Rheumatology and Clinical Immunology Service, Spedali Civili and University of Brescia, Brescia, Italy.
J Rheumatol. 2011 Jul;38(7):1329-34. doi: 10.3899/jrheum.101144. Epub 2011 Apr 1.
To evaluate the frequency of malignancies in Italian patients with systemic sclerosis (SSc) and anti-RNA polymerase III (RNAP III), antitopoisomerase I (topo I), or anticentromere antibodies (ACA); and to characterize the temporal relationship between the 2 diseases, in order to confirm data suggesting a close temporal relationship between the onset of SSc and malignancy in American patients with anti-RNAP III antibodies.
From a cohort of 466 consecutive SSc patients, 360 Italians with isolated positivity for anti-RNAP III (n = 16), anti-topo I (n = 101), or ACA (n = 243) were identified. Malignancy cases were divided according to their relationship with SSc onset into 3 categories: preceding, synchronous with, or metachronous to the onset of SSc (diagnosed more than 6 months before; 6 months before to 12 months after; and more than 12 months after onset of SSc, respectively).
Malignancies were more frequent in the anti-RNAP III group (7/16 patients), than in the anti-topo I (11/101) and ACA groups (21/243) (p < 0.001). This difference was accounted for by the number of patients with cancer synchronous to the onset of SSc (3/16 in the anti-RNAP III group vs 0/101 in the anti-topo I and 1/243 in the ACA group; p < 0.001), whereas neither the number of malignancies preceding nor those metachronous to the onset of SSc was significantly different between the groups.
In a cohort of Italian patients with SSc we observed a significant association between malignancies synchronous to SSc onset and positivity for anti-RNAP III antibodies, similar to that described in American patients with SSc.
评估意大利系统性硬化症（SSc）患者伴抗 RNA 聚合酶 III（RNAP III）、抗拓扑异构酶 I（topo I）或抗着丝点抗体（ACA）的恶性肿瘤发病率，并对这两种疾病之间的时间关系进行特征分析，以证实美国抗 RNA 聚合酶 III 抗体阳性的 SSc 患者中存在 SSc 发病与恶性肿瘤发生之间密切时间关系的相关数据。
从一组 466 例连续 SSc 患者中，鉴定出 360 例意大利患者，他们的抗 RNA 聚合酶 III（n = 16）、抗拓扑异构酶 I（n = 101）或 ACA（n = 243）单独呈阳性。根据其与 SSc 发病的关系，将恶性肿瘤病例分为 3 类：发病前、发病同期或发病后（分别定义为 SSc 发病前超过 6 个月；6 个月前至 12 个月内；以及 SSc 发病后超过 12 个月）。
抗 RNA 聚合酶 III 组（7/16 例患者）的恶性肿瘤发病率高于抗拓扑异构酶 I 组（11/101 例患者）和 ACA 组（21/243 例患者）（p < 0.001）。这一差异归因于发病同期的 SSc 患者中癌症的数量（抗 RNA 聚合酶 III 组 3/16 例患者 vs 抗拓扑异构酶 I 组 0/101 例患者和 ACA 组 1/243 例患者；p < 0.001），而发病前或发病后恶性肿瘤的数量在各组之间没有显著差异。
在意大利 SSc 患者队列中，我们观察到 SSc 发病同期恶性肿瘤与抗 RNA 聚合酶 III 抗体阳性之间存在显著关联，这与美国 SSc 患者的描述相似。
