摘要
目的 了解皮肌炎 (dermatomyositis ,DM)及多发性肌炎 (polymyositis,PM )患者的恶性肿瘤发生情况。方法 收集 1989— 1998年北京协和医院收治的资料完整的DM/PM患者 143例 ,全部符合 1975年Bohan和Peter提出的DM /PM诊断标准 ,对其中 18例合并肿瘤的患者与非肿瘤患者进行临床分析 ,肿瘤诊断及分型标准根据我国卫生部制定的肿瘤诊断分级标准而定。两组资料采用t及 χ2 检验。结果 143例患者男性 46 ,女性 97例。发病年龄 4～ 76岁 ,平均年龄 (42± 38)岁 ,其中PM5 0例 ,男 14例 ,女 36例 ,DM93例 ,男 2 4例 ,女 6 9例。PM的肿瘤发生率为 8% ,DM的肿瘤发生率为 15 6 %。肿瘤分型 :卵巢癌 3例 ,鼻咽癌 3例 ,恶组 2例 ,乳癌 2例 ,宫颈癌、子宫内膜卵巢样癌、肺癌、胃癌、前列腺癌、直肠癌、多发性骨髓瘤、淋巴瘤各 1例。肿瘤先行发生者 3例 (16 6 % ) ,后续发生者 5例 (2 7 8% ) ,同时发生者 10例 (5 5 6 % )。本组病例中 10例死亡 ,从PM /DM诊断到死亡的时间为 1～ 84个月 [中位数为 (18± 2 7)个月 ],死亡原因包括肿瘤扩散 ,肿瘤相关的多脏器功能衰竭及感染。发病年龄、肺部和心脏受累 ,吞咽困难等指标在两组间显示有统计学意义。其中 1例在肿瘤切除 1年后 ,PM症状消失 ,另 1例在肿瘤切除后 1
Objective To observe the association between dermatomyositis/polymyositis (DM/PM).Methods All 143 patients with DM/PM,admitted to Beijing Union Medical College Hospital during the 10 years (1988—1998),were studied for finding out prevalence and features of malignant disease among them. Patients with DM/PM alone,and with DM/PM and malignancy were analyzed clinically.The diagnosis and typing of malignancy in accordance with the China Health Ministry Organized Standard.Statistical analyses were done with χ 2 or t test.Results Of the 143 patients with DM/PM (46 men and 97women), the onset age was 4～76 years (42±38 years).There were 14men and 36 women in 50 PM and 24 men and 69 women in 93 DM patients.malignancy occurred in 4 patients with PM (8%) and 14 with DM (15 6%).More frequency in the DM/PM population was than in the general population. In 10 patients,the malignancy and the DM/PM were diagnosed simultaneously (55 6%),in 3 patients before (16 7%) and in 5 patients after(27 8%).Ovarian,nasopharyngeal,lung and gastric carcinomas,malignant histocytosis,prostate breast and colorectal carcinomas,lymphoma,myeloma endometria and cervical carcinomas were found among these patients.Ten DM/PM patients with malignancy died of infection,tumor spread and multiply organ dysfunction syndrome (MODS) during the study period.Time to death ranged between 1 and 84 months,mean (17±27) months from the diagnosis of DM/PM.Several clinical parameters have shown a statistically significant between two groups:age at onset ( P <0 005),heart involvement ( P <0 001),lung involvement P <0 001,and digestive system involvement ( P <0 002).Clinical manifestation of PM in one patient with ovarian cancer eliminated after ovariectomy 1 year.In another patient with DM and endometria carcinoma,clinical manifestation mitigated after uterectomy 1 5 years.Conclusion It is found that DM/PM is associated with high rate of malignancy.
