older age at onset, higher serum creatine phosphokinase levels and male gender, had more chance of developing concomitant malignancies. Those associated with complications, especially interstitial lung diseases, had a lower risk of associated neoplasia. In multivariate analysis, an older age at onset (odds ratio 9.10) and male gender (odds ratio 4.06) were associated with greater risk of developing malignancies.
The two independent predictive factors for malignancy (P < 0.05) in patients with DM/PM were an older age at onset (> 45 years) and male gender. The primary idiopathic DM group was shown to have higher risk of developing internal malignancies, especially NPC. However, this was not identified as an independent predictive factor for concomitant neoplastic diseases in multivariate analysis. In addition, patients who had the complication of interstitial lung disease had a significantly lower frequency of malignancies (P < 0.001).
皮肌炎（DM）/多发性肌炎（PM）与恶性肿瘤之间的关联在文献中已有广泛报道。数十年来，对这些患者进行恶性肿瘤广泛评估的有效性也一直受到质疑。关于DM/PM中恶性肿瘤迹象和预后因素的报道仅有寥寥数篇。
确定被诊断为DM/PM的患者并发肿瘤性疾病的潜在危险因素。
1983年4月1日至1999年6月30日，台湾台中荣民总医院确诊了147例可能或确诊的DM/PM患者。我们排除了4例在DM/PM之前被诊断患有先前肿瘤性疾病的患者，然后回顾性分析了其余143例患者的数据，并将病例分为四种主要类型：原发性特发性DM、原发性特发性PM、青少年DM/PM和无肌病性DM（ADM）。接下来，我们使用逻辑回归进行单因素分析，以评估恶性肿瘤的可能预测因素，如发病时的平均年龄、性别、发病表现、与其他结缔组织疾病的关联、初始皮肤表现、并发症和实验室数据。然后，我们通过逻辑回归选择显著因素进行多因素分析，以确定DM/PM患者恶性肿瘤的独立危险因素。
在143例患者中，DM是最常见的类型（64%），其次是ADM（14%）、青少年DM/PM（13%）和PM（10%）。总体发病时的平均年龄为42.4岁。22%的患者存在其他结缔组织疾病，尤其是PM患者（50%）和青少年DM/PM患者（28%）。13%的患者存在内部恶性肿瘤，且大多数与DM相关。鼻咽癌（NPC）是最常见的肿瘤。原发性特发性DM患者，发病年龄较大、血清肌酸磷酸激酶水平较高且为男性，发生并发恶性肿瘤的机会更大。那些伴有并发症，尤其是间质性肺病的患者，发生相关肿瘤的风险较低。在多因素分析中，发病年龄较大（比值比9.10）和男性（比值比4.06）与发生恶性肿瘤的风险较高相关。
DM/PM患者发生恶性肿瘤（P<0.05）的两个独立预测因素是发病年龄较大（>45岁）和男性。原发性特发性DM组发生内部恶性肿瘤的风险较高，尤其是NPC。然而，在多因素分析中，这并未被确定为并发肿瘤性疾病的独立预测因素。此外，患有间质性肺病并发症的患者发生恶性肿瘤的频率显著较低（P<0.001）。
