抗中性粒细胞胞浆抗体相关性血管炎伴发肿瘤的临床特征

Clinical Manifestations of Patients with Antineutrophil Cytoplasm Antibody-associated Vasculitis Complicated with Malignancy

目的探讨抗中性粒细胞胞浆抗体(anti-neutrophil cytoplasmic antibody,ANCA)相关性血管炎(anti-neutrophil cytoplasmic antibody-associated vasculitis,AAV)合并肿瘤患者的临床表现及危险因素。方法回顾性分析北京协和医院风湿免疫科2005至2013年AAV住院患者病历资料,并以此作为研究组,按照1∶4以系统抽样方法选取不合并肿瘤的AAV患者作为对照组。对2组患者临床表现及实验室指标进行比较,并分析肿瘤发生高危因素。结果 AAV住院患者共442例,其中合并肿瘤患者16例(肿瘤组),抽样65例非肿瘤患者作为对照组。肿瘤组16例患者中,罹患16例次肿瘤,包括肺癌和淋巴瘤各4例,膀胱癌、前列腺癌和乳腺癌各2例,宫颈癌和胃癌各1例。AAV合并肿瘤患者家族癌症史多于对照组(50.0%vs.18.5%,P<0.05),病程长于对照组[17(0.5,120)个月vs.4(0.1,240)个月,P<0.05],且差异有统计学意义;其心脏受累(25.0%vs.58.5%)、血小板计数升高(18.8%vs.56.9%)、总补体(CH50)升高(25.0%vs.55.0%)、血红蛋白下降(43.8%vs.70.8%)及ANCA阳性(68.8%vs.90.8)比例均低于对照组,差异有统计学意义(P<0.05)。结论 AAV患者合并肿瘤以肺癌和淋巴瘤多见。肿瘤家族史或炎性指标缺如或ANCA阴性的小血管炎者应高度警觉肿瘤伴发可能,心血管病变发生率低需扩大病例进一步研究。

Objectives To explore the clinical features and potential link between cancer and anti-neutrophil cytoplasmic antibody-associated vasculitis （AAV）. Methods This retrospective study involved 442 patients with AVV in Peking Union Medical College Hospital between 2005 and 2013. The clinical manifestations and laboratory parameters were compared between AAV patient with and without cancer, and the sample size of patient with cancer versus without cancer was 1 ： 4. The risk factors of cancer in patients with AAV were analyzed. Results There were 16 cancers were diagnosed in 16 patients, including 4 lung cancer, 4 lymphoma, 2 bladder cancer, 2 prostate cancer, 2 breast cancer, 1 cervical cancer, and 1 gastric cancer. Compared to patients without cancer, more family history of vs. 18.5%, P 〈 0.05 ） and longer disease duration [ 17 （0.5, 120） months vs. 4 P 〈 0. 05 ] were observed in patients with cancer. Furthermore, cardiac involvement elevated platelet （18.8% vs. 56. 9% ）, elevated complement （CH50） （25.0% vs. malignancy （ 50. 0% （0. 1, 240） months, （25.0% vs. 58.5% ） , 55.0% ） , hemoglobin （43.8% vs. 70. 8% ）, and positive ANCA （68.8 % vs. 90. 8 ） were significantly less in patients with cancer （P 〈 O. 05 ）. Conclusions The most prominent cancers observed in our cohort are lung cancer and lymphoma. Patients with normal inflammation markers, negative ANCA, and family history of malignancy are higher risks of cancer. For fewer cases of cardiovascular disease, we need expand the number of eases to study further.