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

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) β-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.

• Authors: A Giovannetti, F Mazzetta, A Cavani, D Pennino, E Caprini, E Ortona, G Donato, E Rosato, F Salsano, M Pierdominici
• Journal: International journal of immunopathology and pharmacology (Int J Immunopathol Pharmacol) 2012 Apr-Jun Vol. 25 Issue 2 Pg. 503-11 ISSN: 0394-6320 [Print] England
• PMID: 22697083 (Publication Type: Case Reports, Letter)

Chemical References

• Biomarkers
• Cytokines
• Receptors, Antigen, T-Cell, alpha-beta

Topics

• Apoptosis
• Biomarkers (metabolism)
• CD4-Positive T-Lymphocytes (immunology, metabolism, pathology)
• CD8-Positive T-Lymphocytes (immunology, metabolism, pathology)
• Cells, Cultured
• Cytokines (metabolism)
• Flow Cytometry
• Humans
• Immunophenotyping
• Lip (immunology, pathology)
• Lymphocyte Activation (immunology)
• Male
• Melkersson-Rosenthal Syndrome (diagnosis, immunology)
• Receptors, Antigen, T-Cell, alpha-beta (biosynthesis, immunology)
• T-Lymphocyte Subsets (immunology, metabolism, pathology)
• Young Adult