The incidence and prevalence of inflammatory bowel disease [IBD] have increased significantly in low- and middle-income countries [LMICs] in recent decades. Managing IBD in these settings presents substantial challenges. This consensus aims to describe the epidemiology of IBD in LMICs and to highlight the key challenges in its diagnosis and treatment.

The consensus-defining strategy followed the previous European Crohn’s and Colitis Organisation [ECCO] consensus guidelines [available at www.ecco-ibd.eu]. The authors reviewed the available evidence and formulated statements accordingly. Provisional ECCO statements and supporting text were drafted based on a comprehensive literature review and further refined through two voting rounds, which included external reviewers and national representatives from ECCO’s 36 member countries. The final ECCO statements, representing a consensus of at least 80% agreement among participants, were approved during an online meeting. Consensus statements should be interpreted in context with their accompanying commentary rather than in isolation and should not be used solely to guide patient management. The supporting text was finalized under the guidance of each working group leader [VP, HY, TK, AH] and subsequently integrated by the consensus leader [AE].

Data on IBD epidemiology in LMICs remain limited. Public and healthcare professional awareness and timely access to early diagnostic modalities, advanced medical and surgical therapies, and specialist multidisciplinary care are key gaps in IBD care in LMICs. The complexity and chronic nature of IBD, along with the necessity for a multidisciplinary approach, pose significant challenges to adopting a holistic management strategy in LMICs.

There is a critical need for further studies to assess the specific needs of LMICs. Such research will help guide resource allocation and improve IBD management in these settings.

Importance

The lack of multidisciplinary workflow guidelines and clear definitions and classifications for neoplasms in and around the ampulla of Vater results in inconsistencies affecting patient care and research.

Objective

The PERIPAN international multidisciplinary consensus group aimed to standardize the multidisciplinary diagnostic workflow and achieve consensus on definitions and classifications in order to ensure proper classification and optimal diagnostic assessment and consequently to improve patient care and future research.

Design

An international team of 43 experts (pathologists, surgeons, radiologists, gastroenterologists, oncologists) from 12 countries identified knowledge gaps, reviewed 37061 articles, and proposed recommendations using the Scottish Intercollegiate Guidelines Network methodology (SIGN), including the Delphi methodology and the AGREEII tool for quality assessment and external validation.

Results

The 38 consensus questions and 51 recommendations provide guidance on the following key aspects: I. More specific anatomic criteria for the definition of what qualifies as “ampullary” neoplasms, their distinction from duodenal and common bile duct tumors, and clinicopathologic characteristics of anatomic subsets; II. Avoidance of the confusing term “periampullary” for final classification; III. Refined definitions of intestinal, pancreatobiliary and mixed subtypes, and introduction of rare histologic subtypes; IV. The use and limitations of immunohistochemical and molecular profiling; V. Biopsy acquisition; VI. Clinical information required for accurate pathology assessment of biopsies and ampullectomy specimens; VII. Key items to be included in pathology reports of endoscopic specimens.

Conclusions and Relevance

Recognition of the Brescia PERIPAN guidelines will allow a more accurate classification of true ampullary cancers and their differentiation from other “periampullary” tumors. This will have significant implications for endoscopic interpretation and management, staging, pathologic diagnosis and therapeutic evaluation as well as oncologic treatment of various anatomic and histologic subsets of ampullary tumors. This will enhance the quality of both clinical care and future research in this complex medical field.