Background: Immunologic abnormalities seem to play an important role in important role in systemic sclerosis (SSc).
Methods: We studied the following immune parameters to get more insight into SSc: autoantibodies (antinuclear antibodies (ANA), anti-Scl-70, anticentromere antibodies (ACA) subsets of lymphocyte subpopulations and markers of their activation, as well as serum levels of IL-2, the soluble IL-2 receptor (SIL-2R), IL-6 and its correlation to N-terminal procollagen-III propeptide (P III P), and finally, the IL-6 production by SSc and normal dermal fibroblasts.
Results: In patients with active SSc, we found a reduced number of CD2+ T-lymphocytes and an increase in the expression of T-lymphocyte activation markers such as CD25+ and CD71+, HLA-DR Ia, as well as elevated serum levels of SIL-2LR and IL-6. SSc fibroblasts did not produce more IL-6 than normal fibroblasts in monolayer cultures.
Conclusions: Our data show that a wide range of immunologic parameters are altered in SSc. In general, T-helper (TH) lymphocytes are activated possibly because of reduced T-suppressor (TS) and natural killer (NK)-cell levels. TH may polyclonally stimulate B cells, which in turn produce higher amounts of autoantibodies. Our findings support the concept that TH cell-derived cytokines/growth factors stimulate matrix protein synthesis by fibroblasts, resulting in generalized fibrosis.