特发性炎性肌病相关性肿瘤的临床研究

Retrospective study of idiopathic inflammatory myopathies associated malignancy

目的研究特发性炎性肌病(IIM,即多发性肌炎和皮肌炎)合并肿瘤的发生率、临床表现特点及危险因素。方法回顾性调查分析卫生部中日友好医院风湿免疫科1986年3月至2007年8月就诊的多发性肌炎、皮肌炎患者143例的临床表现、辅助检查、实验室参数的特点,比较IIM伴发肿瘤与不伴发肿瘤患者的差异。结果在143例特发性炎性肌病患者中,有10例并发肿瘤,发生率6.99%,其中8例为皮肌炎(80.00%),2例为多发性肌炎(20.00%);男4例,女6例;平均发病年龄明显高于不伴肿瘤的IIM患者的发病年龄[(56.6±15.5)岁比(41.6±15.1)岁;P<0.05)]。平均病程(55.3±66.7)个月;3例患者肿瘤发生于IIM诊断前;1例在诊断IIM时同时被诊断为肿瘤;6例发生于诊断之后。发生肿瘤的类型:肺癌3例,乳腺癌2例,鼻咽癌、食管癌、慢性淋巴细胞白血病、卵巢癌、胸腺肿瘤各1例。临床资料完整的8例中没有患者并发肺间质性病变(ILD),不伴肿瘤的IIM患者ILD的发生率为32.3%,但两组ILD的发生率差异无统计学意义(P>0.05)。发热、肌痛、四肢无力、Gottron征、Helitrope征、抬头困难、吞咽困难、肌酸激酶(CK)及血沉(ESR)值的升高程度与是否合并肿瘤均无明显差异。结论较高年龄发病的IIM患者肿瘤发生率更高,皮肌炎较多发性肌炎患者可能更易合并肿瘤,伴发的肿瘤类型具有多样性。临床上有肺间质病变的患者伴发肿瘤的风险可能较低。

Objective To investigate the incidence, clinical feature, risk factors of the idiopathic inflammatory myopathies（IIM） associated with malignant diseases. Methods Clinical and laboratory data of the patients diagnosed with IIM （e. g. polymyositis and dermatomyositis ） between 1986 and 2007 in our hospital were collected and analyzed. Results Out of 143 patients with IIM in our database, malignant diseases were found in 10 cases （4 male and 6 female）. The incidence was 6. 99%. Eight patients had dermatomyositis （80%） , and 2 had polymyositis （20%）. The mean age of IIM patients with malignancies at the time of the diagnosis was higher than that of the patients without manligancy （56. 6 ± 15.5 vs. 41.6± 15.1 ;P 〈 0. 05 ）. Malignancies happened before the diagnosis of IIM in 3 cases ; occurred at the same time in 1 patients, and 6 cases after the diagnosis. Types of the tumor were ： 3 for pulmonary cancer, 2 for breast cancer,1 for nasopharyngeal tumor, esophageal tumor, chronic lymphocytic leukemia, ovarian cancer and thymic tumor. No interstitial lung disease （ILD） were found in the IIM with malignancies. The ILD incidence of the IIM patients without malignancy was 32. 3%, but there was no statistical significant in two groups （P = 0. 106 ）. No significant differences of the other clinical features and laboratory parameters were observed between the IIM with and without malignant diseases. Conclusions Malignant diseases were more frequently occurred in older age patients, and in dermatomyositis than in polymyositis. ILD seems negative correlated with malignancy in IIM patients.