LONG-TERM PROGNOSIS OF NON-CELIAC ENTEROPATHIES AND A SCORE TO IDENTIFY PATIENTS WITH POOR OUTCOMES: A 30-YEAR MULTICENTER LONGITUDINAL STUDY.

Abstract

INTRODUCTION Long-term prognosis of non-celiac enteropathies (NCEs) is poorly understood. We aimed to evaluate long-term outcomes and develop a prognostic score for NCEs. METHODS NCEs patients from an international multicenter cohort (4 Italian centers,1 UK, 1 French,1 Norwegian,1 USA,1 Indian) followed-up over 30 years were enrolled. Complications and mortality were analysed with Kaplan-Meier curves, standardized mortality ratios (SMR) and multivariate Cox regression. A clinical score to identify patients at risk of poor outcomes was developed. RESULTS 261 patients were enrolled (144F, mean age at diagnosis 49±18 years, median follow-up 70 months, IQR 24-109). The most common etiologies were idiopathic villous atrophy (39%), drug-related (17%), common variable immune-deficiency (15%), infectious (10%) and autoimmune enteropathy (9%). 5-year and 10-year complication-free survival were 89% and 77%, respectively, while 5-year and 10-year overall survival were 88% and 74%, respectively. Causes of death included sepsis/major infections (22%), lymphoproliferative disorders (22%), solid-organ malignancies (12%), cardiovascular/metabolic disease (10%), and was unknown in 33%. Mortality was increased in NCEs compared with the general population (SMR 3.17, 95%CI 2.24-4.34). Older age at diagnosis (p<0.001), anemia (HR 2.53,95%CI 1.33-4.80,p<0.01), lack of clinical (HR 3.21,95%CI 1.68-6.18,p<0.01) and histological response (HR 2.14,95%CI 1.08-4.23,p=0.04) were independent predictors of mortality at Cox regression. A 5-point score was developed to identify high-risk patients: very low risk (0pts), low risk (1-2pts), intermediate risk (3pts) and high risk (4-5pts), with 10-year survival rates of 100%, 87%, 62% and 16%, respectively. CONCLUSIONS Mortality in NCEs is increased due to complications and lack of response to current therapies. We developed a clinical score to personalise follow-up. Targeted treatments are needed to improve outcomes.