Department of Clinical Sciences Lund, Nephrology, Lund University; Department of Laboratory Medicine, Clinical Chemistry and Pharmacology, Lund University; Department of Clinical Sciences, Rheumatology, Lund University; Department of Clinical Sciences Lund, Clinical Epidemiology Unit, Lund University, Lund, Sweden; Department of Medicine, University of Cambridge, Cambridge, UK.
C. Heijl, MD, PhD, Specialist in Nephrology, Department of Clinical Sciences Lund, Nephrology, Lund University; K. Westman, MD, PhD, Senior Consultant Nephrologist, Department of Clinical Sciences Lund, Nephrology, Lund University; P. Höglund, MD, PhD, Professor, Department of Laboratory Medicine, Clinical Chemistry and Pharmacology, Lund University; A.J. Mohammad, MD, MPH, PhD, Senior Consultant Rheumatologist, Department of Clinical Sciences, Rheumatology, Lund University, and Department of Clinical Sciences Lund, Clinical Epidemiology Unit, Lund University, and Department of Medicine, University of Cambridge.
J Rheumatol. 2020 Aug 1;47(8):1229-1237. doi: 10.3899/jrheum.181438. Epub 2019 Sep 1.
Patients with ANCA-associated vasculitides (AAV) exhibit higher rates of malignancy than the general population. We assessed whether the cancer risk is increased in a well-characterized population-based cohort of AAV in southern Sweden, followed for a median time of 8 years.
With case record review, the outcomes and malignancy development in a cohort of 195 patients with AAV [granulomatosis with polyangiitis (GPA), microscopic polyangiitis, and eosinophilic GPA] diagnosed between 1997 and 2010 were assessed. The patients were followed until death or December 31, 2015. The age- and sex-standardized incidence ratios (SIR) were estimated using the Swedish population data as a reference.
During the observation period of about 1500 person-years, we found 60 cancers in 52 of the 195 patients. SIR (95% CI) was 2.8 (2.1-3.6) for cancers at all sites, 1.8 (1.3-2.5) for all cancers excluding squamous cell carcinoma (SCC), 12.9 (8.4-18.8) for SCC, 4.3 (1.4-10.0) for bladder cancer, and 7.0 (1.4-20.5) for pancreatic cancer. Cumulative doses of cyclophosphamide (CYC) < 10 g were not associated with higher incidence of cancers other than SCC (SIR 1.63, 95% CI 0.8-2.9).
In contrast to previous publications assessing malignancy risk in patients with AAV, we show in this population-based cohort of patients a persistent increased risk for overall malignancy, bladder cancer, and pancreatic cancer as well as a markedly increased risk for SCC. There was no increase in incidence of cancers other than SCC for those treated with < 10 g CYC.
抗中性粒细胞胞浆抗体相关性血管炎（AAV）患者的恶性肿瘤发病率高于普通人群。我们评估了在瑞典南部一个特征明确的基于人群的 AAV 队列中，中位时间为 8 年的随访中，癌症风险是否会增加。
通过病例记录回顾，评估了 195 例 AAV [肉芽肿性多血管炎（GPA）、显微镜下多血管炎和嗜酸性粒细胞 GPA]患者队列的结局和恶性肿瘤的发生情况。这些患者的随访时间截止到死亡或 2015 年 12 月 31 日。使用瑞典人群数据作为参考，估计年龄和性别标准化发病率比（SIR）。
在大约 1500 人年的观察期间，我们在 195 例患者中的 52 例中发现了 60 例癌症。所有部位癌症的 SIR（95%CI）为 2.8（2.1-3.6），所有部位癌症（不包括鳞状细胞癌[SCC]）的 SIR 为 1.8（1.3-2.5），SCC 的 SIR 为 12.9（8.4-18.8），膀胱癌的 SIR 为 4.3（1.4-10.0），胰腺癌的 SIR 为 7.0（1.4-20.5）。环磷酰胺（CYC）累积剂量<10g 与 SCC 以外癌症的发病率增加无关（SIR 1.63，95%CI 0.8-2.9）。
与评估 AAV 患者恶性肿瘤风险的先前出版物不同，我们在这个基于人群的患者队列中显示出总体恶性肿瘤、膀胱癌和胰腺癌的持续增加风险，以及 SCC 的显著增加风险。对于接受<10g CYC 治疗的患者，SCC 以外癌症的发病率没有增加。
