Background & Aims

Improved therapy has substantially increased survival of persons with cystic fibrosis (CF). But the risk of colorectal cancer (CRC) in adults with CF is 5−10 times greater compared to the general population, and 25−30 times greater in CF patients after an organ transplantation. To address this risk, the CF Foundation convened a multi-stakeholder task force to develop CRC screening recommendations.

Methods

The 18-member task force consisted of experts including pulmonologists, gastroenterologists, a social worker, nurse coordinator, surgeon, epidemiologist, statistician, CF adult, and a parent. The committee comprised 3 workgroups: Cancer Risk, Transplant, and Procedure and Preparation. A guidelines specialist at the CF Foundation conducted an evidence synthesis February−March 2016 based on PubMed literature searches. Task force members conducted additional independent searches. A total of 1159 articles were retrieved. After initial screening, the committee read 198 articles in full and analyzed 123 articles to develop recommendation statements. An independent decision analysis evaluating the benefits of screening relative to harms and resources required was conducted by the Department of Public Health at Erasmus Medical Center, Netherlands using the Microsimulation Screening Analysis model from the Cancer Innervation and Surveillance Modeling Network. The task force included recommendation statements in the final guideline only if they reached an 80% acceptance threshold.

Results

The task force makes 10 CRC screening recommendations that emphasize shared, individualized decision-making and familiarity with CF-specific gastrointestinal challenges. We recommend colonoscopy as the preferred screening method, initiation of screening at age 40 years, 5-year re-screening and 3-year surveillance intervals (unless shorter interval is indicated by individual findings), and a CF-specific intensive bowel preparation. Organ transplant recipients with CF should initiate CRC screening at age 30 years within 2 years of the transplantation because of the additional risk for colon cancer associated with immunosuppression.

Conclusions

These recommendations aim to help CF adults, families, primary care physicians, gastroenterologists, and CF and transplantation centers address the issue of CRC screening. They differ from guidelines developed for the general population with respect to the recommended age of screening initiation, screening method, preparation, and the interval for repeat screening and surveillance.

中文翻译：

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囊性纤维化结直肠癌筛查共识建议

背景与目标

改进的治疗大大提高了囊性纤维化 (CF) 患者的存活率。但与普通人群相比，患有 CF 的成年人患结直肠癌 (CRC) 的风险高 5-10 倍，器官移植后 CF 患者的风险高 25-30 倍。为解决这一风险，CF 基金会召集了一个多方利益相关者工作组来制定 CRC 筛查建议。

方法

这个由 18 名成员组成的工作组由专家组成，包括肺科医生、胃肠病学家、一名社会工作者、护士协调员、外科医生、流行病学家、统计学家、CF 成人和一名家长。该委员会由 3 个工作组组成：癌症风险、移植以及程序和准备工作组。CF 基金会的一位指南专家在 2016 年 2 月至 3 月基于 PubMed 文献搜索进行了证据综合。工作组成员进行了额外的独立搜索。共检索到 1159 篇文章。经过初步筛选，委员会全文阅读了 198 篇文章，分析了 123 篇文章以制定推荐声明。伊拉斯姆斯医学中心公共卫生部进行了一项独立决策分析，评估筛查相对于危害和所需资源的好处，荷兰使用来自癌症神经支配和监测建模网络的微观模拟筛选分析模型。只有在达到 80% 的接受度阈值时，工作组才会在最终指南中包含推荐声明。

结果

该工作组提出了 10 项 CRC 筛查建议，强调共同、个性化的决策制定和熟悉 CF 特定的胃肠道挑战。我们建议将结肠镜检查作为首选筛查方法，从 40 岁开始筛查，每 5 年重新筛查一次，每 3 年监测一次（除非个别检查结果表明间隔时间更短），以及针对 CF 的强化肠道准备。由于与免疫抑制相关的结肠癌的额外风险，患有 CF 的器官移植受者应在移植后 2 年内在 30 岁时开始 CRC 筛查。

结论

这些建议旨在帮助 CF 成人、家庭、初级保健医生、胃肠病学家以及 CF 和移植中心解决 CRC 筛查问题。在建议的筛查开始年龄、筛查方法、准备以及重复筛查和监测的间隔等方面，它们不同于为一般人群制定的指南。