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A loss of telocytes accompanies fibrosis of multiple organs in systemic sclerosis
Author(s) -
Manetti Mirko,
Rosa Irene,
Messerini Luca,
Guiducci Serena,
MatucciCerinic Marco,
IbbaManneschi Lidia
Publication year - 2014
Publication title -
journal of cellular and molecular medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.44
H-Index - 130
eISSN - 1582-4934
pISSN - 1582-1838
DOI - 10.1111/jcmm.12228
Subject(s) - pathology , fibrosis , cd34 , cd31 , biology , interstitial space , connective tissue , muscularis mucosae , stromal cell , anatomy , medicine , immunohistochemistry , stem cell , microbiology and biotechnology
Systemic sclerosis ( SS c) is a complex connective tissue disease characterized by fibrosis of the skin and various internal organs. In SS c, telocytes, a peculiar type of stromal (interstitial) cells, display severe ultrastructural damages and are progressively lost from the clinically affected skin. The aim of the present work was to investigate the presence and distribution of telocytes in the internal organs of SS c patients. Archival paraffin‐embedded samples of gastric wall, myocardium and lung from SS c patients and controls were collected. Tissue sections were stained with M asson's trichrome to detect fibrosis. Telocytes were studied on tissue sections subjected to CD 34 immunostaining. CD 34/ CD 31 double immunofluorescence was performed to unequivocally differentiate telocytes ( CD 34‐positive/ CD 31‐negative) from vascular endothelial cells ( CD 34‐positive/ CD 31‐positive). Few telocytes entrapped in the fibrotic extracellular matrix were found in the muscularis mucosae and submucosa of SS c gastric wall. In the muscle layers and myenteric plexus, the network of telocytes was discontinuous or even completely absent around smooth muscle cells and ganglia. Telocytes were almost completely absent in fibrotic areas of SS c myocardium. In SS c fibrotic lung, few or no telocytes were observed in the thickened alveolar septa, around blood vessels and in the interstitial space surrounding terminal and respiratory bronchioles. In SS c, the loss of telocytes is not restricted to the skin, but it is a widespread process affecting multiple organs targeted by the fibrotic process. As telocytes are believed to be key players in the regulation of tissue/organ homoeostasis, our data suggest that telocyte loss might have important pathophysiological implications in SS c.

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