Early-Onset and Late-Onset Idiopathic Chronic Pancreatitis in the North American Pancreatitis Study (NAPS2-CV) Cohort

Abstract

Introduction: Idiopathic chronic pancreatitis (ICP) defines a group of patients in whom no etiology is evident after standard clinical work-up. ICP is heterogeneous and can be divided into early-onset ICP (EO-ICP) and late-onset ICP (LO-ICP). Layer et al reported clinical differences between EO-ICP and LO-ICP, inlcluding pain, pancreatic insufficiency, and clinical course at Mayo Clinic.[1] These findings have not been replicated or validated in an independent North American cohort. Aims: To compare features of ICP between the NAPS2-CV and Mayo Clinic cohorts and potentially validate classification of ICP into EO-ICP and LO-ICP groups. Methods: NAPS2-CV prospectively ascertained patients with chronic pancreatitis using detailed demographic, phenotypic, and etiological case report forms. The diagnosis of ICP was made by expert physicians and then divided by age (EO-ICP: <35 years, and LO-ICP >35 years). We compared the clinical features of the NAPS2-CV and Mayo Clinic cohorts. Results: ICP was identified in 126/521 (24%). The fractions of ICP as EO-ICP versus LO-ICP were similar between NAPS2-CV and Mayo cohorts. Patients with EO-ICP were more likely to have pain than LO-ICP. EO-ICP and LO-ICP had similar rates of exocrine insufficiency, endocrine insufficiency, and calcifications. Prevalence of distinct clinical features were similar between NAPS2-CV and Mayo cohorts (Table). Conclusion: EO-ICP and LO-ICP differ primarily with respect to pain. The clinical profiles of NAPS2-CV replicate Layer. Further insights will require analysis of deeper data sets.Table 1