Details

Autor(en) / Beteiligte

• Giovannetti, A
• Mazzetta, F
• Cavani, A
• Pennino, D
• Caprini, E
• Ortona, E
• Donato, G
• Rosato, E
• Salsano, F
• Pierdominici, M

Titel

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

Ist Teil von

• International journal of immunopathology and pharmacology, 2012, Vol.25 (2), p.503-511

Erscheinungsjahr

2012

Quelle

Free E-Journal (出版社公開部分のみ）

Beschreibungen/Notizen

• 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.