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Skewed T-cell receptor variable beta repertoire and massive T-cell activation in idiopathic orofacial granulomatosis
- 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.
- Subjects :
- Pathology
medicine.medical_specialty
medicine.medical_treatment
T cell
Immunology
Inflammation
Biology
orofacial granulomatosis
t-cell activation
Lesion
Pathogenesis
cytokines
apoptosis
t-cell receptor repertoire
t cells
medicine
Immunology and Allergy
Pharmacology
T-cell receptor
medicine.disease
Cytokine
medicine.anatomical_structure
Orofacial granulomatosis
medicine.symptom
CD8
Subjects
Details
- Database :
- OpenAIRE
- Journal :
- Scopus-Elsevier
- Accession number :
- edsair.doi.dedup.....07ce46ff3b14a27599f3b2d6caf3f045