Coeliac disease: changing views on gluten-sensitive enteropathy

Area of Science:

• Gastroenterology
• Immunology
• Genetics

Background:

• Coeliac disease research has advanced significantly, necessitating a revised understanding of its pathophysiology and clinical presentation.
• Immunogenetic studies link coeliac disease to the HLA region, while immunological research highlights T-cell responses and specific antibodies.
• Histopathology has evolved, with the Marsh classification of gluten-sensitive enteropathy providing a spectrum of intestinal changes.

Purpose of the Study:

• To provide an update on coeliac disease, focusing on recent advancements in immunogenetics, epidemiology, and particularly histopathology.
• To detail the spectrum of intestinal histopathology in gluten-sensitive enteropathy as defined by the Marsh classification.
• To highlight findings in refractory coeliac disease, including aberrant T cells.

Main Methods:

• Review and synthesis of recent scientific literature on coeliac disease.
• Concentration on the histopathological work of the Arnhem research group.
• Identification and validation of the Marsh classification spectrum for gluten-sensitive enteropathy.

Main Results:

• The spectrum of intestinal histopathology in gluten-sensitive enteropathy is described, encompassing lymphocytic enteritis (Marsh I), with crypt hyperplasia (Marsh II), and villous atrophy (Marsh III - partial, subtotal, total).
• Refractory coeliac disease patients were studied, with a subgroup showing (pre-)malignant aberrant T cells in intestinal mucosa.

Conclusions:

• Coeliac disease is characterized by evolving immunogenetic, epidemiological, and histopathological profiles.
• A new conceptual framework for coeliac disease is emerging, driven by these advancements.
• Understanding the spectrum of histopathology and identifying aberrant cells in refractory cases are crucial for clinical management.