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.
Schiepatti, A., Maimaris, S., Scalvini, D., Raju, S., Ingham, K.E., Johnson, C.M., et al. (2025). LONG-TERM PROGNOSIS of NON-CELIAC ENTEROPATHIES and A SCORE to IDENTIFY PATIENTS with POOR OUTCOMES: A 30-YEAR MULTICENTER LONGITUDINAL STUDY. THE AMERICAN JOURNAL OF GASTROENTEROLOGY, 1, 1-10 [10.14309/ajg.0000000000003331].
LONG-TERM PROGNOSIS of NON-CELIAC ENTEROPATHIES and A SCORE to IDENTIFY PATIENTS with POOR OUTCOMES: A 30-YEAR MULTICENTER LONGITUDINAL STUDY
Caio G.;Volta U.;Marasco G.;Barbara G.;Biagi F.
2025
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
