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Skewed T-cell receptor variable beta repertoire and massive T-cell activation in idiopathic orofacial granulomatosis

Authors :
Elena Ortona
Marina Pierdominici
D. Pennino
Elisabetta Caprini
A. Cavani
Antonello Giovannetti
Giuseppe Donato
Felice Salsano
Edoardo Rosato
Francesca Mazzetta
Source :
Scopus-Elsevier

Abstract

Orofacial granulomatosis (OFG) is a clinicopathologic entity describing oral lesions with noncaseating granulomas including a spectrum of diseases such as the Melkersson-Rosenthal syndrome. The involvement of abnormal T-cell responses has been suggested in the pathogenesis of OFG although few and contrasting data are currently available on this issue. In a patient with OFG, we observed virtually complete CD4 and CD8 T-cell receptor (TCR) ?-chain variable region (BV) repertoires at the lesion level and in circulation. However, oligoclonal profiles were found in CD4 and, to a greater extent, in CD8 subsets. These findings were seen in association with a massive peripheral T-cell activation, decreased naive T cells, reduced thymic output, altered cytokine production, and increased apoptosis. Our data, pointing to a random influx of T cells at the site of inflammation, argue against the hypothesis of a main allergen acting at the level of oral mucosa. The profound dysregulation of the peripheral T-cell compartment suggests that OFG should be regarded as a systemic disorder with localized manifestations.

Details

Database :
OpenAIRE
Journal :
Scopus-Elsevier
Accession number :
edsair.doi.dedup.....07ce46ff3b14a27599f3b2d6caf3f045