抗RNA聚合酶III抗体与系统性硬化症患者的恶性肿瘤：欧洲抗风湿病联盟硬皮病试验与研究队列分析及筛查建议
Malignancies in Patients with Anti-RNA Polymerase III Antibodies and Systemic Sclerosis: Analysis of the EULAR Scleroderma Trials and Research Cohort and Possible Recommendations for Screening.
作者信息
Lazzaroni Maria-Grazia, Cavazzana Ilaria, Colombo Enrico, Dobrota Rucsandra, Hernandez Jasmin, Hesselstrand Roger, Varju Cecilia, Nagy Gabriella, Smith Vanessa, Caramaschi Paola, Riccieri Valeria, Hachulla Eric, Balbir-Gurman Alexandra, Chatelus Emmanuel, Romanowska-Próchnicka Katarzyna, Araújo Ana Carolina, Distler Oliver, Allanore Yannick, Airò Paolo
机构信息
From the Rheumatology and Clinical Immunology, University of Brescia and Spedali Civili of Brescia, Brescia; Rheumatology Unit, Azienda Ospedaliera Universitaria Integrata, Verona; Department of Internal Medicine and Medical Specialties, University Sapienza, Rome, Italy; Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland; Department of Clinical Sciences, Section of Rheumatology, Lund University, Lund, Sweden; Department of Rheumatology and Immunology, Medical Center, University of Pecs, Pecs, Hungary; Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium; Department of Internal Medicine, University Lille Nord-de-France, Lille; Department of Rheumatology, Strasbourg University Hospital, Strasbourg; Department of Rheumatology, University Paris Descartes and Cochin Hospital, Paris, France; B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion - Institute of Technology, Haifa, Israel; Department of Pathophysiology, Medical University of Warsaw and Department of Connective Tissue Diseases, Institute of Rheumatology, Warsaw, Poland; Unidade de Doenças Auto-Imunes, Serviço de Medicina 2, Hospital de Curry Cabral, Centro Hospitalar Lisboa Central, Lisbon, Portugal.
M.G. Lazzaroni, MD, Rheumatology and Clinical Immunology, University of Brescia and Spedali Civili of Brescia; I. Cavazzana, MD, Rheumatology and Clinical Immunology, Spedali Civili of Brescia; E. Colombo, MD, Rheumatology and Clinical Immunology, University of Brescia and Spedali Civili of Brescia; R. Dobrota, MD, Division of Rheumatology, University Hospital Zurich; J. Hernandez, MD, Division of Rheumatology, University Hospital Zurich; R. Hesselstrand, MD, PhD, Department of Clinical Sciences, Section of Rheumatology, Lund University; C. Varju, MD, Department of Rheumatology and Immunology, Medical Center, University of Pecs; G. Nagy, MD, Department of Rheumatology and Immunology, Medical Center, University of Pecs; V. Smith, MD, PhD, Rheumatology, Ghent University Hospital, Ghent University; P. Caramaschi, MD, Rheumatology Unit, Azienda Ospedaliera Universitaria Integrata; V. Riccieri, MD, Department of Internal Medicine and Medical Specialties, University Sapienza; E. Hachulla, MD, PhD, Department of Internal Medicine, University Lille Nord-de-France; A. Balbir-Gurman, MD, PhD, B. Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion - Institute of Technology; E. Chatelus, MD, Department of Rheumatology, Strasbourg University Hospital; K. Romanowska-Próchnicka, MD, Department of Pathophysiology, Medical University of Warsaw and Department of Connective Tissue Diseases, Institute of Rheumatology; A.C. Araújo, MD, Unidade de Doenças Auto-Imunes, Serviço de Medicina 2, Hospital de Curry Cabral, Centro Hospitalar Lisboa Central; O. Distler, MD, PhD, Division of Rheumatology, University Hospital Zurich; Y. Allanore, MD, PhD, Department of Rheumatology, University Paris Descartes and Cochin Hospital; P. Airò, MD, Rheumatology and Clinical Immunology, Spedali Civili of Brescia.
出版信息
J Rheumatol. 2017 May;44(5):639-647. doi: 10.3899/jrheum.160817. Epub 2017 Jan 15.
OBJECTIVE
To analyze the characteristics of anti-RNA polymerase III antibodies (anti-RNAP3)- positive patients with systemic sclerosis (SSc) in the European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR) registry with a focus on the risk of cancer and the characteristics of malignancies, and the aim to provide guidelines about potential cancer screening in these patients.
METHODS
(1) Analysis of the EUSTAR database: 4986 patients with information on their anti-RNAP3 status were included. (2) Case-control study: additional retrospective data, including malignancy history, were queried in 13 participating EUSTAR centers; 158 anti-RNAP3+ cases were compared with 199 local anti-RNAP3- controls, matched for sex, cutaneous subset, disease duration, and age at SSc onset. (3) A Delphi exercise was performed by 82 experts to reach consensus for cancer screening in anti-RNAP3+ patients.
RESULTS
In the EUSTAR registry, anti-RNAP3 were associated in multivariable analysis with renal crisis and diffuse cutaneous involvement. In the case-control study, anti-RNAP3 were associated with gastric antral vascular ectasia, rapid progression of skin involvement, and malignancies concomitant to SSc onset (OR 7.38, 95% CI 1.61-33.8). When compared with other anti-RNAP3+ patients, those with concomitant malignancies had older age (p < 0.001) and more frequent diffuse cutaneous involvement (p = 0.008). The Delphi exercise highlighted the need for malignancy screening at the time of diagnosis for anti-RNAP3+ patients and tight followup in the following years.
CONCLUSION
Anti-RNAP3+ patients with SSc have a high risk of concomitant malignancy. These results have implications for clinical practice and suggest regular screening for cancer in anti-RNAP3+ patients.
目的
分析欧洲抗风湿病联盟硬皮病试验与研究组（EUSTAR）注册中心中抗RNA聚合酶III抗体（抗RNAP3）阳性的系统性硬化症（SSc）患者的特征，重点关注癌症风险和恶性肿瘤的特征，并旨在为这些患者的潜在癌症筛查提供指导。
方法
（1）对EUSTAR数据库进行分析：纳入4986例有抗RNAP3状态信息的患者。（2）病例对照研究：在13个参与的EUSTAR中心查询了包括恶性肿瘤病史在内的额外回顾性数据；将158例抗RNAP3阳性病例与199例当地抗RNAP3阴性对照进行比较，根据性别、皮肤亚型、疾病持续时间和SSc发病年龄进行匹配。（3）82位专家进行了德尔菲法练习，以就抗RNAP3阳性患者的癌症筛查达成共识。
结果
在EUSTAR注册中心，多变量分析显示抗RNAP3与肾危象和弥漫性皮肤受累相关。在病例对照研究中，抗RNAP3与胃窦血管扩张、皮肤受累快速进展以及与SSc发病同时出现的恶性肿瘤相关（比值比7.38，95%置信区间1.61 - 33.8）。与其他抗RNAP3阳性患者相比，伴有恶性肿瘤的患者年龄更大（p < 0.001），弥漫性皮肤受累更频繁（p = 0.008）。德尔菲法练习强调了在抗RNAP3阳性患者诊断时进行恶性肿瘤筛查以及在随后几年进行密切随访的必要性。
结论
抗RNAP3阳性的SSc患者伴有恶性肿瘤的风险较高。这些结果对临床实践有影响，并建议对抗RNAP3阳性患者进行定期癌症筛查。
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