Objectives The association between cancer and IgG4-related disease (IgG4-RD) is evolving. The primary aim of this study was to investigate the prevalence of malignancies in IgG4-RD. The secondary aim was to describe the epidemiological and clinical characteristics of IgG4-RD patients with a history of cancer. Methods Two hundred and ten patients with IgG4-RD were included in this retrospective study. IgG4-RD phenotypes, clinical and serological variables were analyzed. The prevalence of cancer in IgG4-RD was compared with that in the Italian population using the registry of the Global Cancer Observatory (GCO) of the World Health Organization. The Standardized Incidence Ratio (SIR) for cancer in IgG4-RD was obtained based on the 5-years Limited Duration Prevalence (2015–2020) of tumors in the Italian population. Results Thirty-seven/210 patients (18%) developed cancer before or after the diagnosis of IgG4-RD. Solid and hematologic tumors were more frequently observed in pancreato-biliary IgG4-RD. The SIR for malignancy in IgG4-RD patients was 2.54 higher than the general Italian population (p= 0.007). The SIR was 2.78 higher for males (p= 0.005) and 1.15 higher for females (p> 0.05). Thirty-two malignancies were diagnosed before and 16 after IgG4-RD diagnosis. Interval “from IgG4-RD to cancer” was shorter than that “from cancer to IgG4-RD”. Most tumors occurring after IgG4-RD developed within 36 months from diagnosis of IgG4-RD. Conclusions The prevalence of cancer in patients with IgG4-RD is increased compared with the Italian population and mechanistically suggests a possible paraneoplastic association. Close surveillance is warranted for the first 36 months after IgG4-RD diagnosis.

中文翻译：

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IgG4 相关疾病患者恶性肿瘤患病率增加：对临床护理的影响

目标 癌症与 IgG4 相关疾病 (IgG4-RD) 之间的关联正在不断发展。本研究的主要目的是调查 IgG4-RD 中恶性肿瘤的患病率。第二个目的是描述有癌症病史的 IgG4-RD 患者的流行病学和临床特征。方法回顾性研究210例IgG4-RD患者。分析了 IgG4-RD 表型、临床和血清学变量。使用世界卫生组织全球癌症观测站 (GCO) 的登记数据，将 IgG4-RD 中的癌症患病率与意大利人群中的癌症患病率进行了比较。 IgG4-RD 中癌症的标准化发病率 (SIR) 是根据意大利人群中肿瘤的 5 年有限持续流行率（2015-2020 年）获得的。结果 210 名患者中有 37 名 (18%) 在 IgG4-RD 诊断之前或之后患上癌症。实体瘤和血液肿瘤在胰胆管 IgG4-RD 中更常见。 IgG4-RD 患者的恶性肿瘤 SIR 比一般意大利人群高 2.54 (p= 0.007)。男性的SIR高出2.78(p＝0.005)，女性的SIR高出1.15(p＞0.05)。 IgG4-RD 诊断前诊断出 32 例恶性肿瘤，诊断后诊断出 16 例恶性肿瘤。 “从IgG4-RD到癌症”的间隔比“从癌症到IgG4-RD”的间隔短。大多数 IgG4-RD 后发生的肿瘤在 IgG4-RD 诊断后 36 个月内发生。结论 与意大利人群相比，IgG4-RD 患者的癌症患病率有所增加，从机制上提示可能存在副肿瘤相关性。 IgG4-RD 诊断后的前 36 个月内需要进行密切监测。