目的： 研究血清抗转录中介因子1-γ（transcriptional intermediary factor 1-γ, TIF1-γ）抗体在诊断皮肌炎（dermatomyositis, DM）和临床无肌病性皮肌炎（clinically amyopathic dermatomyositis, CADM）合并恶性肿瘤中的价值。方法： 2016年1月至2019年12月间,收集上海交通大学医学院附属瑞金医院皮肤科病房收治的223例DM和CADM患者[包括209例成人DM（107例）和CADM（102例）,5例青少年DM（juvenile DM, JDM）和9例青少年JCADM（juvenile CADM, JCADM）]的临床资料。成人DM和成人CADM分别被分为恶性肿瘤组和无肿瘤组。初诊时血清用酶联免疫吸附测定法检测患者血清抗TIF1-γ抗体,分别比较成人DM和成人CADM患者中恶性肿瘤与无肿瘤患者之间抗TIF1-γ抗体的阳性率,进一步比较抗TIF1-γ抗体阳性与抗体阴性患者之间恶性肿瘤的发生率。结果： ①成人DM和CADM中恶性肿瘤共42例（20.1%）,占前3位的分别是鼻咽癌、乳腺癌和肺癌。JDM和JCADM患者中无一例发生恶性肿瘤。②成人DM和CADM中有74例（74/209,35.4%）血清抗TIF1-γ抗体阳性,其中DM患者抗TIF1-γ抗体阳性率33.6%（36/107）,CADM患者抗TIF1-γ抗体阳性率37.3%（38/102）。5例JDM患者中,无一例抗TIF1-γ抗体阳性;9例JCADM患者中,3例抗TIF1-γ抗体阳性。③成人DM中,恶性肿瘤患者的抗TIF1-γ抗体阳性率显著高于无肿瘤患者[26/35(74.3%)比10/72(13.9%), P<0.000 1];抗TIF1-γ抗体阳性患者恶性肿瘤的发生率显著高于阴性患者[26/36 (72.2%)比9/71 (12.7%), P<0.000 1]。成人CADM中,恶性肿瘤患者与无肿瘤患者之间抗TIF1-γ抗体的阳性率差异无统计学意义[4/7比34/95(35.8%), P=0.420];抗TIF1-γ抗体阳性与抗体阴性患者之间恶性肿瘤的发生率差异无统计学意义[4/38(10.5%)比3/64(4.7%), P=0.420]。结论： 抗TIF1-γ抗体阳性与成人DM患者恶性肿瘤的发生相关,而与成人CADM患者恶性肿瘤发生无关。抗TIF1-γ抗体可能是成人DM合并恶性肿瘤的血清标志物,对于抗TIF1-γ抗体阳性的成人DM患者需要加强恶性肿瘤筛查。

Objective: To explore significance of anti-transcriptional intermediary factor 1-γ (TIF1-γ) antibody detection in diagnosing ermatomyositis (DM) malignancy or clinically amyopathic dermatomyositis (CADM) accompanied by malignancy diseases. Methods: A total of hospitalized 223 patients were enrolled at Department of Dermatology, Ruijin Hospital during Jan. 2016 to Dec. 2019, including 107 cases of adult DM, 102 cases of adult CADM, 5 cases of juvenile DM, and 9 cases of juvenile CADM. DM patients were divided into malignant DM group and non-malignant DM group, and so were the CADM patients. Enzyme-linked immunosorbent assay(ELISA) was used to detect serum anti-TIF1-γ antibodies in patients at first visit. The positive rates of anti-TIF1-γ antibody were compared between malignant DM group and non-malignant DM as well as between malignant CADM group and non-malignant CADM group. Results： It revealed that 35 adult DM and 7 adult CADM patients were diagnosed as having malignant neoplasia, and nasopharyngeal carcinoma, breast cancer and lung cancer were on the top three of cancer-associated myositis in our cohort, however, there was no malignancy detected in JDM and JCADM patients. Seventy-four (35.4%) adult patients had serum anti-TIF1-γ antibody detected, including 36 cased of DM (33.6%) and 38 cases of CADM (37.3%). Serum anti-TIF1-γ antibody was positive in 3 cases of JCADM (33.3%), but none in total of 5 cases of JDM. The positive rate of anti-TIF1-γ antibody in DM patients with malignant tumor was significantly higher than those without malignant tumor[26/35(74.3%) vs 10/72(13.9%), P<0.000 1]. The incidence of malignant tumor in patients with positive anti-TIF1-γ antibody was significantly higher than those with negative antibody[26/36(72.2%) vs 9/71(12.7%), P<0.000 1]. However there was no significant difference in the positive rate of anti-TIF1-γ antibody between patients with malignant tumor and without malignant tumor [4/7 vs 34/95(35.8%), P=0.420] in adult CADM. There was no statistically significant difference in the incidence of malignancy between anti-TIF1-γ antibody-positive and antibody-negative patients[4/38 (10.5%) vs 3/64(4.7%), P=0.420]. Conclusions： Serum anti-TIF1-γ antibody correlates with the occurrence of malignancy diseases in DM, but has no relationship with malignancy diseases in CADM. Thus, anti-TIF1-γ antibody might be the serum biomarker for DM associated with malignancy, and oncology screening should be intensified in adult DM patients with positive anti-TIF1-γ antibody.