Arya Rigya, Sharpe Isobel, Cheng Stephanie Y, Sykes Jenna, Ma Xiayi, Stanojevic Sanja, Rochon Paula A, Li Ping, Quon Brad, Ordon Michael, Stephenson Anne L
St Michael's Hospital, Division of Respirology, Toronto, Ontario, Canada.
Institute for Clinical Evaluative Sciences, Toronto, Ontario, Canada.
Ann Am Thorac Soc. 2025 Jul 28. doi: 10.1513/AnnalsATS.202502-170OC.
Introduction People with cystic fibrosis (pwCF) are living longer with increasing comorbidities. The objective of this study was to estimate the rate of emerging non-pulmonary comorbidities in adults with CF and to compare these rates with the non-CF population. Methods This is a population-based cohort study of adults using Canadian CF Registry data linked with health administrative databases in Ontario, Canada. Cardiovascular disease (CVD) and symptomatic kidney stone cases were identified using diagnostic and procedural codes. Chronic kidney disease (CKD) was defined as eGFR<60 mL/min/1.73m2. Cancer cases were obtained using the Ontario Cancer Registry. Poisson regression was used to estimate the rates per 1,000 person-years of follow-up. Results The age- and sex-adjusted rates of CVD, CKD, kidney stones, and cancer per 1,000 person years in the non-lung transplant cohort were 24.5 (95% CI 21.5-28.0), 3.7 (95% CI 2.7-5.2), 7.4 (95% CI 6.1-9.0), and 5.8 (95% CI 4.5-7.6) respectively. pwCF who received lung transplant had higher rates of all four conditions, and cancer and CKD occurred earlier compared to the non-transplant cohort. When comparing the CF to the non-CF population, pwCF without lung transplant had higher age- and sex-adjusted rates of CVD (RR 2.9, 95% CI 2.6-3.4), CKD (RR 2.1, 95% CI 1.5-2.9), kidney stones (RR 2.9, 95% CI 2.4-3.6) and cancer (RR 1.9, 95% CI 1.5-2.5). These events occurred at a median age of at least 20 years earlier in the CF cohort. In the post-transplant population, the rates of CVD, kidney stones, and cancers were similar between pwCF and the non-CF population, however events occurred earlier in pwCF. Conclusion Non-pulmonary complications occur at a high rate and at a younger age in pwCF compared to the non-CF population which highlights the importance of incorporating these issues in CF care models.
患有囊性纤维化（pwCF）的患者寿命更长，但合并症也日益增多。本研究的目的是估计成年CF患者中出现的非肺部合并症的发生率，并将这些发生率与非CF人群进行比较。方法：这是一项基于人群的成年队列研究，使用加拿大CF登记数据与加拿大安大略省的卫生行政数据库相链接。通过诊断和程序编码识别心血管疾病（CVD）和有症状的肾结石病例。慢性肾脏病（CKD）定义为估算肾小球滤过率（eGFR）<60 mL/min/1.73m²。癌症病例通过安大略癌症登记处获取。使用泊松回归来估计每1000人年随访的发生率。结果：在非肺移植队列中，每1000人年经年龄和性别调整后的CVD、CKD、肾结石和癌症发生率分别为24.5（95%置信区间21.5 - 28.0）、3.7（95%置信区间2.7 - 5.2）、7.4（95%置信区间6.1 - 9.0）和5.8（95%置信区间4.5 - 7.6）。接受肺移植的pwCF这四种疾病的发生率均更高，且与非移植队列相比，癌症和CKD出现得更早。当将CF患者与非CF人群进行比较时，未接受肺移植的pwCF经年龄和性别调整后的CVD发生率（相对风险RR 2.9，95%置信区间2.6 - 3.4）、CKD发生率（RR 2.1，95%置信区间1.5 - 2.9）、肾结石发生率（RR 2.9，95%置信区间2.4 - 3.6）和癌症发生率（RR 1.9，95%置信区间1.5 - 2.5）更高。这些事件在CF队列中的发生年龄中位数至少早20岁。在移植后人群中，pwCF与非CF人群的CVD、肾结石和癌症发生率相似，但pwCF的事件发生更早。结论：与非CF人群相比，pwCF的非肺部并发症发生率高且发病年龄更小，这突出了将这些问题纳入CF护理模式的重要性。
