Abstract

Crohn’s disease (CD) is a relapsing chronic inflammatory disorder that may involve all the gastrointestinal tract with a prevalence of terminal ileum. Intestinal lesions have a characteristic discontinuous and segmental distribution and may affect all layers of the gut wall. Telocytes (TC), a peculiar type of stromal cells, have been recently identified in a variety of tissues and organs, including gastrointestinal tract of humans and mammals. Several roles have been proposed for TC, including mechanical support, spatial relationships with different cell types, intercellular signalling and modulation of intestinal motility. The aim of our study was to investigate the presence and distribution of TC in disease-affected and -unaffected ileal specimens from CD patients compared with controls. TC were identified by CD34/PDGFRα immunohistochemistry. In affected CD specimens TC disappeared, particularly where fibrosis and architectural derangement of the intestinal wall were observed. In the thickened muscularis mucosae and submucosa, few TC entrapped in the fibrotic extracellular matrix were found. A discontinuous network of TC was present around smooth muscle bundles, ganglia and enteric strands in the altered muscularis propria. At the myenteric plexus, the loss of TC network was paralleled by the loss of interstitial cells of Cajal network. In the unaffected CD specimens, TC were preserved in their distribution. Our results suggest that in CD the loss of TC might have important pathophysiological implications contributing to the architectural derangement of the intestinal wall and gut dysmotility. Further functional studies are necessary to better clarify the role of TC loss in CD pathophysiology.

Highlights

  • Crohn’s disease (CD) is a complex chronic inflammatory disorder of the gastrointestinal (GI) tract, formerly described and classified as a form of segmental ileitis [1]

  • The presence of TC was investigated in full-thickness biopsies of terminal ileum obtained from CD patients and controls

  • Telocytes were identified by CD34 immunostaining and were distinguishable by other CD34-positive cells, such as CD31-positive vascular endothelial cells, and by c-kit-positive interstitial cells of Cajal (ICC)

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Summary

Introduction

Crohn’s disease (CD) is a complex chronic inflammatory disorder of the gastrointestinal (GI) tract, formerly described and classified as a form of segmental ileitis [1]. The incidence and prevalence of the disease in the developed world have markedly increased in the last decades [2, 3]. Crohn’s disease is characterized by a segmental and transmural bowel wall relapsing inflammation that waxes and wanes over years, The behaviour of CD substantially varies during the course of the disease, whereas its anatomical location is mostly stable [4]. Crohn’s disease is located in the terminal ileum in 45%, colon in 32%, ileocolon in 19%, and upper GI tract in 4% of cases [5]. Medical treatment consists mainly of symptomatic and supportive care, but it is still considered far from satisfying, and surgical treatment has still a high incidence [2, 5]

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