Development and validation of the Crohn's disease-intestinal failure-wean (CDIF-Wean) Score to predict outcomes of intestinal rehabilitation

Background & aims: Enteral autonomy, a key outcome of intestinal rehabilitation in patients with intestinal failure (IF), is challenging to predict due to disease complexity and heterogeneity. The aim of this cohort study is to develop and validate a multivariate model to predict enteral autonomy in patients with IF caused by Crohn's disease (CDIF), and to derive an outcome-based severity classification for CDIF.

Methods: The CDIF-Wean Score was constructed and internally validated in a cohort of 182 patients with CDIF from a tertiary IF unit. We performed stepwise backward selection to include relevant and significant clinical variables in a binomial regression with inverted probability of censoring weighting. The Score was externally validated in a separate cohort of 107 patients with CDIF from an independent tertiary IF unit. A severity classification, based on the CDIF-Wean Score, was evaluated with cumulative incidence curves for enteral autonomy and death during home parenteral support (HPS).

Results: In the CDIF-Wean Score, age, HPS duration, chronicity of Crohn's disease, intestinal anatomy, and eligibility and type of reconstructive surgery was predictive of enteral autonomy. The Score performed well in discrimination and calibration, with 0.84 and 0.84 area under the receiver operating characteristic curve, and 0.13 and 0.16 Brier scores in internal and external validation, respectively. The CDIF severity classification was significantly associated with both short- and long-term prognosis, where mild patients had a 7.5, 5.8 and 5.2-fold higher probability of enteral autonomy than severe patients at 1, 5 and 10 years (p<0.0001).

Conclusion: The CDIF-Wean Score is the first validated prediction model for IF outcomes, and demonstrates accuracy, robustness and generalisability in the prognostication of CDIF patients.