Background Patients with pediatric-onset immune-mediated inflammatory diseases (pIMID) show more aggressive phenotypes compared to patients diagnosed as adults. Despite this, data on mortality is extrapolated from patients diagnosed in adulthood, which might underestimate the actual risk. We aim to estimate the effect of pIMID compared to reference individuals from the general population on the long-term risk of all-cause mortality. Methods A population-based cohort study using the nationwide Danish health care registers. We included all patients diagnosed with pIMID in Denmark from 1980 to 2018 and matched them to up to ten reference individuals from the general population (with no recorded IMID) based on sex, age at diagnosis, and area of residence. Exposure was pIMID, defined as ICD codes indicative of autoimmune hepatitis, primary sclerosing cholangitis, Crohn’s disease, ulcerative colitis, juvenile idiopathic arthritis, system lupus erythematosus, or vasculitis registered before age 18. The primary outcome was all-cause mortality. The secondary outcome was cause-specific mortality. Cox survival analysis was used to estimate hazard ratios (HR) and Aalen survival analysis to estimate rate differences with corresponding 95% confidence intervals (CI) adjusted for the year of diagnosis and family income. Denmark has universal free health care, and health care data can be accessed through the nationwide health registers, continuously updated since 1980. Results We included 11,581 individuals diagnosed with pIMID, and 99,665 matched reference individuals, accounting for 1,371,994 person-years of follow-up. Median age at pIMID diagnosis was 12.6 years (IQR: 7.9 – 15.9). During follow-up, 152 pIMID patients and 316 reference individuals died, resulting in an all-cause mortality adjusted HR (aHR) of 3.8 (95% confidence interval [CI]: 3.1-4.7) compared to reference individuals without pIMID. This corresponded to 7.8 (95%CI: 6.1-9.5) additional deaths per 10,000 person-years. The strongest associations were found for gastrointestinal disorders (aHR 22.8 [95%CI: 9.6-64.1]), gastrointestinal cancers (aHR 19.2 [95%CI: 5.0-74.2]), and lymphoproliferative diseases (aHR 6.8 [95%CI: 2.8-16.8]). The aHR of suicide was 2.9 (95%CI: 1.6-5.0). Conclusion Patients diagnosed with pIMID have a four-fold increased risk of mortality when followed into adulthood. This underlines the severe disease course of pIMID and highlights the need for lifelong multidisciplinary care.

中文翻译：

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DOP63 儿童发病的免疫介导炎症性疾病的死亡率 – 一项全国性研究

背景 与成人诊断的患者相比，儿科发病的免疫介导的炎症性疾病 (pIMID) 患者表现出更具侵袭性的表型。尽管如此，死亡率数据是根据成年期诊断的患者推断出来的，这可能低估了实际风险。我们的目的是评估 pIMID 与普通人群的参考个体相比对全因死亡长期风险的影响。方法 使用丹麦全国医疗保健登记册进行基于人群的队列研究。我们纳入了 1980 年至 2018 年在丹麦诊断出的所有 pIMID 患者，并根据性别、诊断时年龄和居住地区将他们与来自普通人群（没有记录 IMID）的最多 10 名参考个体进行匹配。暴露为 pIMID，定义为指示 18 岁之前登记的自身免疫性肝炎、原发性硬化性胆管炎、克罗恩病、溃疡性结肠炎、幼年特发性关节炎、系统性红斑狼疮或血管炎的 ICD 代码。主要结局是全因死亡率。次要结果是特定原因死亡率。Cox 生存分析用于估计风险比 (HR)，Aalen 生存分析用于估计率差异，并根据诊断年份和家庭收入调整相应的 95% 置信区间 (CI)。丹麦实行全民免费医疗保健，并且可以通过自 1980 年以来不断更新的全国健康登记册获取医疗保健数据。 结果 我们纳入了 11,581 名诊断为 pIMID 的个体，以及 99,665 名匹配的参考个体，相当于 1,371,994 人年的随访。pIMID 诊断时的中位年龄为 12.6 岁（IQR：7.9 – 15.9）。在随访期间，152 名 pIMID 患者和 316 名参考个体死亡，与没有 pIMID 的参考个体相比，全因死亡率调整后 HR (aHR) 为 3.8（95% 置信区间 [CI]：3.1-4.7）。这相当于每 10,000 人年增加 7.8 例（95%CI：6.1-9.5）例死亡。胃肠道疾病 (aHR 22.8 [95%CI: 9.6-64.1])、胃肠道癌症 (aHR 19.2 [95%CI: 5.0-74.2]) 和淋巴组织增生性疾病 (aHR 6.8 [95%CI: 2.8]) 的相关性最强。 -16.8]）。自杀的 aHR 为 2.9 (95% CI: 1.6-5.0)。结论 诊断出 pIMID 的患者在成年后的死亡风险增加四倍。这凸显了 pIMID 的严重病程，并强调了终身多学科护理的需要。