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. 2025 Jan 29.
doi: 10.14309/ajg.0000000000003331. Online ahead of print.

Long-term Prognosis of Nonceliac Enteropathies and a Score to Identify Patients With Poor Outcomes: A 30-year Multicenter Longitudinal Study

Annalisa Schiepatti  1   2 , Stiliano Maimaris  1   2 , Davide Scalvini  1   2 , Suneil A Raju  3 , Katerina E Ingham  3 , Calvin M Johnson  3 , Alberto Rubio-Tapia  4 , Chiara Maruggi  4 , Georgia Malamut  5 , Marco Vincenzo Lenti  1   6 , Antonio Di Sabatino  1   6 , Giacomo Caio  7 , Umberto Volta  8 , Fabiana Zingone  9 , Giovanni Marasco  8   10 , Giovanni Barbara  8   10 , Govind Makharia  11 , Lalita Mehra  12 , Prasenjit Das  12 , Knut E A Lundin  13 , Simon S Cross  14 , David S Sanders  3 , Federico Biagi  1   2
Affiliations

Long-term Prognosis of Nonceliac Enteropathies and a Score to Identify Patients With Poor Outcomes: A 30-year Multicenter Longitudinal Study

Annalisa Schiepatti et al. Am J Gastroenterol. .

Abstract

Introduction: Long-term prognosis of nonceliac 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 United Kingdom, 1 French, 1 Norwegian, 1 United States, 1 Indian) followed-up over 30 years were enrolled. Complications and mortality were analyzed 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: Two hundred sixty-one patients were enrolled (144 female, mean age at diagnosis 49 ± 18 years, median follow-up 70 months, interquartile range 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%). Five-year and 10-year complication-free survival were 89% and 77%, respectively, whereas 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% confidence interval [CI] 2.24-4.34). Older age at diagnosis ( P < 0.001), anemia (hazard ratio [HR] 2.53, 95% CI 1.33-4.80, P < 0.01), and 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 (0 pts), low risk (1-2 pts), intermediate risk (3 pts), and high risk (4-5 pts), with 10-year survival rates of 100%, 87%, 62%, and 16%, respectively.

Discussion: Mortality in NCEs is increased because of complications and lack of response to current therapies. We developed a clinical score to personalize follow-up. Targeted treatments are needed to improve outcomes.

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References

    1. Schiepatti A, Sanders DS, Baiardi P, et al. Nomenclature and diagnosis of seronegative coeliac disease and chronic non-coeliac enteropathies in adults: the Paris consensus. Gut 2022;71(11):2218–25.
    1. Leonard MM, Lebwohl B, Rubio-Tapia A, et al. AGA clinical practice update on the evaluation and management of seronegative enteropathies: Expert review. Gastroenterology 2021;160(1):437–44.
    1. Jansson-Knodell CL, Murray JA, Rubio-Tapia A. Management of small bowel villous atrophy in patients seronegative for celiac disease. Am J Gastroenterol 2020;115(4):492–7.
    1. Schiepatti A, Sanders DS, Zuffada M, et al. Overview in the clinical management of patients with seronegative villous atrophy. Eur J Gastroenterol Hepatol 2019;31(4):409–17.
    1. Al-Toma A, Volta U, Auricchio R, et al. European Society for the Study of Coeliac Disease (ESsCD) guideline for coeliac disease and other gluten-related disorders. United Eur Gastroenterol J 2019;7(5):583–613.

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