Cancer risk in Sjögren’s disease: A longitudinal cohort study on incidence, predictors, and mortality impact

IF 4.6 2区医学 Q1 RHEUMATOLOGY

Seminars in arthritis and rheumatism Pub Date : 2025-05-04 DOI:10.1016/j.semarthrit.2025.152743

Olga Rusinovich-Lovgach , Zulema Plaza , Mónica Fernández Castro , Jose Rosas-Gómez de Salazar , Victot Manuel Martínez Taboada , Alejandro Olive , Raúl Menor Almagro , Belen Serrano Benavente , Judit Font Urgelles , Angel Garcia-Aparicio , Sara Manrique-Arija , Jesús Alberto Garcia Vadillo , Ruth Lopez-Gonzalez , Javier Narvaez García , Mª Beatriz Rodriguez Lozano , Carlos Galisteo , Jorge Juan Gonzalez Martin , Paloma Vela Casasempere , Elena Rabadán , Antonio Naranjo , José Luis Andréu Sánchez

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Abstract

Objectives

This study aimed to evaluate standardized incidence ratios (SIRs) of overall malignancies, hematologic malignancies and solid tumors in patients with Sjögren’s disease (SjD) compared to the general population. Furthermore, it sought to identify independent predictors of malignancy and quantify the impact of cancer on mortality.

Methods

This prospective, multicenter study included 314 patients clinically diagnosed with SjD and fulfilling 2002 American-European Consensus Group criteria, with a median follow-up of 9.5 years. Clinical, demographic, and serological data were collected, along with malignancy incidence and mortality outcomes. SIRs were calculated using GLOBOCAN data. Multivariate Cox regression identified malignancy predictors. The relative risk (RR) of death and the etiologic fraction in exposed individuals (EFE) assessed cancer-related mortality.

Results

A total of 22 malignancies (7.01%) were identified, including 11 hematologic malignancies (50%) and 11 solid tumors (50%). The overall cancer risk was increased (SIR: 1.68, 95% CI: 1.68–1.69), with a substantially higher risk for hematologic malignancies (SIR: 3.55, 95% CI: 3.54–3.56) and a moderate increase for solid tumors (SIR: 1.54, 95% CI: 1.53–1.55). All hematologic malignancies were non-Hodgkin lymphomas (NHL). Independent predictors of malignancy included older age, smoking, lymphadenopathy, splenomegaly, and cryoglobulinemia. Cancer was responsible for 23.8% of deaths (RR: 2.21, EFE: 55%).

Conclusions

Patients with SjD have an elevated malignancy risk, mainly driven by NHL, while solid tumor risk remains modest. Malignancy was a significant contributor to mortality. These findings underscore the need for better risk stratification and targeted surveillance in high-risk SjD patients for early detection and intervention.

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Sjögren疾病的癌症风险：一项关于发病率、预测因素和死亡率影响的纵向队列研究

目的：本研究旨在评估Sjögren 's disease （SjD）患者总体恶性肿瘤、血液恶性肿瘤和实体肿瘤的标准化发病率（SIRs）与普通人群的比较。此外，它还试图确定恶性肿瘤的独立预测因子，并量化癌症对死亡率的影响。方法本前瞻性多中心研究纳入314例临床诊断为SjD并符合2002年美欧共识组标准的患者，中位随访时间为9.5年。收集了临床、人口统计学和血清学数据，以及恶性肿瘤发病率和死亡率结果。使用GLOBOCAN数据计算sir。多变量Cox回归确定了恶性肿瘤的预测因子。死亡的相对危险度（RR）和暴露个体的病因学分数（EFE）评估癌症相关死亡率。结果共检出恶性肿瘤22例（7.01%），其中血液恶性肿瘤11例（50%），实体肿瘤11例（50%）。总体癌症风险增加（SIR: 1.68, 95% CI: 1.68 - 1.69），血液系统恶性肿瘤的风险显著增加（SIR: 3.55, 95% CI: 3.54-3.56），实体肿瘤的风险适度增加（SIR: 1.54, 95% CI: 1.53-1.55）。所有血液恶性肿瘤均为非霍奇金淋巴瘤（NHL）。恶性肿瘤的独立预测因子包括年龄较大、吸烟、淋巴结病、脾肿大和冷球蛋白血症。癌症占死亡的23.8%（相对危险度：2.21,EFE: 55%）。结论：SjD患者恶性肿瘤风险升高，主要由NHL驱动，而实体瘤风险保持适度。恶性肿瘤是死亡率的重要因素。这些发现强调了对高危SjD患者进行更好的风险分层和有针对性的监测以早期发现和干预的必要性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Seminars in arthritis and rheumatism 医学-风湿病学

CiteScore

9.20

自引率

4.00%

发文量

176

审稿时长

46 days

期刊介绍： Seminars in Arthritis and Rheumatism provides access to the highest-quality clinical, therapeutic and translational research about arthritis, rheumatology and musculoskeletal disorders that affect the joints and connective tissue. Each bimonthly issue includes articles giving you the latest diagnostic criteria, consensus statements, systematic reviews and meta-analyses as well as clinical and translational research studies. Read this journal for the latest groundbreaking research and to gain insights from scientists and clinicians on the management and treatment of musculoskeletal and autoimmune rheumatologic diseases. The journal is of interest to rheumatologists, orthopedic surgeons, internal medicine physicians, immunologists and specialists in bone and mineral metabolism.