The intestinal mucosal lesion in celiac disease is characterized by a predominant T-cell infiltration of both epithelium and lamina propria. However, a restricted use of T-cell receptors (TCR) in T lymphocytes infiltrating che jejunal mucosa of celiac patients has not been reported. Based on an immunohistochemical survey of jejunal biopsies from a cohort of untreated celiac patients, we demonstrated a small but significant increase of V beta 8.1/2(+) T cells in the lamina propria, but not in the epithelium nor in the peripheral blood, Sequence analysis indicated the existence of a variable degree of clonality of V beta 8(+) T cells in the celiac mucosa. More importantly, the recurrence of identical CDR3 regions in some patients was also observed. The altered distribution of V beta 8(+) T cells and the presence of identical CDR3 regions in celiac patients, but not in controls vies independently confirmed by CDR3 size analysis in a further cohort of patients. These findings suggest that disease-specific variations of the TCRBV8 repertoire are present in the small intestinal mucosa of untreated celiac patients. (C) American Society for Histocompatibility and Immunogenetics, 1997. Published by Elsevier Science Inc.