The presence of dominant T-cell clones in peripheral blood of patients with collagen vascular disorders: a prospective study of 97 cases

Br J Dermatol. 2006 Mar;154(3):445-9. doi: 10.1111/j.1365-2133.2005.07044.x.

Abstract

Background: T-lymphocyte dysfunction has been seldom investigated in collagen vascular disorders. The search for dominant T-cell clones has been scarcely reported, although the presence of such clones might be expected in disorders showing immune responses directed against a variety of autoantigens.

Objectives: We conducted a systematic search for dominant T-cell clones in peripheral blood in patients with collagen vascular disorders. Patients and methods Ninety-seven patients with collagen vascular disorders were studied (7 cutaneous and 38 systemic lupus erythematosus; 8 multiple morphea; 12 regional scleroderma; 32 systemic sclerosis of the CREST type). A dominant T-cell clone was searched for in peripheral blood by polymerase chain reaction targeting the T-cell receptor gamma chain followed by a size analysis of amplified fragments. Peripheral blood from patients with nonlymphocyte-dependent disorders and matched by age and sex was assessed in the same conditions. Results in both groups were compared using nonparametric statistical tests.

Results: Overall, a circulating dominant T-cell clone was found in 52% of patients compared with 16.9% in controls. More precisely, such a dominant clone was present in 43% and 37% of cutaneous and systemic lupus erythematosus, respectively, in 75% of multiple morphea, 75% of regional scleroderma and 60% of CREST syndrome patients. The percentages in all subsets of patients were significantly higher than in the control group.

Conclusions: The presence of a dominant T-cell clone in peripheral blood is significantly more frequent in collagen vascular disorders than in controls, especially in patients with scleroderma, whatever the clinical subset, which suggests T-cell involvement in the immune response dysfunction in these diseases classically characterized by disturbances of B lymphocytes. The relevance of such a dominant clone regarding diagnosis, pathomechanisms, long-term outcome and visceral prognosis of these diseases as well as therapeutic decisions remains to be evaluated.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Autoimmune Diseases / immunology*
  • CREST Syndrome / immunology
  • Child
  • Child, Preschool
  • Clone Cells / immunology
  • Connective Tissue Diseases / immunology*
  • Female
  • Humans
  • Lupus Erythematosus, Cutaneous / immunology
  • Lupus Erythematosus, Systemic / immunology
  • Male
  • Middle Aged
  • Prospective Studies
  • Receptors, Antigen, T-Cell, gamma-delta / genetics
  • Scleroderma, Localized / immunology
  • Scleroderma, Systemic / immunology
  • T-Lymphocytes / immunology*

Substances

  • Receptors, Antigen, T-Cell, gamma-delta